Updated: 12/27/2021

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
  • ETIOLOGY
    • 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
  • Epidemiology
    • Demographics
      • elderly patients
      • Caucasian males
  • 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
  • Prognosis
    • Median survival is ~10 years from time of diagnosis

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