George Orphanos,a Panteleimon Kountourakisb
on 17/10/2012 11:10:00 The advent of targeted therapies has revolutionized the treatment of certain types of cancer. Identification of molecular targets on cancer cells has led to the design of novel drugs, which either used as single agents or in combination with chemotherapy, has prolonged survival in metastatic disease, or contributed to curative treatment in the adjuvant setting. A literature review was conducted to identify and present current knowledge on the molecular function of the HER2 receptor, its role in the pathogenesis of breast cancer and anti-HER2 targeted drugs in use or under development. Many molecular targets have been identified in breast cancer, with the HER family of receptors being the ones most extensively studied. Trastuzumab and lapatinib target the HER2 receptor and are approved drugs for the treatment of metastatic breast cancer. Several other targeted agents, including T-DM1, pertuzumab, neratinib, afatinib and ertumaxomab, are currently being tested in vivo as well as in clinical studies. The use of targeted therapies in metastatic breast cancer has improved prognosis, increased survival and dramatically changed the way we treat breast cancer patients today. Mechanism of paroxysmal nocturnal hemoglobinuria clonal dominance: possible roles of different apoptosis and CD8+ lymphocytes in the selection of paroxysmal nocturnal hemoglobinuria clones ================================================================================Rajita Kunyaboon, Wanchai Wanachiwanawin, Yaowalak U-Pratya, Anchalee Thedsawad, Orathai Taka
on 17/10/2012 11:00:00 BACKGROUND AND OBJECTIVES: Paroxysmal nocturnal hemoglobinuria (PNH), a clonal hematopoietic stem cell disorder, manifests when the PNH clone populates in the hematopoietic compartment. We explored the roles of different apoptosis of GPI+ and GPI- (glycosylphosphatidylinositol) cells and CD8+ lymphocytes in a selection of PNH clones. PATIENTS AND METHODS: Granulocytes from PNH patients and normal controls were subjected to an apoptosis assay using annexin V. Hematopoietic cell in semisolid media were cultured with or without CD8+ lymphocytes. RESULTS: In PNH, CD59+ granulocytes exhibited more apoptosis than their CD59- counterparts, after 0 or 4 hours in liquid growth culture system (mean [standard error of mean]: 2.1 (0.5) vs 1.2 (0.2), P=.01 at 0 hour and 3.4 [0.7] vs 1.8 [0.3], P=.03 at 4 hour, respectively). The presence of mononuclear cells (MNCs) rendered a greater difference in apoptosis. The percentages of apoptotic CD59+ granulocytes measured at 4 hours with or without MNC fraction were correlated with the sizes of PNH clones (r=0.633, P=.011; and r=0.648, P=.009; respectively). The autologous CD8+ lymphocytes inhibited CFU-GM and BFU-E colony formation in PNH patients when compared with normal controls (mean [SEM] of percentages of inhibition: 61.7 (10.4) vs 11.9 (2.0), P=.008 for CFU-GM and 26.1 (6.9) vs 4.9 (1.0), P=.037 for BFU-E). CONCLUSIONS: Increased apoptosis of GPI+ blood cells is likely to be responsible in selection and expansion of PNH clones. MNCs or possibly CD8+ lymphocytes may play a role in this phenomenon. Clinical outcome of docetaxel in advanced or metastatic endometrial cancer ================================================================================Rasha Hamdy Hamed, Seham Elsayed Abdelkhalek
on 17/10/2012 10:51:00 BACKGROUND AND OBJECTIVES: The aim of this study was to evaluate the efficacy, safety and toxicity of docetaxel as first-line chemotherapy or in previously treated patients (one regimen) with recurrent or metastatic endometrial cancer. DESIGN AND SETTING: Prospective phase II study in patients referred to the Department of Clinical Oncology and Nuclear Medicine, Faculty of Medicine, Mansoura University, Egypt. PATIENTS AND METHODS: Fifty patients with advanced or metastatic endometrial cancer were enrolled to receive docetaxel 70 mg/m2 administered intravenously on day 1 of a 3-week cycle. If patients responded well to docetaxel, additional cycles were administered until progressive disease or unacceptable toxicity occurred. Therapy response was evaluated every 6 weeks. RESULTS: Of 50 patients with a median age of 60 years (range, 40-70 years) who entered the study, 17 patients (34%) had received one prior chemotherapy regimen. All patients were evaluable for efficacy, yielding an overall response rate of 34% (95% confidence interval, 14.8%–55.6%); complete response and partial response (PR) were 4% and 30%, respectively. Of 17 pretreated patients, 5 (29%) had a PR. The median duration of response was 2 months. The median time-to-progression was 4 months and the median survival time was 18 months. The predominant toxicity was grade 3-4 neutropenia, occurring in 92% of the patients, although febrile neutropenia arose in 10% of the patients. Edema was mild and infrequent. CONCLUSION: The study clearly demonstrated that docetaxel is active in the treatment of endometrial cancer. Toxicity was manageable and predominantly hematologic. Addition of amifostine to the CHOP regimen in elderly patients with aggressive non-Hodgkin lymphoma: a phase II trial showing reduction in toxicity without altering long-term survival ================================================================================Henry L. Gómez,a César Samanéz,a Frank Campana,a Silvia P. Neciosup,a Luis Vera,b Luis Casanova,a Jorge Leon,a Claudio Flores,c Fernando Hurtado de Mendoza,b Carlos A. Casteñeda,a Joseph A. Pinto,c Carlos S. Vallejosc
on 17/10/2012 10:35:00 BACKGROUND AND OBJECTIVES: We report the 8-year follow-up of 34 patients aged ≥69 years old with NHL included in a phase IIb open-label randomized parallel groups study to evaluate the effectiveness of amifostine in preventing the toxicity of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP regime) . PATIENTS AND METHODS: Patients were randomized to receive classical CHOP (cyclophosphamide 750 mg/ m2, doxorubicin 50 mg/m2, vincristine 1.4 mg/m2 [maximum 2 mg] on day 1 and prednisone 100 mg/day for 5 days) or CHOP plus amifostine (6 cycles of amifostine 910 mg/m2 on day 1). Efficacy (time to progression, TTP; disease-free survival, DFS; overall survival, OS) and toxicity endpoints were evaluated. RESULTS: Thirty-four patients were randomized to A-CHOP (n=18) or CHOP (n=16). Patients with A-CHOP vs CHOP had significantly lower toxicity; neutropenia grade 4 ocurred in 13/92 (13%) vs 23/85 (27%, P=0.007) cycles, febrile neutropenia in 3/92 A-CHOP (3%) vs 8/85 (10%, P=.056) CHOP cycles, hospitalization for toxicity in 4/92 (4%) A-CHOP vs 11/85 (13%, P=.05) CHOP cycles. Median hospitalization stay for toxicity was 5 days with A-CHOP vs 8 days with CHOP (P=.05). There were no significant differences at 8 years in TTP (A-CHOP, 48.9% vs CHOP, 36.3%; P=.65), DFS (A-CHOP, 72.9% vs CHOP 55.6%; P=.50) and OS (A-CHOP, 44.3% vs CHOP, 54.4%). There was no long-term toxicity of clinical interest. The only prognostic factor identified to 8 years was the International Prognostic Index (IPI low/low intermediate risk vs high intermediate/high risk; HR=2.98; CI 95%:1.01-8.77; P=.048). CONCLUSION: These results show that amifostine can be added to the standard CHOP treatment schedule with less acute toxicity and without influencing the outcome. The effect of low altitude on blood count parameters ================================================================================Suleimman A. Al-Sweedan, Moath Alhaj
on 17/10/2012 10:28:00 BACKGROUND AND OBJECTIVES: High altitude has an effect on blood count parameters, but low altitude (especially below sea level) has not been studied. DESIGN AND SETTING: A cross-sectional study of aymptomatic subjects aged between 18 to 35 years of age who had reported to the blood bank at the King Abdullah University Hospital (KAUH)/Irbid and Ministry of Health, Jordan, during the period between January 2010 to June 2011 for blood donation. METHODS: Hematological values were compared in healthy adult blood donors living in areas 200 to 300 meters below sea level and areas 500 to 1500 meters above sea level. The study population consisted of 800 females and 666 males aged between 18 to 35 years. RESULTS: The mean values for hemoglobin level, mean corpuscular volume and leukocyte counts were significantly higher in people living above sea level than in people living below sea level (P A New Hint to Clonal Dominance in PNH ================================================================================Syed Z. A. Zaidi
