Hematogones With Lambda Light Chain Restriction in a 4-Year-Old Boy With Burkitt Lymphoma: A Potential Diagnostic Pitfall.

Hematogones are immature normal B cell precursors with a characteristic immunophenotype profile on flow cytometry that typically do not express surface immunoglobulin light chains. In this report, we describe a case in which the hematogones exhibit light chain restriction. Our patient was a 4-year-old boy with a complicated medical history involving treatment for a presumed bilateral Wilms tumor of the kidney that on later resection was diagnosed as Burkitt lymphoma. Flow cytometry analysis of his bone marrow revealed a small distinct population of cells expressing dim cluster of differentiation (CD)10, CD19, CD22, CD38, dim CD58, human leukocyte antigen-D related (HLA-DR), and dim CD45, which are characteristic of hematogones. These cells, however, demonstrated dim surface immunoglobulin lambda light-chain restriction. Molecular study results for immunoglobulin heavy and kappa light-chain gene rearrangements were negative. We present this case to raise awareness of the potential pitfalls of working up bone marrow for involvement by B cell lymphoproliferative disorder.

Successful treatment with modified CHOP-rituximab in pediatric AIDS-related advanced stage Burkitt lymphoma.

Burkitt lymphoma is the most common AIDS-related lymphoma (ARL) in childhood. The major issues in adult and pediatric ARL include identifying the optimal chemotherapy regimen and the concurrent treatment of both rituximab and highly active anti-retroviral therapy (HAART). We present a case of advanced stage Burkitt lymphoma in an 8-year-old female with acquired immunodeficiency syndrome (AIDS), who was successfully treated with a 3 month course of modified CHOP-R (cyclophosphamide, daunorubicin, vincristine, prednisone, and rituximab) and HAART therapy. The combination of rituximab and chemotherapy with HAART therapy may be well-tolerated and effective in HIV/AIDS patients with Burkitt lymphoma.