[IgG-variant Bing-Neel syndrome diagnosed by detecting MYD88 L265P mutation in the cerebrospinal fluid cells].
Rinsho Ketsueki
; 62(10): 1493-1498, 2021.
Article
in 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.
Key words
Full text:
1
Database:
MEDLINE
Main subject:
Waldenstrom Macroglobulinemia
/
Myeloid Differentiation Factor 88
Type of study:
Diagnostic_studies
Limits:
Aged
/
Humans
/
Male
Language:
Ja
Journal:
Rinsho Ketsueki
Year:
2021
Type:
Article