on 17/10/2012 10:21:00 Allogeneic stem cell transplantation for acute myeloid leukemia with del(7q) following untreated chronic lymphocytic leukemia ================================================================================Zachariah DeFilipp, Donny V. Huynh, Salman Fazal, Entezam Sahovic
on 17/10/2012 10:03:00 The development of hematologic malignancy in the presence of chronic lymphocytic leukemia (CLL) is rare. We present a case of acute myeloid leukemia (AML) with del(7q) occurring in a patient with a 4-year history of untreated CLL. Application of flow cytometry and immunohistochemistry allowed for characterization of two distinct coexisting malignant cell populations. After undergoing induction and consolidation chemotherapy, the patient achieved complete remission of AML with the persistence of CLL. Allogeneic transplantation was pursued given his unfavorable cytogenetics. Subsequent matched unrelated donor allogeneic stem cell transplantation resulted in full engraftment and complete remission, with no evidence of AML or CLL. Due to a scarcity of reported cases, insight into treatment and prognosis in cases of concurrent AML and CLL is limited. However, prognosis seems dependent on the chemosensitivity of AML. CLL did not have a detrimental effect on treatment or transplant outcome in our case. This is the first reported case of concomitant de novo AML and CLL to undergo allogeneic transplantation. The patient remained in complete hematologic and cytogenetic remission of both malignancies over a year after transplantation. The t(9;11) confers good prognosis in AML patients treated with stem cell transplantation as compared to non-t(9;11) and other adverse-risk abnormalities ================================================================================Claudia Ulrike Walter,a Fahad Al Mohareb,b Naeem Chaudhri,b Fahad Alsharif,b Hazzaa Al Zahrani,b Said Mohamed,b Walid Rasheed,b Abu Jafar Saleh,b Wahiba Chebbo,b Ghada El Gohary,b Mahmoud Aljurf,b Maher Albitara
on 17/10/2012 09:55:00 Oral chemotherapy in cancers: what about adherence? ================================================================================Rachid Tanz, Tarik Mahfoud, Adil Debbagh, Mohammed Reda Khmamouch, Aziz Bazine, Tarik Namad, Mohammed Ichou, Hassan Errihani
on 17/10/2012 09:45:00 Extramedullary plasmacytoma of the liver ================================================================================Hicham El Maaroufi,a Kamal Doghmi,a Issam Rharrassi,b Mohammed Mikdamea
on 17/10/2012 08:59:00 Haploidentical Stem Cell Transplantation ================================================================================Amr Ahmed Nassar
on 22/07/2012 10:04:00 The feasibility of stem cell transplantation across the major histocompatibility (MHC) barrier—as in haploidentical stem cell transplantation (SCT)—has been proved for some time in several studies. The main limitations include a higher graft failure rate, delayed immune reconstitution after transplantation with high rates of life-threatening infections, a higher incidence of post-transplant lymphoproliferative disease (PTLPD), and severe acute and chronic graft-versus-host disease (GVHD). In an attempt to reduce the transplant-related morbidity/mortality, several techniques had been evaluated involving conditioning regimen intensity, graft engineering, post-transplant cellular therapy and immunosuppression. This review will describe the current situation. It will also discuss initiatives and strategies to overcome the limitations associated with transplant across the MHC barrier. Lymphoproliferative disorders in pediatric liver allograft recipients: a review of 212 cases ================================================================================Hossein Khedmat,a Saeed Taherib
on 22/07/2012 09:57:00 BACKGROUND AND OBJECTIVES: Due to the limited incidence of posttransplant lymphoproliferative disorders (PTLD) in pediatric liver graft recipients, there is a scarcity of data on the characteristics of the disease in this population. We aimed to analyze the special features and behavior of PTLD arising after pediatric liver transplantation. DESIGN: A comprehensive search of the literature was conducted for the available data on PTLD in pediatric liver recipients pediatric PTLD through a search of Pubmed and Google Scholar using appropriate terms. METHODS: We sought data on liver recipients younger than 18 years of age at the time of transplantation. From 51 reports, 43 fulfilled the inclusion criteria. Overall 250 cases of PTLD (212 pediatric PTLD) were found from 43 reports. Data on pediatric patients was compared to adults. RESULTS: Pediatric PTLD lesions were more likely of the polymorphic type (P=.004) and polyclonal (when age cut-off was defined at 12 years; P=.023). Remission rates, metastasis frequency and organ involvements were not different between the groups (P>.1 for all). Survival analysis showed no disparity between pediatric PTLD and adult patients (P>.1); but when data was reanalyzed for patients surviving at least 4 months post diagnosis, the log rank test showed that pediatric patients have a superior outcome compared to adults (P=.045). CONCLUSIONS: Pediatric liver recipients developing PTLD have relatively better disease presentation and behavior than that in adults. Stomach involvement was also more frequently seen in patients younger than 12 years, and should be more intensively evaluated. Future studies with a prospective approach and larger population size are needed for confirming our results. Intersite validations of the pixel-wise method for liver R2* analysis in transfusion-dependent thalassemia patients: a more accessible and affordable diagnostic technology ================================================================================Pairash Saiviroonporn,a Vip Viprakasit,b Kleebsabai Sanpakit,b John C. Wood,c Rungroj Krittayaphongd
on 22/07/2012 09:51:00 BACKGROUND AND OBJECTIVES: MRI-R2* has been accepted as a clinical tool for monitoring iron overload in thalassemia patients, especially for monitoring liver iron concentration (LIC). The most optimal and practical method of analysis however, is still open to further investigations. Our objective was to investigate intra- and intersite observer variability of the pixel-wise method for liver R2* analysis in thalassemia patients using a mono-exponential with a constant offset model. PATIENTS AND METHODS: We performed 88 liver R2* measurements on 72 thalassemia major patients. A single breath-hold multi-echo gradient-echo sequence was acquired and analyzed at both the reference (REF) and local (LOC) sites. The analysis defined the region of interest in the whole liver parenchyma, excluding the great vessels, and were reported as median values. RESUlTS: The R2* values from the REF and LOC were statistically comparable for all comparisons. The intrasite and intersite observer variation were 0.75% (less than 0.9%) and 2.5%, respectively, both of which are comparable to previous reports, but substantially lower than conventional region-based approaches. CONCLUSION: The low variation of the R2* also yielded excellent variation in the tabulated hepatic iron content. However, caution is required when comparing the results to different implementation methods and appropriate evaluation and validation of methodology for any new scan site is essential before its clinical use. Bcl-2 protein expression in mucoepidermoid carcinoma of salivary glands: a single institution experience ================================================================================Omer Sefvan Janjua,a Sana Mehmood Qureshi,b Tariq Sarfraz Khan,b Wajiha Alamgirb
