Burkitt's Lymphoma Presenting as Acute Cholecystitis and Vaginal Bleeding

Introduction Burkitt lymphoma is a highly aggressive B cell non-Hodgkin lymphoma characterized by the translocation t(8,14) and deregulation of the MYC gene on chromosome 8. The endemic form presents classically as an expanding mass in the jaw. Nonendemic form often presents with an abdominal mass. We present an interesting case of Burkitt's Lymphoma with atypical features. Case Description A thirty-five-year-old lady with no significant medical history presented to the hospital with a three week complaint of vaginal bleeding, lower abdominal pain/cramps associated with night sweats and chills. Gynecologic workup with an ultrasound revealed endometrial thickening followed by a hysteroscopic dilatation and curettage procedure. Labs significant for direct hyperbilirubinemia and elevated liver enzymes. MRCP showed gallbladder wall thickening but no biliary obstruction. A diagnosis of acalculous cholecystitis was considered and she underwent a laproscopic cholecystectomy and liver biopsy. Hospital course significant for rapidly worsening leucocytosis from 13000 to 81000 over 3-4 days. A peak WBC of 81,000 with peripheral blood blasts as high as 31% was noted. Peripheral smear exam revealed moderate sized immature wbc precursors/blasts with high nuclear-cytoplasmic ratio. Bone marrow aspirate and biopsy was done and pathology was positive for Burkitt's lymphoma/leukemia, t(8,14) was positive, involving bone marrow, gallbladder, liver and endometrium. Treatment was initiated with dexamethasone and nitrogen mustard as elevated bilirubin levels precluded standard treatment. Hyper-CVAD multi-agent chemotherapy was subsequently initiated along with intrathecal chemotherapy (cytarabine and methotrexate). Rituxan was included as CD20 positive cells. Discussion Burkitt's lymphoma is an aggressive neoplasms with a tumor doubling time of a few days. The usual presentation is with constitutional symptoms and adenopathy or a mass lesion. Hepatic parenchymal involvement is rare. Gallbladder involvement with endoluminal deposits is even rarer. Simultaneous hepatic, gallbladder, uterine, nodal and leukemic involvement at presentation is unique. Treatment of Burkitt is primarily with systemic chemotherapy and multi agent regimens effective in acute lymphoblastic leukemia and/or aggressive lymphomas have been used successfully in this condition with a complete response rate of 80%-90% with a long-term survival rate of approximately 60%.1355_A.tif Figure 1: Bone Marrow aspirate with Burkitt cells1355_B.tif Figure 2: Gall Bladder with Burkitt lymphoma1355_C.tif Figure 3: Liver with Burkitt lymphoma