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A. Characteristics navigator

  1. Low grade lymphoid malignancy which produces monoclonal IgM
    1. Related to multiple myeloma
    2. Tumor of plasma cells which have not undergone class switching
  2. Sometimes called lymphoplasmacytic lymphoma [8]
    1. Originally, this was presence of monoclonal cells without full syndrome
    2. Unclear if this is real distinction
  3. Usually affects older individuals
  4. The large size of IgM (~800 kD) leads to serum hyperviscosity and occlusive events

B. Presentation [2,3,4] navigator

  1. Fatigue is often very prominant
    1. Generalized immune dysfunction and chronic inflammatory state
    2. Anemia - bone marrow insufficiency and chronic disease features
  2. Lymphadenopathy
  3. Elevated Serum Viscosity (Hyperviscosity)
    1. Red cell sludging
    2. Occlussive events, especially retinal ("boxcar in vessels"), renal, minor strokes
    3. Peripheral neuropathies can also occur
  4. Plasmacytoma may develop
  5. Splenomegaly
  6. Direct infiltration of central nervous system (CNS) can occur (Bing-Neel Syndrome)

C. Laboratory navigator

  1. Ig fraction is increased, usually with significant hyperviscosity
    1. Hyperviscosity much more common with IgM than with IgA or IgG monoclonal protein
    2. Symptoms typically occur at viscosity units of 7.0 or higher
  2. Anemia [8]
    1. Usually of chronic disease type
    2. Bone marrow infiltration also contributes
    3. Splenomegaly can contribute [4]
    4. Iron deficiency often present
  3. Rouleau formation is often striking
  4. Erythrocyte sedimentation rate often highly elevated
  5. Lymphocytosis and/or monocytosis often found
  6. Bone marrow aspiration usually required for diagnosis and classification
  7. Mature B cell Phenotype
    1. Cells are CD19+, CD43+
    2. CD5, CD10 are variable; CD23 usually negative
  8. Abnormally low HDL cholesterol has been reported due to measurement errors [8]

D. Treatment [8] navigator

  1. Plasmapheresis - control serum viscosity [4]
  2. Glucocorticoids
  3. Alkylating agents
    1. Busulfan
    2. Melphalan
    3. Cyclophosphamide
    4. Chlorambucil
  4. Fludarabine (fluorinated adenine derivative) [5] See Cancer Chemotherapy II"
  5. 2-Chlorodeoxyadenosine [4,6]
  6. Progressive disease may be treated with stem cell transplantation
  7. Radiation may be used urgently or emergently for CNS symptoms [3]
  8. Bortezomib (Velcade®) has shown efficacy in refractory myeloma and in Waldenstrom's [7]

References navigator

  1. Barlogie B, Alexanian R, Jagannath S. 1992. JAMA. 268(20):2946 abstract
  2. Chad DA and Harris NL. 1999. NEJM. 340(21):1661 (Case Record)
  3. Wong ET and Louis DN. 2001. NEJM. 344(10):832
  4. Raje N and Ferry JA. 2001. NEJM. 345(9):682 (Case Record)
  5. Dimopoulos MA, O'Brien S, Kantarjian H, et al. 1993. Am J Med. 95(1):49 abstract
  6. Dimopoulos MA, Kantarjian H, Estey E, et al. 1993. Ann Intern Med. 118(3):195 abstract
  7. Richardson PG, Barlogie B, Berenson J, et al. 2003. NEJM. 348(26):2609 abstract
  8. Murali MR, Kratz A, Finberg KE. 2006. NEJM. 355(26):2772 (Case Record) abstract