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Updated: Dec 29 2021

Thrombotic Thrombocytopenic Purpura (TTP)

Images
https://upload.medbullets.com/topic/120242/images/purpura.jpg
https://upload.medbullets.com/topic/120242/images/ttp.jpg
  • Snapshot
    • A 24-year-old woman presents to the ED with acute-onset fatigue, fever, and blurry vision. On chart review, it is revealed that she was recently diagnosed with AIDS and was on multiple anti-retroviral medications. On physical exam, her skin is mildly jaundiced. She has multiple purpura over her extremities. Her peripheral smear reveals with schistocytes.
  • 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
    • African-Americans
  • ETIOLOGY
    • 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
          • damages brain and kidneys
          • important underlying risk factor, but requires a secondary trigger
      • triggers
        • drugs (quinine, ticlopidine, clopidogrel, and cyclosporine)
        • SLE
        • infections
        • AIDS
        • malignancies
  • Presentation
    • Symptoms (recall pentad)
      • thrombocytopenia
        • easy bleeding
        • epistaxis
      • neurological symptoms
        • confusion
        • seizures
        • headaches
      • nonspecific symptoms
        • weakness
        • vomiting
        • nausea
      • thrombosis
      • renal dysfunction (less so than HUS)
    • Physical exam
      • fever
      • pallor (from anemia)
      • purpura/petechiae
      • jaundice (from hemolysis)
      • splenomegaly (unlike ITP)
  • STUDIES
    • Complete blood count
      • anemia
      • thrombocytopenia
    • Peripheral blood smear
      • schistocytes (helmet cells)
    • Serum
      • ↑ LDH
      • ↑ creatinine
      • negative Coomb’s
    • Normal PT/PTT
    • ↑ bleeding time
  • Differential
    • TTP
    • HUS
    • DIC
    • Other causes of microangiopathic hemolytic anemia:
      • prosthetic valve hemolysis
      • metastatic adenocarcinoma
      • malignant HTN
      • vasculitis
        • Characteristics
        • DIC
        • ITP
        • TTP
        • HUS
        • Fever
        • No
        • No
        • Yes
        • Yes/No
        • Splenomegaly
        • No
        • No
        • Yes
        • Yes
        • Platelets
        • Low
        • Low
        • Low
        • Low
        • Bleeding time
        • PT
        • -
        • -
        • -
        • PTT
        • -
        • -
        • -
        • Shistocytes
        • Yes
        • No
        • Yes
        • Yes
        • Trauma
        • Yes
        • No
        • No
        • No
  • Treatment`
    • First-line therapy
      • plasma exchange transfusion with fresh frozen plasma
    • Anecdotal evidence
      • steroids
      • splenetcomy
        • if recurrent
    • Platelet transfusion contraindicated
      • platelet transfusion will worsen disease by feeding the platelet consumption
  • Complications
    • Permanent neurologic sequelae
    • Chronic kidney disease
      • especially with quinine-induced TTP
  • Prognosis
    • Remission with plasma exchange in majority of patients
    • Mortality rate 13-15%
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