MYD88 mutation-positive indolent B-cell lymphoma with CNS involvement: Bing-Neel syndrome mimickers.
J Clin Exp Hematop
; 64(3): 252-260, 2024 Sep 28.
Article
en En
| MEDLINE
| ID: mdl-39218689
ABSTRACT
MYD88 p.L265P mutation occurs in over 90% of Waldenström's macroglobulinemia (WM), which is characterized by lymphoplasmacytic lymphoma (LPL) with monoclonal IgM. WM requires careful diagnosis due to overlapping features with other B-cell malignancies. Bing-Neel syndrome (BNS), a rare complication of WM, involves central nervous system (CNS) invasion. This report describes two cases of morphologically low-grade B-cell lymphoma in the bone marrow accompanied by the presence of a large B-cell lymphoma in the brain and a common MYD88 p.L265P mutation, which were eventually established as BNS mimickers. Although the two components in these cases showed the same identical light-chain restriction, different immunoglobulin heavy-chain rearrangement peaks indicated distinct lymphoma stem cells for CNS and bone marrow lesions. These clinical cases emphasize the challenges in diagnosing BNS. Based on the findings, biopsy is recommended for accurate identification of the clonal relationship and MYD88 mutation status.
Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Linfoma de Células B
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Macroglobulinemia de Waldenström
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Factor 88 de Diferenciación Mieloide
Idioma:
En
Revista:
J Clin Exp Hematop
/
J. clin. exp. hematop. (Online)
/
Journal of clinical and experimental hematopathology (Online)
Asunto de la revista:
HEMATOLOGIA
/
PATOLOGIA
Año:
2024
Tipo del documento:
Article