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Updated: Dec 18 2021

Mechanical Destruction: Fragmentation Hemolysis

Images
https://upload.medbullets.com/topic/120237/images/thrombi_in_patient_with_thrombotic_thrombocytopenic_purpura_.jpg
https://upload.medbullets.com/topic/120237/images/differential_for_anemia_-_moises_d.jpg
  • Snapshot
    • A 30-year-old man presents to urgent care for a week duration of fatigue, weakness, and nausea. He endorsed several episodes of nonbloody diarrhea. He also reports a rash on his lower extremities. On physical exam, he is noted to have nonblanching red papules scattered on his lower extremities. Laboratory examination show thrombocytopenia as well as anemia. His creatinine is mildly elevated. A peripheral blood smear shows fragmented red blood cells. Concerned, his physician sends a specific laboratory test for ADAMTS13 activity. He is treated with plasma exchange therapy.
  • Introduction
    • Overview
      • fragmentation hemolysis is an example of a non-immune mediated extrinsic hemolytic anemia
        • due to mechanical destruction of the red blood cells (RBCs)
        • often associated with microvascular abnormalities, hence the term microangiopathic hemolytic anemia
  • Epidemiology
    • Risk factors and associated conditions
      • prosthetic heart valve
        • Waring blender syndrome
          • hemolytic anemia in the setting of prosthetic heart valves
      • HELLP syndrome
      • disseminated intravascular coagulopathy (DIC)
      • thrombotic thrombocytopenic purpura (TTP)
      • hemolytic uremic syndrome (HUS)
      • drug-induced thrombotic microangiopathy (DITMA)
        • quinine, oxymorphone, and ecstasy
      • malignancy
      • malignant hypertension
      • scleroderma renal crisis
      • Kasabach-Merritt phenomenon
        • rare consumptive coagulopathy associated with vascular tumors
  • ETIOLOGY
    • Pathogenesis
      • mechanism
        • RBCs are often sheared by a variety of factors, including fibrin strand formation or foreign body such as mechanical valves
  • Presentation
    • Symptoms
      • common symptoms
        • pallor
        • lightheadedness
        • jaundice
      • other signs of underlying conditions
        • petechiae or purpura
        • dark urine
        • bleeding
        • splenomegaly
  • Studies
    • Serum labs
      • hemolysis labs
        • increased indirect bilirubin
        • increased lactate dehydrogenase (LDH)
        • decreased haptoglobin
    • Peripheral blood smear
      • schistocytes, also known as helmet cells
        • small and irregular triangular or crescent-shaped cells, with lack of central pallor
  • Differential
    • Acute hemolytic reaction (ABO incompatibility)
      • key distinguishing factors
        • often occurs acutely during transfusion
        • presents with fevers, chills, and hypotension
  • Treatment
    • Medical
      • treat underlying medication condition
  • Complications
    • Renal failure
    • Shock
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