Hematology and Oncology Book

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Thrombotic Thrombocytopenic PurpuraAka: TTP

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  1. See Also
    1. Thrombocytopenia
  2. Epidemiology
    1. Peak age 30 to 40 years old
    2. Slightly more common in women
    3. Rare: 3.7 cases per year per 1 million people
  3. Pathophysiology
    1. Etiology unclear
    2. Overlap with several Gastroenteritis related conditions
      1. Shiga-toxin Enterocolitis
      2. Hemolytic Uremic Syndrome
  4. Classic Presentation
    1. Thrombocytopenic Purpura
    2. Microangiopathic Hemolytic Anemia
    3. Neurologic changes (Seizures, CVA)
    4. Fever
    5. Renal Disease
  5. Symptoms
    1. Headaches
    2. Paresthesias
  6. Signs
    1. Fever (90% of cases)
    2. Skin
      1. Petechiae or Purpura
      2. Bleeding sites
      3. Jaundice
    3. Neurologic changes
      1. Altered Level of Consciousness
  7. Differential Diagnosis
    1. See Thrombocytopenia
    2. See Hemolytic Anemia
    3. Hemolytic Uremic Syndrome
  8. Labs
    1. Complete Blood Count
      1. Platelet Count <50,000
      2. Hemoglobin <10 g/dl
    2. Creatinine increased in severe cases
    3. Urinalysis
      1. Hematuria
      2. Proteinuria
    4. Peripheral Smear with Hemolysis signs
      1. Schistocytes
      2. RBC fragments
  9. Management
    1. First line therapy
      1. Plasmapheresis (fresh frozen plasma exxchange)
    2. Adjunctive measures
      1. Corticosteroids
      2. Aspirin or Dipyridamole
    3. Management in refractory cases
      1. Splenectomy
      2. Gammaglobulin
      3. Vincristine
  10. Prognosis
    1. Untreated: 80% mortality within 3 months
    2. Treatment with plasmapheresis: 17% mortality
  11. References
    1. Marx (2002) Rosen's Emergency Med, p. 1693
    2. Nabhan (2003) Hematol Oncol Clin North Am 17:177

Purpura, Thrombotic Thrombocytopenic (C0034155)

Definition (MSH)An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE.
Definition (NCI)The syndromes of microangiopathic hemolytic anemia, thrombocytopenia, and variable signs of organ impairment, due to platelet aggregation in the microcirculation.
ConceptsDisease or Syndrome (T047)
ICD9446.6, 446.6
MSHD011697
EnglishMoschcowitz Disease, Moschcowitz syndrome, Moschcowitz's syndrome, MOSCHKOWITZ DIS, Moschkowitz Disease, Moschowitz's syndrome, PURPURA THROMBOPENIC THROMBOTIC, THROMBOT MICROANGIOPATHY, Thrombotic microangiopathy, Thrombotic Thrombocytopenic Purpura, Thrombotic Thrombopenic Purpura, TTP, TTP - Thrombotic thrombocytopenic purpura
Spanishmicroangiopatia trombotica, PTT, purpura trombocitopenica trombotica, sindrome de Moschowitz
Parent ConceptsHemorrhage (C0019080), Hemorrhagic Disorders (C0019087), Decreased platelet count (C0392386), CLOTTING DECREASED (C0549625), Polyarteritis nodosa and allied conditions NOS (C0155757), Thrombosis (C0040053), Thrombophilia (C0398623), Thrombocytopenic purpura (C0857305), Blood Coagulation Disorders (C0005779), Purpura, Thrombotic Thrombocytopenic (C0034155), Microangiopathic hemolytic anemia (C0221021), Capillary thrombosis (C0265026), Disorder of hematopoietic structure (C1532235), Ambiguous concept (C1274012), Duplicate concept (C1274013)
SourcesCOSTAR, CST, DXP, ICD9CM, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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