Hematology and Oncology Book

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Paroxysmal Nocturnal HemoglobinuriaAka: PNH

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  1. Pathophysiology
    1. Rare intrinsic RBC membrane defect
    2. Increased RBC sensitivity to complement damage
  2. Symptoms and signs
    1. Chronic Anemia
    2. Abdominal Pain
    3. Retrosternal pain
    4. Lumbar back pain
    5. Superficial migratory thrombophlebitis
    6. Nocturnal Hemoglobinuria
  3. 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
  4. Complications
    1. Acute Myelocytic Leukemia (5-10%)
    2. Thrombotic Complications
    3. Chronic Anemia
  5. 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!

Paroxysmal nocturnal hemoglobinuria (C0024790)

Definition (CSP)disorder characterized by intravascular hemolysis and hemoglobinuria; some cases occur on exposure to cold and are due to the presence of an autohemolysin in the serum; other cases are more marked during or immediately after sleep and are considered to be due to an acquired intracorpuscular defect.
ConceptsDisease or Syndrome (T047)
ICD9283.2
MSHD006457
EnglishMarchiafava - Micheli syndrome, Marchiafava Micheli disease, Marchiafava Micheli Syndrome, Marchiafava-Micheli Syndrome, Nocturnal Paroxysmal Hemoglobinuria, Parox. noct. haemoglobinuria, Parox. noct. hemoglobinuria, Paroxysmal noctural hemoglobinuria, Paroxysmal nocturnal haemoglobinuria, paroxysmal nocturnal hemoglobinuria, PNH, PNH - Paroxysmal nocturnal haemoglobinuria, PNH - Paroxysmal nocturnal hemoglobinuria
Spanishhemoglobinuria paroxistica nocturna, HPN, sindrome de Marchiafava - Micheli
Parent ConceptsHemoglobinuria (C0019048), Non-Neoplastic Hematologic and Lymphocytic Disorder (C1518374), Precancerous Conditions (C0032927), Anemia due to intrinsic red cell abnormality (C0272030), Ambiguous concept (C1274012)
SourcesCSP, DXP, MSH, MTH, MTHICD9, NCI, OMIM, PDQ, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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