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Waldenstrom's macroglobulinemia

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Wal·den·ström's macroglobulinemia (väldn-strmz, -strmz)
n.
Macroglobulinemia occurring in older persons, especially women, characterized by anemia, hyperglobulinemia, and the proliferation of cells resembling white blood cells or plasma cells in the bone marrow.

Waldenström's macroglobulinemia
Waldenstrom's disease Oncology A rare CA characterized by proliferation of B-cells that produce an IgM monoclonal protein there is no rationale for separating Pts based on monoclonal IgM levels; Pts may present with IgM > 3 g/dL and have a significant BM infiltration with lymphocytes and plasma cells, without major Sx, hepatosplenomegaly, lymphadenopathy or anemia; biologically, WM is an MGUS with IgM and BM involvement much greater than MGUS; asymptomatic Pts are classified as smoldering or asymptomatic macroglobulinemia Clinical Constitutional Sx–recurrent fever, night sweats, fatigue due to anemia, weight loss require therapy; anemia with a Hb ≤ 10 g/dL or platelets <100x109/L due to BM infiltration justifies treatment; hyperviscosity syndrome–HS, symptomatic sensorimotor peripheral neuropathy, systemic amyloidosis, renal failure–rare, or symptomatic cryoglobulinemia may also be indications for therapy Therapy Treatment should not be based on IgM levels because they correlate poorly with WM's clinical findings–ie, don't treat asymptomatic Pts; chlorambucil, cyclophosphamide, either alone or with other drugs; rituximab; symptomatic HS may warrant immediate plasmapheresis. See Bence-Jones protein, Hyperviscosity syndrome, Monoclonal gammopathy of undetermined significance.


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Despite apparent morphologic and immunophenotypic heterogeneity, Waldenstrom's macroglobulinemia is consistently composed of cells along a morphologic continuum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells.
We wish to alert the medical community that (1) conservative management of even extensive nasal skin loss should be considered when clinically acceptable, and (2) there may be an association between anti-CD2O antibody therapy for Waldenstrom's macroglobulinemia and skin necrosis.
head of CLL and Waldenstrom's Macroglobulinemia program at Weill Medical College of Cornell University in New York.
 
 
 
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