Updated: 3/26/2020

Thrombotic Thrombocytopenic Purpura (TTP)

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Snapshot
  • A 21-year-old woman with past medical history of SLE presents with fever, fatigue, and altered mental status. On physical exam, a petechial rash is seen on her upper extremities.  Her temperature is 103°F (39.4°C). Her labs are notable for Hgb 7.3 g/dL, platelets 17,000/mm3, and creatinine of 3.4 mg/dL.
Introduction
  • Thrombotic microangiopathy characterized by a pentad
    • microangiopathic hemolytic anemia
    • acute renal failure
    • thrombocytopenia
    • fever
    • neurologic abnormalities
  • Similar to HUS but with fever and neurologic symptoms
  • Epidemiology
    • female > male
    • most commonly in young adults
  • Pathogenesis
    • deficiency or inhibition of metalloproteinase ADAMTS13  
      • most commonly disabled by an autoantibody 
      • ADAMTS13 degrades vWF multimers
      • deficiency → large vWF multimers → increased platelet adhesion → platelet thrombosis 
      • important underlying risk factor, but requires a secondary trigger
    • triggers
      • drugs (ticlopidine, clopidogrel, and cyclosporine)
      • SLE
Presentation
  • Symptoms (recall pentad)
    • thrombocytopenia
      • easy bleeding
      • epistaxis
    • neurological symptoms
      • confusion
      • seizures
    • thrombosis
    • renal dysfunction (less so than HUS)
  • Physical exam
    • fever
    • pallor (from anemia)
    • purpura/petechiae
    • jaundice (from hemolysis)
    • splenomegaly
Evaluation
  • Complete blood count
    • anemia
    • thrombocytopenia
  • Peripheral blood smear
    • schistocytes (helmet cells)
  • Serum
    • ↑ LDH
    • ↑ creatinine
    • negative Coomb’s
  • ↑ bleeding time
  • normal PT/PTT
Differential Diagnosis
  • DIC
  • HUS
  • Sepsis
Treatment
  • Plasma exchange transfusion with fresh frozen plasma
  • Steroids
  • Splenectomy
  • Platelet transfusion contraindicated
    • platelet transfusion will worsen disease by feeding the platelet consumption
Prognosis, Prevention, and Complications
  • Prognosis
    • remission with plasma exchange in majority of patients
    • mortality rate 13-15%
  • Complications
    • permanent neurologic sequelae
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(M1.HE.13.203) A 27-year-old male presents to the emergency department with a one day history of progressive confusion and oliguria. The patient has had several episodes of epistaxis and bleeding gums since the onset of his illness. Past medical history is unremarkable and the patient does not take medications. Temperature is 39C, blood pressure is 100/62 mm Hg, pulse is 95/min, and respiratory rate is 18/min. Physical exam is notable for a purpuric rash in the lower extremities bilaterally. Peripheral blood smear is shown in Figure A. Which of the following is the most likely mechanism behind the patient's illness? Tested Concept

QID: 106270
FIGURES:
1

Defect in platelet aggregation

2%

(4/191)

2

Deficiency in degradation of von Willenbrand Factor (vWF) multimers

58%

(111/191)

3

Peripheral platelet destruction

26%

(50/191)

4

Defect in platelet-to-collagen adhesion

4%

(8/191)

5

Decreased levels of von Willenbrand Factor (vWF)

8%

(16/191)

M 1 C

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