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VOLUME 2 | ISSUE 1 | FIRST QUARTER ISSUE | 2009

Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma

Asim Belgaumi,a Amani A. Al-Kofide,a Yasser Khafaga,b Nicey Joseph,a Rubina Jamil-Malik,a Khawar S. Siddiqui,a Rajeh S. Sabbaha

aDepartment of Pediatric Hematology/Oncology and the bKing Faisal Cancer Centre,  King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia

How to cite this article:

Belgaumi A, Al-Kofide AA, Khafaga Y, Joseph N, Jamil-Malik R, Siddiqui KS, Sabbaha RS. Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma. Hematol Oncol Stem Cell Ther 2009; 2(1): 278-284.

 

Abstract

BACKGROUND AND OBJECTIVES: While treatment outcomes for patients with Hodgkin lymphoma (HL) have improved remarkably, patients with disseminated disease still have a poorer outcome.  Stage IV HL is often reported with other ‘advanced stage’ categories, confusing the specific contribution of disease dissemination to the outcome. This single-institution report looks at characteristics and outcomes of this specific category.


PATIENTS AND METHODS: The medical records of pediatric HL patients (<14 years) from 1975 through 2003 were retrospectively reviewed and the data analyzed. 


RESULTS: Stage IV patients (n=67) had more poor-risk characteristics than patients in stages I-III (n=300) (B symptoms 86.6% vs. 19.3%, bulky disease 57.6% vs. 45.5% and mediastinal mass 77.6% vs. 29.7%; P<.001 for all characteristics). The liver was the most common extralymphatic site (in 51.5% of patients with stage IV disease.  Stage IV patients received chemotherapy (CT) alone (n=55) or combined modality therapy (CMT) (n=12). Fifty-four patients (80.6%) achieved complete remission, 2 (3%) partial remission, 10 (14.9%) had progressive disease and 1 was lost to follow up.  Overall survival was 79.4% and event-free survival (EFS) was 63.9% at 5 years.  There was a non-significant benefit for CMT (OS=91.7% v. 77.1%, P=.3; EFS=70.7% v. 62.7%, P=.3).  Ten of 12 relapsed and only 1 of 10 progressive disease patients were salvaged.  On multivariate analysis, failure to achieve complete remission with CT was associated with a poorer outcome.


CONCLUSION: Stage IV disease is associated with poor risk features and confers a worse outcome than stage I-III disease.  Achievement of complete remission with CT is an important prognostic feature. Slow responders may require novel and/or aggressive therapy to achieve complete remission.