on 22/07/2012 09:43:00 BACKGROUND AND OBJECTIVES: Mucoepidermoid carcinoma is the most common salivary gland tumor with varying behavior among different histopathological grades. The objective of this study was to determine the expression of Bcl-2 protein in mucoepidermoid carcinoma (MEC) and to correlate with histological grades. METHODS: The records of 40 cases of MEC were collected from the histopathology department. Fresh slides were prepared and fresh diagnoses were made using the grading criteria for MEC. Immunohistochemical markers for Bcl-2 were applied and the results analyzed using the chi-square test. RESULTS: Of 40 cases, 20 were males and 20 were females. The range in age of the patients was 6 to 67 years mean (SD) was 42.6 (1.85) years. Twenty-two were low grade (55%), 11 high grade (27.5%) and 7 (17.5%) were intermediate grade MEC. Among these 40 cases, Bcl-2 expression was positive in 24 cases and negative in 16 cases. In 22 cases of low-grade MEC, 19 were positive while only 3 were negative. In high-grade tumors, all 11 cases were found to have a negative expression of Bcl-2 protein. In intermediate-grade MEC, 5 cases showed positive expression while only 2 cases showed negative expression. CONCLUSION: Bcl-2 protein expression showed positive expression in low-grade and negative expression in high-grade MEC. Intermediate grade showed more than 50% positive results for Bcl-2. Correlation between grades of MEC and expression of Bcl-2 is statistically significant and can be used for the depicting the prognosis of MEC along with other prognostic and clinico-pathological parameters. Clinicopathologic features and prognosis of triple-negative breast cancer in patients 40 years of age and younger in Saudi Arabia ================================================================================Omalkhair Abulkhair,a Jeelan S. Moghraby,b Motasim Badri,b Abdulmohsen Alkushic
on 22/07/2012 09:08:00 BACKGROUND AND OBJECTIVES: Triple-negative breast cancer (TNBC) has a poor prognosis and overall survival (OS) compared to other types of breast cancer tumors. However, there is to date no evidence that this is also the case in Saudi Arabia. DESIGN AND SETTING: Retrospective review of breast cancer patients who were treated from January 2001 to December 2008 (517 patients) at the King AbdulAziz Medical City, Riyadh, Saudi Arabia. PATIENTS AND METHODS: Patients were selected as TNBC if all three markers of estrogen receptor (ER), progesterone receptor (PR) and the human epidermal growth factor (HER2) tested by immunohistochemistry as negative. They were then age- and stage-matched, and compared with non-TNBC patients to examine differences, if any, in their clinicopathologic features, prognosis and OS. RESULTS: Twenty-six patients with a follow up time of at least three years were identified as TNBC. Thirty-three patients who were age- and stage-matched were selected as the non-TNBC controls. Clinicopathologic results illustrated significantly more grade 3 tumors (P=.02) and CK 5/6 expression (P40 years (P=.01), and when compared to the non-TNBC group (P=.04). CONCLUSION: The incidence of TNBC in our cohort is similar to what has been illustrated in previous studies in Western population. There was no significant difference in 3-year survival between TNBC and non-TNBC groups. However, the aggressiveness of this type of tumor and OS is significantly higher in younger patients aged ≤40 years, compared to those over 40 years of age. High-dose chemotherapy followed by stem cell transplantation in the management of retinoblastoma: a systematic review ================================================================================Imad Jaradat,a Rasmi Mubiden,a Ahmed Salem,a Fawzi Abdel-Rahman,b Iyad Al-Ahmad,b Abdelatief Almousaa
on 22/07/2012 08:59:00 BACKGROUND AND OBJECTIVES: In recent years, there has been an increasing role for stem cell transplantation in the management of retinoblastoma. The aim of this study was to systematically review the role high-dose chemotherapy followed by stem cell transplantation in the treatment of patients with metastatic or relapsed, trilateral or bilateral advanced retinoblastoma, and in patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension. DESIGN: Systematic literature review. METHODS: We performed an extensive PubMed database search on 25 February 2012 for studies describing the use of high-dose chemotherapy followed by stem cell transplantation in the management of patients with retinoblastoma. RESULTS: We located 15 studies that met the inclusion criteria and that included 101 patients. Following treatment for metastatic and relapsed disease, 44 of 77 patients (57.1%) were alive with no evidence of disease at the time of follow-up. However, a higher rate of local relapse developed in patients with CNS metastases (73.1%), which dropped to 47.1% in patients who received thiotepa. In patients with trilateral or bilateral advanced retinoblastoma, 5 of 7 (71.4%) with reported outcome data were alive with no evidence of disease at the time of follow-up. In patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension, 6 of 7 patients (85.7%) were alive with no evidence of disease at the time of follow-up. CONCLUSIONS: Durable tumor control is possible in patients with non-CNS metastases, trilateral or bilateral advanced retinoblastoma, and in patients with tumor at the surgical margin of the optic nerve and/or extrascleral extension. Patients with CNS metastases require thiotepa to improve tumor control. Myeloid sarcoma of the vulva post-bone marrow transplant presenting as isolated extramedullary relapse in a patient with acute myeloid leukemia ================================================================================Ahmed Nazer,a Ismail Al-Badawi,a Wahiba Chebbo,a Naeem Chaudhri,b Ghada El-Goharyb
on 22/07/2012 07:56:00 Myeloid sarcoma is a tumor of myoblasts or immature myeloid cells occurring in an extramedullary site. Myeloid sarcoma of the female genital tract as an isolated initial presentation or isolated relapse is very rare as evidenced from a literature review. We report a case of vulvar myeloid sarcoma presenting as isolated relapse of acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplant (HSCT). A 41-year-old female diagnosed with AML M5 achieved remission with chemotherapy and underwent allogeneic HSCT from an HLA-matched sibling donor. The post-transplant period was complicated with chronic graft-versus-host disease. At 10 months post-transplant, she presented with a vulvar mass of six weeks duration. Excisional biopsy of the vulvar mass confirmed the diagnosis of myeloid sarcoma as extramedullary relapse. Bone marrow biopsy was without evidence of leukemia. Involvement of the vulva, vaginal and adjacent cervical area only was confirmed. She received re-induction chemotherapy with clinical regression of both the vulvar, vaginal and the cervical masses; this was followed by radiation therapy to an extramedullary site. The correct diagnosis of myeloid sarcoma, particularly of an isolated mass in the genital area, is important because of its rarity and the need for appropriate institution of therapy. Post-dengue fever severe aplastic anemia: a rare association ================================================================================Mohammed Ramzan, Satya PrakashYadav, Anupam Sachdeva
on 22/07/2012 07:52:00 Dengue fever has rarely been reported as an etiology for aplastic anemia. An 8-year-old girl was admitted with fever, myalgia and petechiae. Dengue virus IgM antibodies were positive. She recovered completely, but her thrombocytopenia persisted. Six weeks later she became pancytopenic. A bone marrow aspirate and biopsy showed severe aplastic anemia. She was treated with antithymocytic immunoglobulin, methylprednisolone and cyclosporine. She became transfusion independent 6 months later. Dengue-virus induced aplastic anemia is a rare entity, but it must be identified early for better outcome. Immunosuppressive therapy can induce remission. Sponge eating: an unusual cause of severe anemia ================================================================================Illias Tazi, Hatim Nafil, Lahoucine Mahmal
on 22/07/2012 07:45:00 To the Editor: A 20-year-old Moroccan girl was referred to our department because of profound asthenia, palpitations, and dyspnea after very moderate effort. On physical Favorable outcome in a patient with vulvar mucormycosis during acute myeloid leukemia induction with medical management alone ================================================================================Karthik Udupa, Prasanth Ganesan, Urmila Majhi, Rejiv Rajendranath, Tenali Gnana Sagar
