De novo double-hit B-cell precursor leukemia/lymphoma - an unusual presentation as peritoneal lymphomatosis

Abstract

Peritoneal lymphomatosis (PL) is a rare presentation of extranodal precursor leukemia/lymphoma. The presentation is often non-specific, leading to delayed diagnosis and treatment. In this case, though the preliminary diagnosis was established on ascitic fluid cytology, the disease progressed rapidly, leading to demise before initiating chemotherapy. Immunophenotyping and molecular studies, performed later, established a diagnosis of de novo B-cell precursor leukemia/ lymphoma with MYC, BCL2 rearrangements (Double-hit lymphoma). MYC, BCL2 rearrangements are rarely reported in precursor B-lymphoma/leukemia which carry dismal prognosis. In this report, we illustrate autopsy findings of PL in an elderly gentleman who presented with ascites for evaluation.

Clinical characteristics and treatment outcomes of children and adolescents with aggressive mature B-cell lymphoma: a single-center analysis

“…Currently, HGBL with MYC and BCL2 translocations accounts for approximately 10% of all lymphomas and occurs in older patients (median age, 60 yr; range, 6–82 yr) [ 24 ]. Although several studies reported such cases as a poor prognostic group in both pediatric and adult patients [ 25 , 26 ], the recommended treatment for double-hit and triple-hit lymphomas in the pediatric population is similar to that for other high-grade mature B-cell lymphomas. Among 51 patients with available immunohistochemistry or fluorescence in situ hybridization data in our study, 6 patients were suspicious for double-hit lymphoma with MYC and BCL6 rearrangements.…”