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Hematogones With Lambda Light Chain Restriction in a 4-Year-Old Boy With Burkitt Lymphoma: A Potential Diagnostic
Tesha Guillory1, Shiyong Li1, Daniel J Bergsagel2
1Department of Pathology, Emory University, Atlanta, GA.
Insights
Hematogones, immature B cell precursors, typically lack surface immunoglobulin light chains. This case highlights rare hematogones exhibiting lambda light chain restriction, posing diagnostic challenges for B cell lymphoproliferative disorders.
Area of Science:
- Hematology
- Immunology
- Pediatric Oncology
Background:
- Hematogones are normal B cell precursors identified by flow cytometry.
- They typically lack surface immunoglobulin light chain expression.
- Distinguishing reactive hematogones from malignant B cells is crucial in pediatric oncology.
Purpose of the Study:
- To report a rare case of hematogones with light chain restriction.
- To emphasize potential diagnostic pitfalls in bone marrow workups for B cell lymphoproliferative disorders.
- To increase awareness among clinicians regarding atypical hematogone immunophenotypes.
Main Methods:
- Flow cytometry analysis of bone marrow aspirate.
- Immunophenotyping using a panel of cell surface markers including CD10, CD19, CD22, CD38, CD58, HLA-DR, and CD45.
- Assessment of surface immunoglobulin light chain expression (kappa and lambda).
- Molecular studies for immunoglobulin gene rearrangements (heavy and kappa light chain).
Main Results:
- A distinct population of cells with hematogone immunophenotype (dim CD10, CD19, CD22, CD38, dim CD58, HLA-DR, dim CD45) was identified.
- These hematogones exhibited dim surface immunoglobulin lambda light-chain restriction.
- Molecular studies for immunoglobulin heavy and kappa light-chain gene rearrangements were negative.
Conclusions:
- Hematogones can rarely display light chain restriction, mimicking malignant B cell populations.
- This finding presents a diagnostic challenge in the evaluation of pediatric bone marrow samples.
- Awareness of this phenomenon is essential to avoid misdiagnosis of B cell lymphoproliferative disorders.
Abstract:
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.
