Hemolysis

Paroxysmal Nocturnal Hemoglobinuria

search

Paroxysmal Nocturnal Hemoglobinuria, PNH

  • Pathophysiology
  1. Rare intrinsic RBC membrane defect
  2. Increased RBC sensitivity to complement damage
  • Symptoms and signs
  1. Chronic Anemia
  2. Abdominal Pain
  3. Retrosternal pain
  4. Lumbar back pain
  5. Superficial migratory Thrombophlebitis
  6. Nocturnal Hemoglobinuria
  • Labs
  1. Coombs Test
    1. Negative
  2. Peripheral Smear
    1. Reticulocytosis
    2. Hypochromasia (Chronic urinary iron loss)
  3. Urine
    1. Hemoglobinuria may be present
    2. Hemosiderin more often present
  4. Complete Blood Count
    1. Hemoglobin or Hematocrit consistent with Anemia
    2. Leukopenia
  5. Hemosiderin
    1. Leukocytes and Urine
  6. Ham Test Positive (Insensitive but highly specific)
    1. Increased Hemolysis in acid solution
  7. Sucrose Hemolysis Test (Sensitive but less specific)
    1. Increased Hemolysis in sucrose solution
  • Complications
  1. Acute Myelocytic Leukemia (5-10%)
  2. Thrombotic Complications
  3. Chronic Anemia
  • Management
  1. Anemia
    1. Folic Acid supplementation
    2. Iron Supplementation
    3. Androgen Trial for 2 months
      1. Fluoxymesterone 5-40 mg PO qd
      2. Oxymetholone 1-5 mg/kg/day PO
      3. Nandrolone decanoate 25-200 mg each week IM
  2. Hemolysis
    1. Prednisone
      1. Dose: 0.25 - 1.0 mg/kg/day (15-40 mg PO qd)
      2. Daily steroids not recommended unless critical need
      3. Alternate day therapy may be helpful
  3. Transfusion
    1. Most patients become transfusion dependent
    2. Blood Antibody development is common
      1. Washed RBCs or frozen deglycerolized RBCs
  4. Thrombotic Complications
    1. Use Heparin with caution!