Macroglobulinemia

Aka: Macroglobulinemia, Waldenstrom's Macroglobulinemia, Primary Macroglobulinemia

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II. Pathophysiology

  1. Lymphoproliferative Malignancy
    1. Proliferation of plasma cells and Lymphocytes
  2. Similar to Multiple Myeloma
  3. Produces a large monoclonal IgM Protein

III. Epidemiology

  1. Older men more frequently affected
  2. Hereditary component

IV. Symptoms

  1. Weakness
  2. Fatigue
  3. Oronasal bleeding
  4. Blurred Vision or Decreased Visual Acuity (33%)
  5. No Bone pain
    1. Contrast with Multiple Myeloma
  6. Hyperviscosity results in neurologic symptoms
    1. Dizziness or Vertigo
    2. Headache
    3. Nystagmus
    4. Paresthesias
    5. Decreased Level of Consciousness (Somnolence to Coma)
  7. Sudden Deafness
  8. Progressive spinal muscular atrophy

V. Signs

  1. Pallor
  2. Splenomegaly
  3. Hepatomegaly
  4. Peripheral Lymphadenopathy
  5. Retinal lesions
    1. Retinal Hemorrhage
    2. Retinal Exudate
    3. Venous congestion
  6. Peripheral Neuropathy
  7. Pulmonary Disease
    1. Infiltrates or Pleural Effusion

VI. Labs

  1. Complete Blood Count
    1. Normocytic normochromic Anemia
  2. Peripheral Smear
    1. Rouleau formation
  3. Erythrocyte Sedimentation Rate markedly elevated
  4. Bone Marrow Biopsy
    1. Hypocellular
    2. Extensive Infiltrations by lymphoid and plasma cells
    3. Increased Mast Cell numbers
  5. Immunoelectrophoresis
    1. Monoclonal IgM Protein >3 g/dl

VII. Differential Diagnosis

  1. Multiple Myeloma
  2. Chronic Lymphocytic Leukemia (CLL)
  3. Lymphoma

VIII. Management

  1. Suppression of Lymphocyte and Plasma Cell proliferation
    1. Chlorambucil
    2. Cyclophosphamide
    3. Combination chemotherapeutic regimens
  2. Hyperviscosity
    1. Plasmapheresis with cell separator

IX. Prognosis

  1. Five Year median survival after diagnosis

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