on 22/07/2012 07:22:00 To the Editor: Primary cutaneous mucormycosis is an uncommon infection caused by rare filamentous fungi Zygomycetes of the order Mucorales. A high degree of suspicion, Recommended Screening and Preventive Practices for Long-Term Survivors after Hematopoietic Cell Transplantation ================================================================================Navneet S. Majhail,ab J. Douglas Rizzo,c Stephanie J. Lee,d Mahmoud Aljurf,e Yoshiko Atsuta,f Carmem Bonfim,g Linda J. Burns,h Naeem Chaudhri,e Stella Davies,i Shinichiro Okamoto,j Adriana Seber,k Gerard Socie,l Jeff Szer,m Maria Teresa Van Lint,n John R Wingard,o Andre Tichellip for the Center for International Blood and Marrow Transplant Research (CIBMTR), American Society for Blood and Marrow Transplantation (ASBMT), European Group for Blood and Marrow Transplantation (EBMT), Asia-Pacific Blood and Marrow Transplantation Group (APBMT), Bone Marrow Transplant Society of Australia and New Zealand (BMTSANZ), East Mediterranean Blood and Marrow Transplantation Group (EMBMT) and Sociedade Brasileira de Transplante de Medula Ossea (SBTMO)
on 26/03/2012 07:10:00 Advances in hematopoietic cell transplantation (HCT) technology and supportive care techniques have led to improvements in long-term survival after HCT. Emerging indications for transplantation, introduction of newer graft sources (eg, umbilical cord blood) and transplantation of older patients using less intense conditioning regimens have also contributed to an increase in the number of HCT survivors. These survivors are at risk for developing late complications secondary to pre-, peri-, and posttransplantation exposures and risk factors. Guidelines for screening and preventive practices for HCT survivors were published in 2006. An international group of transplantation experts was convened in 2011 to review contemporary literature and update the recommendations while considering the changing practice of transplantation and international applicability of these guidelines. This review provides the updated recommendations for screening and preventive practices for pediatric and adult survivors of autologous and allogeneic HCT. Endometrial stromal sarcoma: prognostic factors and impact of adjuvant therapy in early stages ================================================================================Rachid Tanz, Tarik Mahfoud, Aziz Bazine, Rachid Aassab, Nourredine Benjaafar, Brahim El Khalil El Gueddari, Mohamed Ichou, Hassan Errihani
on 26/03/2012 07:23:00 BACKGROUND AND OBJECTIVES: Endometrial stromal sarcomas (ESS) are rare uterine neoplasms. Surgery remains the cornerstone of treatment for early stages and consists of an abdominal hysterectomy with bilateral salpingo-oopherectomy. Despite appropriate surgical treatment, relapse rates are high (18% to 45%) and the value of adjuvant therapies is not clear. We evaluated prognostic factors and the impact of adjuvant treatment on localized ESS (stages I and II). DESIGN AND SETTING: Retrospective, case-control study conducted at the National Institute of Oncology in Rabat, Morocco over 10 years from 2000 to 2009. PATIENTS AND METHODS: Twenty-one cases of localized ESS were included in the analysis. RESULTS: Standard surgery was performed in 71.4% of our patients. Myometrial invasion was noted in 57.1% of cases. Mitotic activity was considered high in five patients. Adjuvant treatment was given to 52.3% of patients: endocrine therapy in five patients and radiotherapy in six. Survival was significantly longer in the group of patients who underwent standard surgical treatment (P=.0007), in the absence of deep myometrial invasion (P=.0248) in cases with a low mitotic index (P A randomized trial of brief treatment of early-stage Hodgkin lymphoma: Is it effective? ================================================================================Rasha Hamdy Hamed, Abeer Hussein Anter, Ibrahim Aly Awad
on 26/03/2012 07:31:00 BACKGROUND AND OBJECTIVES: Whether it is possible to reduce the intensity of treatment in early (stage I or II) Hodgkin lymphoma with a favorable prognosis remains unclear. Therefore, we conducted this randomized trial, comparing two treatment groups consisting of a combination chemotherapy regimen of two different intensities followed by involved-field radiation therapy at two different dose levels. DESIGN AND SETTING: Prospective, randomized, in patients referred to the Department of Clinical Oncology and Nuclear Medicine. PATIENTS AND METHODS: Ninety-eight patients with histologically proven early-stage Hodgkin lymphoma with a favorable prognosis were enrolled in this study between January 2008 and June 2010. They were randomly assigned in one of two treatment arms: arm I received four cycles of ABVD (Adriamycin, belomycin, vinblastine, dacarbazine) followed by 30 Gy of involved-field radiation therapy; arm II received two cycles of ABVD followed by 20 Gy of involved-field radiation therapy. RESULTS: During the follow-up period, the 2-year relapse free survival rates were 96% and 95% in arm I and arm II, respectively(P=.8), while the 2-year overall survival rates were 98% and 95% in arm I and arm II, respectively (P=.16). Acute toxicity affected 54% of patients treated with four cycles of ABVD, who had grade III or IV toxicity, as compared with 30% of those receiving two cycles (P A phase 2 study of the combination of gemcitabine and cisplatin in patients with locally advanced or metastatic breast cancer previously treated with anthracyclines with/without taxanes ================================================================================Hanna Kohail,a Samir Shehata,b Omar Mansour,c Youssri Gouda,a Rabbab Gaafar,c Thoraya Abdel Hamid,c Saiid El Nowieam,a Ahmed Al Khodary,c Heba El Zawahry,c Ahmed Abdel Wareth,c Iman Abdel Halim,d Fouad Abou Taleb,e Emad Hamada,f Mohsen Barsoum,c Mohamed Abdullah,f Mohamed Meshreff
on 26/03/2012 07:41:00 BACKGROUND AND OBJECTIVES: Many patients with relapsed metastatic breast cancer are pre-treated with taxanes and anthracyclines, which are usually given in the neoadjuvant/adjuvant setting or as first-line treatment for metastatic disease. The primary objective of this study was to determine the overall response rate for combination treatment with gemcitabine and cisplatin in patients with locally advanced or metastatic breast cancer who had relapsed after receiving one adjuvant/neoadjuvant or first-line metastatic chemotherapy regimen containing an anthracycline with/without a taxane. Secondary endpoints included duration of response, time to progression, one-year survival probability, and toxicity. DESIGN AND SETTING: A single-arm, open-label, phase 2 study conducted at 17 investigative sites in Egypt. PATIENTS AND METHODS: Treatment consisted of gemcitabine (1250 mg/m2) on Days 1 and 8 and cisplatin (70 mg/m2) on Day 1 of each 21-day cycle. Treatment continued until disease progression or a maximum of 6 cycles. RESULTS: Of 144 patients all were evaluable for safety and 132 patients were evaluable for efficacy. The overall response rate was 33.3% and 45.5% of the patients with stable disease as their best response. The median time to progression was 5.1 months and the one-year survival probability was 73%. The most common grade 3/4 adverse events were nausea/vomiting (20.1%), neutropenia (19.4%), anemia (13.9%), asthenia (11.1%), diarrhea (9.7%), stomatitis (7.6%), leucopenia (7.6%), and thrombocytopenia (6.2%). Twelve (8.3%) patients had serious adverse events. CONCLUSIONS: The results of this study indicate that gemcitabine and cisplatin were active and generally well tolerated in pretreated patients with locally advanced or metastatic breast cancer. A simplified method for autologous stem cell transplantation in multiple myeloma ================================================================================Mohamed-Amine Bekadja,a Mohamed Brahimi,a Soufi Osmani,a Abdessamed Arabi,a Rachid Bouhass,a Nabil Yafour,a Badra Entasoltan,a Walid Rasheed,b Fadela Attafa
