Premium
Burkitt lymphoma/leukaemia transformed from a precursor B cell: clinical and molecular aspects
Author(s) -
Hassan Rocio,
Felisbino Fabricio,
Stefanoff Claudio Gustavo,
Pires Virginia,
Klumb Claudete E.,
Dobbin Jane,
Seuánez Héctor N.,
Renault Ilana Zalcberg
Publication year - 2008
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2007.00992.x
Subject(s) - phenotype , lymphoma , biology , b cell , gene rearrangement , cancer research , pathogenesis , cell , immunology , antibody , gene , genetics
Burkitt lymphoma/leukaemia (BL/L) is a heterogeneous disease with respect to epidemiological patterns and cell origin. The occurrence of BL/L with an immature phenotype raises the question whether this phenotype might be a consequence of early B‐cell transformation or, alternatively, a secondary feature of transformed, mature B cells. It also poses important clinical questions regarding diagnosis and therapeutic procedures. Here we describe the case of a 4‐yr‐old child with BL/L and FAB L3 morphology, with phenotypic and genotypic characteristics of a CD10+ precursor B‐cell acute lymphoid leukaemia (ALL) associated with t(8;14)(q24;q32). Molecular analysis showed expression of RAG1 and RAG2 and an unmutated VDJCμ immunoglobulin rearrangement coinciding with a lack of AICDA expression, indicating an immature B‐cell origin. His clinical response suggested that FAB L3 ALL with MYC rearrangement and an aberrant precursor B‐cell phenotype is clinically similar to BL/L. Moreover, short, intensive chemotherapeutic protocols seemed to be beneficial. This case also allowed us to refine the description of cellular and molecular variants of BL/L regarding the cell origin and pathogenesis of this biologically heterogeneous disease.