[IgG-variant Bing-Neel syndrome diagnosed by detecting MYD88 L265P mutation in the cerebrospinal fluid cells].
Rinsho Ketsueki
; 62(10): 1493-1498, 2021.
Article
en Ja
| MEDLINE
| ID: mdl-34732622
ABSTRACT
Bing-Neel syndrome (BNS), which presents with a variety of neurological complications, is a rare manifestation of the lymphoplasmacytic lymphoma (LPL) and is characterized by the infiltration of LPL cells into the central nervous system. In this study, we report the case of a patient with BNS, which was confirmed by detecting MYD88 L265P mutation in the cerebrospinal fluid (CSF) cells. A 74-year-old patient was diagnosed with IgG-variant LPL. He achieved a very good partial response to the treatment with rituximab and bendamustine (RB) and was stable for over 5 years, when presenting a slowly progressive motor deficit in the lower limbs. It was difficult to confirm BNS from morphological analysis of the CSF cells. After detecting MYD88 L265P mutation in the CSF cells, he was subsequently diagnosed with BNS and treated with RB and intrathecal chemotherapy, resulting in rapid clinical improvement. With the onset of neurological manifestation during the clinical course of LPL, the detection of MYD88 L265P mutation in the CSF cells could be helpful for the diagnosis and management of BNS.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Macroglobulinemia de Waldenström
/
Factor 88 de Diferenciación Mieloide
Tipo de estudio:
Diagnostic_studies
Límite:
Aged
/
Humans
/
Male
Idioma:
Ja
Año:
2021
Tipo del documento:
Article