Journal article
Waldenström Macroglobulinemia: Clinical and Immunological Aspects, Natural History, Cell of Origin, and Emerging Mouse Models
ISRN hematology, Vol.2013, pp.1-25
2013
DOI: 10.1155/2013/815325
PMCID: PMC3782845
PMID: 24106612
Abstract
Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function mutation in the MYD88 adapter protein. This not only provides an intriguing link to new findings that natural effector IgM+IgD+memory B-cells are dependent on MYD88 signaling, but also supports the hypothesis that WM derives from primitive, innate-like B-cells, such as marginal zone and B1 B-cells. Following a brief review of the clinical aspects and natural history of WM, this review discusses the thorny issue of WM’s cell of origin in greater depth. Also included are emerging, genetically engineered mouse models of human WM that may enhance our understanding of the biologic and genetic underpinnings of the disease and facilitate the design and testing of new approaches to treat and prevent WM more effectively.
Details
- Title: Subtitle
- Waldenström Macroglobulinemia: Clinical and Immunological Aspects, Natural History, Cell of Origin, and Emerging Mouse Models
- Creators
- Siegfried Janz - Department of Pathology, University of Iowa Carver College of Medicine, 1030 ML, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- ISRN hematology, Vol.2013, pp.1-25
- DOI
- 10.1155/2013/815325
- PMID
- 24106612
- PMCID
- PMC3782845
- NLM abbreviation
- ISRN Hematol
- ISSN
- 2090-4428
- eISSN
- 2090-4428
- Grant note
- DOI: 10.13039/100014310, name: International Women's Media Foundation, award: 2010-1, R01CA151354
- Language
- English
- Date published
- 2013
- Academic Unit
- Pathology
- Record Identifier
- 9984083837402771
Metrics
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