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Clinicopathological spectrum and therapeutic outcomes in Burkitt’s lymphoma: A ten year experience in a tertiary care setting

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Author(s): Farheen Mahar | Bushra Moiz | R. Sajid | Muhammad Owais Khan | S. N. Adil (1); M.Usman | Khurshid

Journal: International Journal of Collaborative Research on Internal Medicine & Public Health
ISSN 1840-4529

Volume: 3;
Issue: 3;
Start page: 205;
Date: 2011;
Original page

Keywords: Burkitt’s lymphoma | ICD

ABSTRACT
Objective: To evaluate clinico-pathological spectrum of Burkitt’s Lymphoma and to evaluate treatmentoutcomes along with survival studies.Background: Burkitt’s lymphoma (BL) is one of the most aggressive non-hodgkin’s lymphoma (NHL)characterized morphologically by monomorphic population of medium sized cells with round nuclei,basophilic cytoplasm and prominent cytoplasmic vacuolation. Cytogenetically there is a translocationbetween the long arm of chromosome 8 and either one of the following three translocations: Ig heavychain region on chromosome 14, the k light chain region on chromosome 2 or the lambda light chainlocus on chromosome 22. Cells of the BL have very high proliferative index with almost 100 %expression of Ki-67.There are three forms of BL:endemic, sporadic and associated with immunodeficiency.Treatment of BL is with multiagent chemotherapeutic agents and despite being an aggressive tumor theresults are excellent with long term remission and survival rates.There is no local published data from Pakistan /South Asia on Burkitt’s lymphoma. This study wasundertaken to determine the clinicopathologic spectrum of BL at a tertiary care center with survival dataand response to chemotherapy. In areas where BL is endemic ie. some parts of Africa and New Guinea,the incidence is around 15 per 100000 children aged 5 - 10 years.Methods: All patients with established diagnosis of Burkitt’s lymphoma who presented between 1998and 2008 to The Aga Khan University Hospital were reviewed retrospectively. The patients wereidentified using International classification of diseases ICD version 9.0. Medical records were evaluatedfor clinical and laboratory details using an in- house questionnaire. Statistics like multivariate analysisand survival studies (Kaplan Meier) were computed through SPSS version16.0 . The data includedclinicopathologic features, stage of the disease, laboratory parameters, treatment protocols, complicationsof chemotherapy, remission status and treatment outcomes.The different treatment protocols that were used in patients are given in table1. (Included the following:CALBG, CODOX, COPADM, Hyper CVAD and ICE. Among these the most commonly used protocolwas COPADM.)Results: During the study period, 34 patients were diagnosed with Burkitt’s lymphoma. There were 30(88 %) males and 4 (12 %) females. Median age was 9 ± years (range 3.5-67 years). Anemia,hepatomegaly, splenomegaly and lymphadenopathy were observed in 25 (73.5 %), (26.5%),6 (17.6 %),14 (41.2 %) patients. Central nervous system was involved in three patients and jaw swelling was noted inonly two patients at initial presentation. Eighty percent of the patients presented with abdominalsymptoms like pain, mass or lymphadenopathy. Initial hemogram showed mean hemoglobin was 9.4±G/dl (1.7-15) Normal Range (Men:13.8 to 18.0 g/dL, Women: 12.1 to 15.1 g/dL , Children: 11 to 16 g/dL), white blood cells 10.5 x109/L ± (2.6-33.1) Normal Range (4 - 10 x 109 cells per liter) and platelets 315x109/L ±(10-994) Normal Range (150 to 400 x 109/liter) Clinical presentation, baseline hematologicaland biochemical parameters are summarized as follows.Demographic and laboratory parameters at presentation in 37patients with Burkitt’s LymphomaResults of cytogenetics were available in 9 patients only with normal cytogenetics in 5 patients, t(8;14) inthree patients while one patient showed hyperploidy.Induction chemotherapy was given with COPADM in 9, CALBG in 5, Hyper CVAD in 2, CODOX inone patient respectively. Rest of the patients received other chemotherapy protocols comprising ofcyclophosphamide, prednisolone, vincristine and doxorubicin. CNS prophylaxis and growth factors wasgiven in all patients.Complete remission was attained in 13 (35.9 %) while partial remission was achieved in one patient(residual disease seen after four cycles of chemotherapy on CT scan) who completed treatment but wasthereafter lost to followup. Twelve patients died secondary to complications of chemotherapy. Out of theremaining 11 patients, 2 couldn’t receive chemotherapy while 9 patients didn’t complete chemotherapydue to financial constraints. The cumulative survival for all patients was 17.75 months. 2/13 whoachieved CR relapsed after 10 months and 3 years respectively. One patient was reinduced and achievedcomplete remission again whereas the other patient was not reinduced and was lost to follow up. At thetime of this review 10 patients were alive in complete remission and twelve patients had died. Rest of the15 patients were lost to follow up. The most common complications of chemotherapy were tumor lysis in18(46%) and Febrile neutropenia in almost 100 % of the patients. The causes of death included renalfailure in 5, cardiac arrest in 4 and septic shock in 3 patients respectively. The survival curve is asfollows.Conclusion: Burkitt’s lymphoma has a male predominance and is commonly seen in children. We foundabdominal presentation as the most common manifestation of Burkitt’s lymphoma. It is an uncommonmalignancy but carries a poor prognosis.
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