on 26/03/2012 08:06:00 BACKGROUND AND OBJECTIVES: We evaluated the efficacy and safety of non-cryopreserved storage of autologous hematopoietic stem cells with no post-transplant granulocyte colony-stimulating factor (G-CSF) support in adult patients undergoing autologous stem cell transplantation (ASCT) for multiple myeloma (MM). DESIGN AND SETTING: Retrospective review of patients undergoing ASCT from May 2009 to July 2011. PATIENTS AND METHODS: Autologous stem cell were mobilized using G-CSF. Leukapheresis to harvest stem cells was performed on day -2 and -1. The grafts were kept in a conventional blood bank refrigerator at 4°C until reinfusion on day 0. The conditioning regimen consisted of melphalan 200 mg/m2 in all patients. The postchemotherapy myeloablative phase was managed without growth factors. RESULTS: Between May 2009 to July 2011, 54 adults with MM were treated in our center in Oran. The median age at ASCT was 55 years (range, 35-65). There were 37 males and 17 females. The median harvested CD34+ cell count was 3.60×106/kg (range, 1.90 to 10.52). All patients had neutrophil engraftment on the median of day 10 (range, 6-17) and platelet transfusion independence on the median of day 13 (range 9-24). In the 47 evaluable patients the median post-transplant overall survival had not been reached; the estimated overall survival at 30 months was 93.8% (0.05%) , and the estimated disease-free survival at 27 months was 93.6% (0.05%). CONCLUSION: High-dose chemotherapy and ASCT using non-cryopreserved stem cells and no G-CSF support is safe and feasible in the treatment of MM under our work conditions in developing countries. Prevalence of HBV and HCV infection among multi-transfused Egyptian thalassemic patients ================================================================================Ahmed Kamel Mansour,a Rabab Mahfouz Aly,b Sohier Yahia Abdelrazek,a Doaa Mahmoud Elghannam,b Sherin Mohamed Abdelaziz,b Dina Abdalhalim Shahine,c Nadia Mohamed Elmenshawy,b Ahmad Mohamad Darwisha
on 26/03/2012 08:22:00 BACKGROUND AND OBJECTIVES: Though regular blood transfusion improves the overall survival of patients with b-thalassemia, it carries a definite risk of infection with blood-borne viruses. The present study was carried out to estimate the real frequency of hepatitis B virus (HBV) and hepatitis C virus (HCV) among Egyptian b-thalassemic patients, and determine the infection-associated risk factors in these patients. DESIGN AND SETTING: A prospective study conducted in a university hospital from January 2009 to January 2010. PATIENTS AND METHODS: Two hundred patients with b-thalassemia major were enrolled in this study. Using enzyme-linked immunoabsorbent assay (ELISA), their sera were tested for hepatitis B surface antigen (HBsAg), antibody to hepatitis C core antigen (anti-HBc), and HCV antibody (HCV Ab). The positive HCV Ab results were confirmed by second generation recombinant immunoblot assay (RIBA). RESULTS: The study sample consisted of 111 males and 89 females, with a median age of 13 years. Eighty-one (40.5%) patients were HCV Ab positive by ELISA and 39 (19.5) were anti-HCV positive By RIBA; 58 (29.0%) were HBsAg positive and 13 (6.5%) were anti-HBc positive. Older age, an increased number of transfusion units, and HBsAg seropositivity were significantly associated with a higher prevalence of HCV and HBV. CONCLUSION: The prevalence of HCV and HBV infections are very high among Egyptian b-thalassemic patients, which calls for a critical look into the prevailing transfusion practices and adoption of stricter donor selection criteria to decrease the incidence rate of both HCV and HBV infections effectively. Furthermore, there is a compressing need for the use of more specific and sensitive methods for HCV testing in Mansoura University Hospitals. Choroidal metastasis as the sole initial presentation of metastatic lung cancer ================================================================================Samer Salah,a Jamal Khader,b Yacoub Yousef,c Ahmed Salem,b Maysa Al-Hussaini,d Rafid Al-Asadye
on 27/03/2012 11:58:00 Choroidal metastasis as an initial presenting feature of metastatic lung cancer is exceedingly rare. External beam radiotherapy (EBRT) is an effective and widely accepted therapeutic modality. However, data addressing the effectiveness of other treatment strategies is limited. We present a patient with choroidal metastases secondary to lung cancer and review the relevant literature. A 25-year-old male presented with deterioration of vision. His evaluation revealed bilateral choroidal metastasis secondary to adenocarcinoma of the lung. Unfortunately, his vision continued to deteriorate despite treatment with EBRT and chemotherapy. Choroidal metastasis as an initial presentation of metastatic lung cancer is exceedingly rare, as only 30 cases have been reported. EBRT and systemic chemotherapy are effective therapeutic modalities. This case report could prove helpful to clinicians faced with a similar exceedingly rare scenario. Bone mineral density in ex-thalassemic pediatric patients ================================================================================Amir Ali Hamidieh,a Zohreh Hamidi,b Leila Nedaeifard,a Ramin Heshmat,b Kamran Alimoghaddam,a Bagher Larijani,b Ardeshir Ghavamzadeh,a Mohammad-Reza Mohajeri-Tehranib
on 27/03/2012 12:39:00 JAK2 mutation-negative essential thrombocythemia in a child presenting with cerebral venous thrombosis ================================================================================
Afaq Ahmad Khan,a Vijay Kumar,b Ish Anand,a Meet Kumar,c Prashant Sharma,d Manorama Bhargavaa
on 27/03/2012 12:07:00 Essential thrombocythemia (ET) is a rare myeloproliferative disorder occurring predominantly in the elderly population. Its occurence in the pediatric age group is even more rare. We report a 13-year-old girl who presented with isolated cerebral venous thrombosis and was diagnosed with essential thrombocythemia. Family screening for any thrombocytosis was 20 negative. With no secondary cause apparent for persistent thrombocytosis, we looked for the JAK2 mutation, but the result was negative. In contrast to linkage of JAK2 mutation positivity with increased thrombotic risk, our case showed that thrombosis can occur in the absence of JAK2 mutation in a case of essential thrombocythaemia. The indications for treatment and the best treatment of children with ET are currently not known, and guidelines for the management of children with ET are needed. Adult patients have near-normal life expectancy because of the low rate of leukemic conversion, but no child has been monitored long enough to assess prognosis. Incidence of gynecological malignancy among Saudi population ================================================================================Ahmed Nazer, Ismail A. Al-Badawi
on 27/03/2012 12:16:00 Higher infused CD34+ cell dose and overall survival in patients undergoing in vivo T-cell depleted, but not t-cell repleted, allogeneic peripheral blood hematopoietic cell transplantation ================================================================================Abraham Sebastian Kanate, Michael Craig, Aaron Cumpston, Ayman Saad, Gerry Hobbs, Sonia Leadmon, Pamela Bunner, Kathy Watkins, Deirdre Bulian, Laura Gibson, Jame Abraham, Scot C. Remick, Mehdi Hamadani
on 28/12/2011 07:54:00 BACKGROUND AND OBJECTIVES: Understanding the effect of cellular graft composition on allogeneic hematopoietic cell transplantation (AHCT) outcomes is an area of great interest. The objective of the study was to analyze the correlation between transplant-related outcomes and administered CD34+, CD3+, CD4+ and CD8+ cell doses in patients who had undergone peripheral blood, AHCT and received either in vivo T-cell depleted or T-cell replete allografts. DESIGN AND SETTING: Comparison of consecutive patients who underwent peripheral blood AHCT in our institution between January 2003 and December 2009. PATIENTS AND METHODS: The cohort of 149 patients was divided into two groups; non T-cell depleted (NTCD) (n=54) and T-cell depleted (TCD) (n=95). Study endpoints were overall survival (OS), progression free survival (PFS), engraftment kinetics (neutrophil and platelet recovery), incidence of acute graft versus host disease (acute GVHD), chronic GVHD, non relapse mortality (NRM) and disease relapse. RESULTS: Multivariate analysis showed that higher infused CD34+ cell dose improved OS (relative risk 0.58, 95% CI 0.34-0.98, P=.04), PFS (relative risk 0.59, 95% CI 0.35-1.00, P=.05) and NRM (relative risk 0.49, 95% CI 0.24-0.99, P=.048) in the TCD group. By multivariate analysis, there was no difference in engraftment, grades II-IV acute GVHD, extensive chronic GVHD and relapse in the two groups relative to the infused cell doses. There was a trend towards improved OS (relative risk 0.54, 95% CI 0.29-1.01, P=.05) with higher CD3+ cell dose in the TCD group. CONCLUSION: Our findings suggest that higher CD34+ cell dose imparts survival benefit only to in vivo TCD peripheral blood AHCT recipients. Preliminary experience with the use of bendamustine: a peculiar skin rash as the commonest side effect ================================================================================Baswantrao Malipatil, Prasanth Ganesan, Shirley Sundersingh, Tenali Gnana Sagar
