Hematology and Oncology Book

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Thrombotic Thrombocytopenic Purpura

Aka: Thrombotic Thrombocytopenic Purpura, 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: 4 to 11 cases per year per 1 million people per year in the United States
  3. Pathophysiology
    1. Etiology unclear
    2. Overlap with several Gastroenteritis related conditions
      1. Shiga-toxin Enterocolitis
      2. Hemolytic Uremic Syndrome
  4. Symptoms
    1. Headaches
    2. Paresthesias
  5. Signs: Classic Presentation
    1. Thrombocytopenic Purpura
    2. Microangiopathic Hemolytic Anemia
    3. Neurologic changes (Seizures, CVA)
    4. Fever
    5. Renal Disease
  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
      1. Children with TTP and Acute Renal Failure, bloody Diarrhea and Abdominal Pain
      2. Associated with Shiga toxin-producing Escherichia coli infection
  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. Admit all patients
    2. First line therapy
      1. Plasmapheresis (Fresh Frozen Plasma exxchange)
    3. Adjunctive measures
      1. Corticosteroids
      2. Aspirin or Dipyridamole
    4. 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 17:177-99

Purpura, Thrombotic Thrombocytopenic (C0034155)

Definition (NCI) An acute or subacute syndrome characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenic purpura, fever, renal abnormalities and neurologic abnormalities such as seizures, hemiplegia, and visual disturbances. Drugs and bacteria have been implicated as etiologic factors. The introduction of plasma exchange has significantly lowered the mortality rate. If untreated, the mortality rate is high.
Definition (NCI) A disorder characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenic purpura, fever, renal abnormalities and neurological abnormalities such as seizures, hemiplegia, and visual disturbances. It is an acute or subacute condition.
Definition (CHV) a kind of blood disorder that causes blood clots to form in blood vessels around the body
Definition (CHV) a kind of blood disorder that causes blood clots to form in blood vessels around the body
Definition (CHV) a kind of blood disorder that causes blood clots to form in blood vessels around the body
Definition (CHV) a kind of blood disorder that causes blood clots to form in blood vessels around the body
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.
Concepts Disease or Syndrome (T047)
MSH D011697
ICD10 M31.1
SnomedCT 360402008, 155443009, 195359000, 195358008, 78129009
English Thrombotic-thrombocytopenic purpura, Moschcowitz's syndrome, T.T.P. syndrome, Disease, Moschkowitz, Moschkowitz Disease, Purpura, Thrombotic Thrombocytopenic, Purpura, Thrombotic Thrombopenic, Thrombocytopenic Purpura, Thrombotic, Thrombopenic Purpura, Thrombotic, Thrombotic Thrombopenic Purpura, THROMBOTIC THROMBOCYTOPENIC PURPURA, MOSCHCOWITZ SYNDROME, PURPURA THROMBOPENIC THROMBOTIC, PURPURA, THROMBOCYTOPENIC, THROMBOTIC, PURPURA, THROMBOHEMOLYTIC, TTP - Thromb thrombocytop purp, Thrombot thrombocytopen purpur, MOSCHKOWITZ DIS, Moschcowitz Disease, Disease, Moschcowitz, thrombotic thrombocytopenic purpura (diagnosis), TTP (thrombotic thrombocytopenic purpura), thrombotic thrombocytopenic purpura, Thrombocytopenic purpura, thrombotic, Purpura thrombopaenic thrombotic, Purpura thrombopenic thrombotic, Moschowitz Disease, Thrombotic Thrombocytopenic Purpura, Purpuras, Thrombotic Thrombocytopenic, Thrombopenic Purpuras, Thrombotic, Purpuras, Thrombotic