Macroglobulinemia, waldenstrom, susceptibility to, 1

Preferred Label : Macroglobulinemia, waldenstrom, susceptibility to, 1;

Symbol : WM1;

CISMeF acronym : WM1;

Type : Phenotype, molecular basis known;

Description : Waldenstrom macroglobulinemia (WM) is a malignant B-cell neoplasm characterized by lymphoplasmacytic infiltration of the bone marrow and hypersecretion of monoclonal immunoglobulin M (IgM) protein (review by Vijay and Gertz, 2007). The importance of genetic factors is suggested by the observation of familial clustering of WM (McMaster, 2003). Whereas WM is rare, an asymptomatic elevation of monoclonal IgM protein, termed 'IgM monoclonal gammopathy of undetermined significance' (IgM;

Inheritance : Somatic mutation;

Molecular basis : Susceptibility conferred by somatic mutation in the MYD88 innate immune signal transduction adaptor gene (MYD88, 602170.0004);

Neoplasia : Increased frequency of lymphoma, leukemia, and adenocarcinoma of lung;

Prefixed ID : #153600;

Details

Origin ID

153600;

UMLS CUI

C1835192;

Broader ORDO disease(s)
- Waldenström macroglobulinemia [ORDO Disease]
DO Cross reference
- Waldenstroem's macroglobulinemia [DO Concept]
Genes related to phenotype
- Myd88 innate immune signal transduction adaptor [OMIM gene]
HPO term(s)
- Impaired lymphocyte transformation with phytohemagglutinin [HPO term]
- Leukemia [HPO term]
- Lymphoma [HPO term]
- Monoclonal immunoglobulin M proteinemia [HPO term]
- Polyclonal elevation of IgM [HPO term]
- Polyneuropathy [HPO term]
- Somatic mutation [HPO term]
ORDO concept(s)
- Waldenström macroglobulinemia [ORDO Disease]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]

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