on 28/12/2011 08:19:00 BACKGROUND AND OBJECTIVES: Bendamustine has been recently approved for the treatment of low-grade lymphoproliferative disorders. There is little data on the effectiveness or toxicity of this drug outside the trial setting. This is the first report on the use of bendamustine from the Indian subcontinent. SETTINGS AND DESIGN: Retrospective descriptive analysis of response and side effects of bendamustine in eight patients with chronic lymphocytic leukemia and eight patients with follicular lymphoma. METHODS: Data was collated from a review of case records. We examined any association between side effects and clinical parameters. RESULTS: The median age of patients was 52 years and three-quarters had received prior treatment with alkylators or fludarabine. Three different protocols of bendamustine were used (single agent, in combination with rituximab or in combination with vincristine and prednisolone). The overall response rate was 80% (47% complete response, 33% partial response, and 20% progressive disease). The drug was well tolerated with very few grade 3/4 toxicities. More than half the patients (9/16) developed a characteristic erythematous, papular skin rash that resolved after completion of chemotherapy. CONCLUSION: Bendamustine is a safe and useful addition to the drug arsenal against lymphoproliferative disorders. A peculiar skin rash was the commonest side effect noted in Indian patients treated with this drug. A population-based study of the epidemiology and clinical features of adults with acute myeloid leukemia in Algeria: report on behalf of the Algerian Acute Leukemia Study Group ================================================================================Mohamed Amine Bekadja,a Rose Marie Hamladji,b Meriem Belhani,c Fatima Zohra Ardjoun,d Mohand Tayeb Abad,e Hadj Touhami,f Hocine Ait-Ali,g Zahia Zouaoui,h Noureddine Sidimansour,i Selma Hamdi,j Fatiha Grifi,k Naima Mesli,l Mahdia Saidi,m Nemra Mehalhal,n Aissa Bachiri,o Rachid Bouhass,p Yousuf Mohamed Said,q Nouri Midounr
on 28/12/2011 08:35:00 BACKGROUND AND OBJECTIVES: In Algeria, the incidence of hematologic malignancies has been difficult to estimate for many years. Today, many hematological centers, including 14 university hospitals, have been developed in the entire north and have useful epidemiological data pertinent to acute myeloid leukemia (AML). We studied the incidence of AML and its subtypes, age distribution, geographic distribution and trends in the rate of diagnosis over the last 5 years in Algeria. Secondary goals were to study trends of referral of AML cases from various regions to specific centers to assess the needs for health infrastructure and change of current practices. DESIGN AND SETTING: Retrospective analysis of nationwide survey of all adult cases of AML (>16 years) diagnosed between 1 January 2006 and 31 December 2010. PATIENTS AND METHODS: A survey form was distributed to all departments of hematology at the 15 participating centers. RESULTS: The 1426 cases of AML diagnosed during the study period represented an annual incidence of 0.91/100 000 persons with a male to female (M/F) ratio of 1:16 and a median age of 45 years (range, 16-82 years). Nationally, 20% of cases AML were diagnosed in the whole western region of the country, 47% in the central and 33% in the east. There was a trend of continuous increase in the rate with age and in the rate of diagnosis over the last 5 years. The most common subtype was M2, followed by M4 and M5. CONCLUSION: An overall increase in the number of AML patients diagnosed nationwide over the last five years indicates a need for additional health care resources including curative and therapy-intense strategies, such as stem cell transplant facilities to optimize outcome. The relatively younger age of patients compared to the Western countries may be due to the demographic composition of our population. An approach to finding indications and contraindications for nephrectomy in post-transplant renal graft lymphomas: PTLD.Int Survey ================================================================================Aghil Gholipour-Shoiili,a Hani Gholipour-Shoiili,a Saeed Taherib
on 28/12/2011 09:01:00 BACKGROUND AND OBJECTIVES: Allograft involvement can occur in some renal transplant recipients who develop post-transplant lymphoproliferative disorders (PTLD). We aimed to find indications and/or contraindications for nephrectomy in renal allograft PTLD based on an outcome analysis of previous reports. DESIGN AND SETTING: A comprehensive search of Pubmed and Google scholar was performed to find reports of different treatment strategies addressing PTLD occurring within the allograft after renal transplantation. PATIENTS AND METHODS: Patients who underwent nephrectomy due to kidney allograft localization by PTLD were categorized as the case group, and renal recipients with kidney PTLD for whom nonsurgical treatment modalities were used served as controls. RESULTS: Survival analysis demonstrated that patients with renal allograft involvement who underwent allograft nephrectomy had a significantly better outcome compared to patients for whom a non-surgical approach was used (P=.03). In patients with disseminated PTLD, nephrectomy was not useful (P>.1). Patients with simultaneous kidney and lung complications by PTLD benefitted from nephrectomy. CONCLUSIONS: We found that patients with renal graft complication with disseminated PTLD do not benefit from nephrectomy, which can be considered the only contraindication. However, some particular PTLD co-localizations were not as likely to adversely affect the benefit of nephrectomy in these patients, and these can be considered indications for the procedure. Future multicenter studies are needed to confirm our results. Evidence for the use PET for radiation therapy planning in patients with cervical cancer: a systematic review ================================================================================Ahmed Salem,a Abdel-Fattah Salem,b Akram Al-Ibraheem,c Isam Lataifeh,d Abdelatief Almousa,a Imad Jaradata
on 28/12/2011 10:43:00 BACKGROUND AND OBJECTIVE: In recent years, the role of positron emission tomography (PET) in the staging and management of gynecological cancers has been increasing. The aim of this study was to systematically review the role of PET in radiotherapy planning and brachytherapy treatment optimization in patients with cervical cancer. DESIGN AND SETTING: Systematic literature review. METHODS: Systematic review of relevant literature addressing the utilization of PET and/or PET-computed tomography (CT) in external-beam radiotherapy planning and brachytherapy treatment optimization. We performed an extensive PubMed database search on 20 April 2011. Nineteen studies, including 759 patients, formed the basis of this systematic review. RESULTS: PET/ PET-CT is the most sensitive imaging modality for detecting nodal metastases in patients with cervical cancer and has been shown to impact external-beam radiotherapy planning by modifying the treatment field and customizing the radiation dose. This particularly applies to detection of previously uncovered para-aortic and inguinal nodal metastases. Furthermore, PET/ PET-CT guided intensity-modulated radiation therapy (IMRT) allows delivery of higher doses of radiation to the primary tumor, if brachytherapy is unsuitable, and to grossly involved nodal disease while minimizing treatment-related toxicity. PET/ PET-CT based brachytherapy optimization allows improved tumor-volume dose distribution and detailed 3D dosimetric evaluation of risk organs. Sequential PET/ PET-CT imaging performed during the course of brachytherapy form the basis of “adaptive” brachytherapy in cervical cancer. CONCLUSIONS: This review demonstrates the effectiveness of pretreatment PET/ PET-CT in cervical cancer patients treated by radiotherapy. Further prospective studies are required to define the group of patients who would benefit the most from this procedure. Durable complete response induced by paclitaxel-nimotuzumab-methotrexate chemotherapy in a patient with metastatic head and neck squamous cell carcinoma ================================================================================Leonardo Verduzco-Rodríguez, Elizabeth Haydee Aguirre-González, Haydee Cristina Verduzco-Aguirre