Thrombopenic, Thrombocytopenic Purpuras, Thrombotic, Thrombotic Thrombocytopenic Purpuras, Thrombotic Thrombopenic Purpuras, Purpura, Thrombotic Thrombocytopenic [Disease/Finding], ttp, thrombotic thrombocytopenic purpura (TTP), thrombotic thrombopenic purpura, moschcowitz's syndrome, TTP, Moschowitz's syndrome, Thrombotic thrombocytopenic purpura, TTP - Thrombotic thrombocytopenic purpura, Moschcowitz syndrome, Thrombotic thrombocytopenic purpura (disorder), Moschcowitz, purpura; thrombocytopenic, thrombotic, purpura; thrombotic thrombocytopenic, thrombocytopenic; purpura, thrombotic, thrombotic thrombocytopenic; purpura, Thrombotic thrombocytopenic purpura [Ambiguous]
Spanish PURPURA TROMBOTICA TROMBOPENICA, Purpura trombopénica trombótica, Thrombotic thrombocytopenic purpura, TTP, TTP - Thrombotic thrombocytopenic purpura, Moschcowitz syndrome, Moschowitz's syndrome, Thrombotic thrombocytopenic purpura [Ambiguous], PTT, púrpura trombocitopénica trombótica (trastorno), púrpura trombocitopénica trombótica, síndrome de Moschowitz, Púrpura trombocitopénica trombótica, Enfermedad de Moschkowitz, Púrpura Trombocitopénica Trombótica, Púrpura Trombótica Trombopénica, Purpura Trombocitopenica Trombotica, Purpura Trombotica Trombopenica
Dutch trombocytopenische trombotische purpura, purpura; trombocytopenisch, trombotisch, purpura; trombotisch trombocytopenisch, trombocytopenisch; purpura, trombotisch, trombotisch trombocytopenisch; purpura, trombotische trombocytopenische purpura, Microangiopathie, trombotische, Moschkowitz-ziekte, Purpura, trombotische trombocytopenische (TTP), TTP, Trombotische trombocytopenische purpura
French PTP, PURP THROMBOCYTOPENIQUE THROMBOT, PTT (Purpura Thrombotique Thrombocytopénique), Purpura thrombocytopénique thrombotique, Purpura thrombotique thrombocytopénique, Syndrome de Moschcowitz, Maladie de Moschcowitz, Micro-angiopathie thrombotique thrombocytopénique, Microangiopathie thrombotique thrombocytopénique, Purpura thrombopénique thrombotique
German Thrombozytopenische Purpura, thrombotisch, TTP, Purpura thrombopenisch thrombotisch, PURPURA THROMBOZYTOPENISCH THROMB, trombotisch-thrombozytopenische Purpura, Mikroangiopathie, thrombotische, Purpura, thrombotisch-thrombopenische, Purpura, thrombotisch-thrombozytopenische, Moschcowitz-Syndrom
Portuguese Púrpura trombopénica trombótica, PURPURA TROMBOCITOPENICA TROMBOTI, Púrpura Trombótica Trombocitopênica Familiar, Púrpura Trombótica Trombocitopênica Congênita, Púrpura Trombótica Trombocitopênica, Púrpura trombocitopénica trombótica, Doença de Moschkowitz, Púrpura Trombocitopênica Trombótica, Púrpura Trombótica Trombopênica
Japanese ＴＴＰ, ｹｯｾﾝｾｲｹｯｼｮｳﾊﾞﾝｹﾞﾝｼｮｳｾｲｼﾊﾝﾋﾞｮｳ, TTP, ベール･シフリン病, Moschkowitz病, 紫斑病-血栓性血小板減少性, モシュコビッツ症候群, 血栓性血小板減少性紫斑病, Moschowitz病, Moschowitz症候群, モシュコビッツ病, モスコウイッツ病, モスコウイッツ症候群
Swedish Purpura, trombotisk trombocytopen
Czech purpura trombotická trombocytopenická, TTP, Trombotická trombocytopenická purpura
Finnish Tromboottinen trombosytopeeninen purppura
Russian PURPURA TROMBOTICHESKAIA TROMBOTSITOPENICHESKAIA, MOSHKOVICHA BOLEZN', PURPURA TROMBOTICHESKAIA TROMBOPENICHESKAIA, МОШКОВИЧА БОЛЕЗНЬ, ПУРПУРА ТРОМБОТИЧЕСКАЯ ТРОМБОПЕНИЧЕСКАЯ, ПУРПУРА ТРОМБОТИЧЕСКАЯ ТРОМБОЦИТОПЕНИЧЕСКАЯ
Italian Sindrome di Moschkowitz, Porpora trombotica trombocitopenica
Polish Plamica małopłytkowa zakrzepowa, Zespół Moschcowitza
Hungarian TTP, thrombotikus thrombocytopeniás purpura, thrombotikus thrombopaenica purpura
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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