Updated: 8/4/2019

Waldenstrom Macroglobulinemia

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Snapshot
  • A 72-year-old man presents to his primary care physician with fatigue. His symptoms are associated with fevers, chills, and nightsweats. He lost 25 pounds over the course of 3 months unintentionally. Laboratory studies are remarkable for an anemia. Other serum studies demonstrates a monoclonal IgM gammopathy. Bone marrow biopsy demonstrates 12% bone marrow infiltration of small lymphocytes with plasma cell differentiation.
Introduction
  • Overview
    • B-cell lymphoproliferative neoplasm leading to excess production of monoclonal immunoglobulin (IgM) protein
      • secondary to lymphoplasmacytic cell infiltration in the bone marrow
  • Epidemiology
    • demographics
      • elderly patients
      • Caucasian males
  • Pathophysiology
    • excessive proliferation of IgM-producing plasmacytoid lymphocytes, leading to the overproduction of abnormal IgM antibodies
      • autoantibody attack against myelin-associated glycoprotein
        • results in neuropathy
      • IgM against the patient's red blood cells
        • Coombs-positive autoimmune cold hemolytic anemia
      • IgM deposition in the kidneys, gastrointesttinal tract, or skin
      • leads to serum hyperviscosity
        • secondary to IgM's pentameric configuration
      • precipitates in serum in cold temperatures
        • cryoglobulinemia
    • malignant B cells may infiltrate hematopoietic tissues leading to
      • cytopenias
      • lymphadenopathy
      • hepatomegaly
      • splenomegaly
  • Genetics
    • inheritance pattern
      • somatic mutation
    • mutations
      • MDY88 gene
  • Associated conditions
    • anemia
  • Prognosis
    • median survival is ~10 years from time of diagnosis
Presentation
  • Symptoms
    • fatigue
    • hyperviscosity syndrome
      • headache
      • vision impairment
      • epistaxis
      • leg cramps
Studies
  • Serum labs
    • IgM monoclonal gammopathy
      • any concentration
  • Invastive studies
    • bone marrow biopsy
      • indication
        • required to confirm the diagnosis
  • Histology
    • ≥ 10% infiltration by small lymphocytes with plasmacytoid or plasma cell differentiation
Differential
  • Multiple myeloma
    • differentiating factors
      • CRAB findings
        • hyperCalcemia
        • Renal insufficiency
        • Anemia
        • Bone lytic lesions
      • ≥ 10% clonal plasma cells
  • Monoclonal gammopathy of undetermined significance (MGUS)
    • differentiating factors
      • < 10% clonal plasma cells
      • absence of lytic lesions
      • serum M-protein is low
Treatment
  • There is no standard therapy 
  • Medical
    • rituximab with chemotherapy
      • indication
        • symptomatic Waldenstrom macroglobulinemia
Complications
  • Hyperviscosity
  • Peripheral neurology
  • Cryoglobulinemia
  • Amyloid amyloidosis
 

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