on 28/12/2011 11:04:00 A 61-year-old male patient with metastatic poorly differentiated squamous cell carcinoma of larynx to lymph nodes and lung was treated with a third-line chemotherapy scheme with paclitaxel, nimotu zumab and low-dose methotrexate receiving a total of 30 cycles. The response was complete until today and maintained at 16 months. Nimotuzumab is a humanized mono clonal antibody used to treat squamous cell carcinomas of the head and neck. This third-line chemotherapy combination with pacli taxel-nimotuzumab-methotrexate seems to be an active combination and needs further clinical trials to validate its use in heavily treated patients. Eight primary malignancies: case report and review of literature ================================================================================Ahmed Slem, Ramiz Abu-Hijlih, Fadwa Abdelrahman, Rim Turfa, Rula Amarin, Naim Farah, Maher Sughayer, Abdelatief Almousa, Jamal Khader
on 28/12/2011 11:17:00 The incidence of multiple primary malignancies has increased over the past years secondary to the long-term survival of cancer patients due to improvements in the early detection and adequate treatment of cancer. We present a patient with eight primary malignant tumors and review the relevant literature. Our patient was a 59-year-old female with Crohn disease with an otherwise non-contributory medical history. Risk factors for multiple primary tumors were not detected in our patient. At a follow-up of 108 months from the time of diagnosis of the first malignancy, our patient was still alive. Similar long-term survival has been reported in the literature. Due to the realistic potential for long-term survival, we recommend aggressive treatment of these patients. Primary anaplastic large cell lymphoma of trachea with subcutaneous emphysema and progressive dyspnea ================================================================================Kian Khodadad,a Shirin Karimi,b Mehrdad Arab,c Zahra Esfahani-Monfareda
on 28/12/2011 11:25:00 Primary anaplastic large cell lymphoma of the trachea is a rare tumor. Common complaints are dyspnea and cough that could mimic a partially refractory asthma in some cases. We report a 16-year-old female with an anaplastic large cell lymphoma (null cell type) in which tracheal involvement was presented with life-threatening airway obstruction and subcutaneous emphysema. After debulking the tumor by endobronchial curettage, the patient was treated with chemotherapy followed by local radiotherapy. She had no evidence of local or distant recurrence after 25 months. Primary anaplastic large cell lymphoma of the trachea is a rare life-threatening disease. Nevertheless, this condition has a good prognosis if diagnosed immediately and treated with chemotherapy and radiotherapy. Intensity-modulated radiotherapy in head and neck cancer: How safe is safe? ================================================================================Ahmed Salem, Abdulla Al-Rashdan, Imad Jaradat, Sameh A. Hashem, Abdelatief Almousa
on 28/12/2011 11:34:00 Mesothelioma in Cyprus: a case series (1997-2007) ================================================================================George Orphanos, Charis Charalambous, Ilias Vrezas, Stefanos N. Kales, Haris Haralambous, Nicos Katodritis, Gregoris Christodoulides, Michalis Maimaris, Elpidoforos S. Soteriades
on 28/12/2011 11:43:00 Long-term results of non-fludarabine versus fludarabine-based stem cell transplantation without total body irradiation in Fanconi anemia patients ================================================================================Amir Ali Hamidieh, Kamran Alimoghaddam, Mohammad Jahani, Seyyed Asadollah Mousavi, Masoud Iravani, Babak Bahar, Mahdi Jalili, Arash Jalali, Maryam Behfar, Ardeshir Ghavamzadeh
on 03/10/2011 08:07:00 BACKGROUND AND OBJECTIVE: Hematopoietic cell transplantation (HCT) is the only therapeutic modality capable of correcting the hematologic manifestations of Fanconi anemia (FA). The development of well-tolerated immunosuppressive conditioning regimens for FA patients undergoing HCT has proven to be a challenging task for hematologists. DESIGN AND SETTINGS: Retrospective, patients referred to the hematology, oncology and stem cell transplantation research center. PATIENTS AND METHODS: We analyzed the outcome of 53 FA patients who had undergone HCT between 1992 and 2010. The median age at transplantation was 9 years. Patients received transplants from an HLA-identical sibling (n=39) or matched relative (n=9) and one-antigen locus mismatched other relative/sibling (n=5). All of the patients underwent transplantation with fludarabine and non-fludarabine-based conditioning regimens. No radiation therapy was given. RESULTS: The median follow-up period for survivors was 13.5 months (range, 3 months-13.5 years). The 3-year overall survival (OS) was 60.6%. The 3-year OS for patients who did or did not receive fludarabine-based preparative regimens for the allograft was 36.4%, and 70%, respectively. However, there were no statistically significant differences in OS rates between these two groups (P=.112). Graft failure occurred in 4 patients (7.5%). All of these 4 patients had received fludarabine-based conditioning regimens. The incidence of acute GVHD after fludarabine-based regimens was 45% versus 79% in non-fludarabine-based regimens (P=.03). CONCLUSION: Despite the high incidence of acute GVHD (78.6%) in the non-fludarabine group, which resulted in the death of some patients, the OS rate was significantly better than in fludarabine recipients. Therefore, in spite of the fact that recent studies advocate the fludarabine-based conditioning regimens, we propose to conduct a multicenter, prospective study to evaluate the outcomes of regimens employed in FA patients. The challenges of managing glioblastoma multiforme in developing countries: a between cost and quality of care ================================================================================Ahmed Salem, Sameh A. Hashem, Abdulla Al-Rashdan, Najeeb Ezam, Ala’a Nour, Amer Alsharbaji, Maher Sughayer, Issa Mohamad, Maher Elyan, Ala’a Addas, Maysa Al-Hussaini, Abdelatif Almousa
on 03/10/2011 08:49:00 BACKGROUND AND OBJECTIVES: The management of glioblastoma multiforme (GBM) in developing countries is hindered by the paucity of clear protocols due in part to growing economic constraints and the lack of availability of expensive chemotherapeutic agents. We evaluated the deliverable treatment protocols and achievable outcomes for patients with GBM in a low-income country prior and subsequent to the worldwide adoption of temozolomide. DESIGN AND SETTING: Retrospective case series. PATIENTS AND METHODS: Charts of consecutive patients with a pathologic diagnosis of high-grade glioma diagnosed between January 2003 and December 2008 were retrospectively reviewed. RESULTS: We identified 146 adult patients, including 105 males and 41 females between 19 and 81 years of age (median age, 51 years), with histologically confirmed high-grade glioma. All patients underwent craniotomy. Eighty-two patients were treated with radiotherapy and temozolomide, of whom 42 patients received temozolomide concurrent with radiation followed by adjuvant temozolomide; 40 patients received irradiation followed sequentially by 6 cycles of temozolomide. In 40 patients irradiation was utilized as a single modality treatment adjuvant to surgery. The follow-up ranged from 1 to 56 months (median, 9.4 months). The median survival for the whole cohort was 10.2 months. The median survival for the radiotherapy-alone group was 5.3 months and for combined radiotherapy/temozolomide was 14.8 months. Survival was similar in both concurrent and sequential groups. Temozolomide conferred a statistically significant survival benefit of 9 months compared with standard therapeutic modalities. CONCLUSIONS: The results compare favorably to those reported in developed nations. Current management of GBM in developing countries should include maximal surgical resection followed by radiotherapy/temozolomide whenever medically and/or financially feasible. Outcomes comparable to those obtained within the context of randomized trials can be expected in low-income settings if healthcare delivery is carefully planned. Our results indicate that concurrent and sequential regimens are equally effective in these patients. Outcome of allogeneic stem cell transplantation with a conditioning regimen of busulfan, cyclophosphamide and low-dose etoposide for children with myelodysplastic syndrome ================================================================================Amal Al-Seraihy, Mouhab Ayas, Randa Al-Nounou, Hassan El-Solh, Ali Al-Ahmari, Abdullah Al-Jefri, Asim Belgaumi
