Adult Burkitt lymphoma- an Island between lymphomas and leukemias

Abstract

Background: Burkitt lymphoma is a rare, aggressive and rapidly fatal, B-cell non-Hodgkin’s lymphoma. It has an incidence of 0.4/100,000 age-adjusted to the USA standard population. Here we describe the case of a 77-year-old patient who presented with Burkitt lymphoma.Case: A 77-year-old male presented to his primary care physician with fatigue and listlessness and was referred to the hospital with a white blood cell count (WBC)-23.7 K/uL (neutrophils 37%, lymphocyte 11%, blasts 9%) and platelets-19 K/uL. During his stay in the hospital, repeat investigations revealed WBC-29.9 K/uL (neutrophils 22%, lymphocyte 27%, atypical lymphocytes 5%, blasts 20%) and platelets-10 K/uL with no evidence of mucosal bleeds, neck or abdominal masses or generalized lymphadenopathy. Bone marrow aspirate revealed the presence of MYC rearrangements (8q24) on flow cytometry and fluorescent in-situ hybridization (FISH), indicative but not typical of BL. He was transfused with platelets due to a rapidly deteriorating platelet count and episodes of epistaxis. He was discharged after four days with a plan of outpatient chemotherapy over a period of 4 months. An Ommaya reservoir was placed in the right ventricle for intrathecal chemotherapy. After four months of chemotherapy, computerized tomography of the chest, abdomen, and pelvis confirmed remission. A magnetic resonance imaging of the brain a month after completion of chemotherapy revealed metastatic lymphoma in the temporal, parietal and occipital lobes. He was discharged to hospice for palliative care.Conclusion: Unconventional presentations, as seen in our case of a leukemia-like picture in the absence of a bulky disease, are the quagmire that might delay aggressive management and result in poorer outcomes.

Sporadic Burkitt lymphoma presenting with peritoneal lymphomatosis

“… 1 , 4 Patients mostly present with abdominal pain, an abdominal mass, or ascites associated with fever, night sweats, and weight loss. 4 , 6 , 7 Peritoneal effusions in lymphomas have multiple mechanisms. These include impaired lymphatic drainage due to lymph node involvement, thoracic duct obstruction, venous obstruction, or serous involvement by the lymphoma.…”

“…Peritoneal lymphomatosis, especially if secondary to BL, has a rapid progression with a doubling rate of 24 h. 7 …”

“…Biopsy with morphology, immunophenotyping, and genetic analysis are used for the diagnosis. 7 Biopsy could be CT‐guided or surgical. On gross examination, BL presents as a hemorrhagic, necrotic mass with a fish‐flesh appearance.…”

“…The modified Ann Arbor is used for staging (adult burkitt lymphoma). Treatment for young adults is based on standard regimens such as CODOX‐M/IVAC (cyclophosphamide, vincristine, doxorubicin, and high‐dose methotrexate with ifosfamide, cytarabine, etoposide, and intrathecal methotrexate) 1,7 . Our patient received intravenous adriamycine, cyclophosphamide, vincristine associated with oral prednisone.…”

“…Treatment for young adults is based on standard regimens such as CODOX-M/ IVAC (cyclophosphamide, vincristine, doxorubicin, and high-dose methotrexate with ifosfamide, cytarabine, etoposide, and intrathecal methotrexate). 1,7 Our patient received intravenous adriamycine, cyclophosphamide, vincristine associated with oral prednisone. Aggressive chemotherapy regimens with central nervous system prophylaxis have good success rates with complete remission in 75-90% of cases and overall survival up to 50%-70%.…”