on 03/10/2011 08:46:00 BACKGROUND AND OBJECTIVES: Allogeneic stem cell transplantation (SCT) offers the best chance of cure and long-term survival for children with myelodysplastic syndromes (MDS). DESIGN AND SETTING: Retrospective analysis of pediatric patients with primary MDS treated with allogeneic SCT at a single institution treated between January 1993 and December 2008. PATIENTS AND METHODS: Of 16 consecutive children who received allogeneic SCT for treatment of MDS in our center, 14 patients met the criteria of MDS according WHO I and II criteria. The median age was 4.8 years (range, 1-14 years) and 64% were male. The median time from diagnosis to transplant was 6 months. MDS stage was refractory cytopenia (RC) in 9, refractory anemia with excess blasts (RAEB) in 5. Monosomy 7 was present in 35% of the patients. The majority of patients (11/14) were conditioned with a busulfan-based myeloablative (MA) regimen with addition of low-dose of etoposide (30 mg/kg). All but one received a bone marrow graft. RESULTS: Nine patients achieved complete remission (CR), and seven remain alive. At a median follow-up of 3 years (range, 2-14 years) the OS and EFS was 57% (95%CI, 0.28-0.78). Cumulative EFS at 10 years was 43% (95% CI: 0.14–0.70). Relapse-related mortality was 21.4%; nonrelapse mortality (NRM) was 28.57%. All the survivors had etoposide in their conditioning regimen. Patients younger than 10 years had better survival (P=.001). CONCLUSION: Children with MDS achieve encouraging OS and EFS following allogeneic SCT. A busulfan-based regimen with a lower dose of etoposide is an effective and less toxic regimen. The outcomes are best in younger patients. Retinoblastoma in Jordan: An epidemiological study (2006-2010) ================================================================================Imad Jaradat, Yacoub A. Yousef, Mustafa Mehyar, Iyad Sultan, Samer Khurma, Khalil Al-Rawashded, Matt Wilson, Ibrahim Qaddoumi, Ahmed Salem, Ibrahim Alnawaiseh
on 03/10/2011 08:41:00 BACKGROUND AND OBJECTIVE: The epidemiological characteristics of retinoblastoma have been extensively studied in developed countries, however epidemiological data is scarce in the Middle East. We present a detailed epidemiological analysis of retinoblastoma in Jordan in an attempt to aid national and regional strategies for improved cancer surveillance and control. DESIGN AND SETTING: Retrospective review of retinoblastoma cases presenting to the sole and exclusive ocular oncology referral center in Jordan. PATIENTS AND METHODS: Forty children (59 eyes) presenting with clinically and/or histologically confirmed retinoblastomas were treated at King Hussein Cancer Center (Amman, Jordan) between January 2006 and December 2010. This case series included 28 boys and 12 girls. Data relating to age at diagnosis, laterality, gender, treatment modality and survival were recorded. RESULTS: The mean age-adjusted incidence of retinoblastoma in Jordan was 9.32 cases per million children per year for children aged 0-5 years. The male: female ratio was 2.3:1. Bilateral cases were encountered in 19 patients (47.5%) while 21 patients (52.5%) harbored unilateral retinoblastoma. At the time of follow-up, 38 patients (95%) were alive. Overall, 40 eyes (67.8%) were successfully preserved without the need for enucleation. CONCLUSIONS: The national epidemiological data gathered in this study indicates that the incidence of retinoblastoma in Jordan is similar to that reported in various countries of the world. Jordanian boys, however, are at significantly higher risk for developing retinoblastoma than age-matched girls. Furthermore, Jordanian patients are more likely to harbor bilateral retinoblastoma. Small lymphocytic lymphoma in a patient with CREST syndrome ================================================================================Basem M. William, Tracey Harbert, Apar K. Ganti, Philip J. Bierman
on 03/10/2011 08:39:00 We report a case of a 61-year-old man with a history of CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who presented for evaluation of thrombocytopenia. He had evident cervical adenopathy and lymph node biopsy showed small lymphocytic lymphoma (SLL) with evident systemic adenopathy and bone marrow involvement. The patient achieved a complete remission with FCR (fludarabine/cyclophosphamide/rituximab) chemotherapy. About 30 cases of lymphomas are reported in the literature in association with systemic sclerosis. To our knowledge, there are no reports of a small lymphocytic lymphoma (SLL) in association with limited cutaneous systemic sclerosis with classic features of the CREST syndrome. Association of hyperplastic polyposis syndrome, colorectal cancer and meningioma ================================================================================Mahvish Muzaffar,a John Irlam,b Iman Mohamedc
on 03/10/2011 08:35:00 Recent research has provided compelling evidence that a subset of hyperplastic polyps may be associated with a risk of colorectal cancer. Colorectal cancer with extracolonic manifestation is usually seen in a hereditary syndrome setting, but some association with meningioma has been reported. The association of colorectal cancer with hyperplastic polyposis and meningioma is extremely rare. This report in a 57-year-old female with no family history of colon cancer or polyps, could be the first case of hyperplastic polyposis syndrome, colorectal cancer and meningioma. Hyperplastic polyposis syndrome was diagnosed as per WHO criteria at the time of colon cancer diagnosis. Within 4 months of colon cancer diagnosis she developed seizures. Imaging of the brain revealed meningioma of the left cerebellopontine angle. The patient underwent surgery followed by chemotherapy. Mediastinal endodermal sinus tumor associated with fatal hemophagocytic syndrome ================================================================================Ikram Ul Haq Chaudhary,a Shoukat Ahmad Bojal,a Adel Attia,b Battal Al-Dossary,b Afra Qasem Al Dayel,c Samir Sami Amrc
on 03/10/2011 08:31:00 The association of endodermal sinus tumor, known also as yolk sac tumor, of the mediastinum with hemophagocytic syndrome is exceedingly rare with only a few cases on record. We report a 24-year-old male who had a large mediastinal germ cell tumor, proven to be an endodermal sinus tumor on biopsy. The patient developed pancytopenia and coagulopathy related to associated hemophagocytic syndrome, with a fatal outcome. A brief review of the relevant literature is presented as well. Acute massive splenic infarction with complete liquefaction of the spleen in sickle cell disease ================================================================================Salam Alkindi, Norman Machado, Pradeep Chopra, Mohammed Al-Huneini, Khalil AlFarsi, Anil Pathare
on 03/10/2011 08:28:00 Uterine sarcoma: a rare cause of uterine inversion ================================================================================Ezzeldin Korshid,a Ismail A. Al-Badawib
on 03/10/2011 08:23:00 Lead intoxication mimickiing a malignancy ================================================================================Reena Nair,a Vikas Ostwal,a Manju Sengar,a Amol Kothekar,a Jigeeshu V. Divatia,a Urmila Thatteb
on 03/10/2011 08:19:00