🩸 Chapter 514 · Hemolytic Anemias Secondary to Other Extracellular Factors

Nelson Textbook of Pediatrics 22nd Edition | Microangiopathic hemolytic anemias: TTP, HUS, DIC, Kasabach-Merritt syndrome. Schistocytes, thrombocytopenia. Thermal injury, Wilson disease (acute hemolytic anemia precedes neurologic symptoms), toxins/venoms (clostridial, snake, bee), liver disease (target cells, acanthocytes), renal disease, mechanical heart valves.

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📋 30 Clinical Scenarios — Extracellular Hemolytic Anemias (MAHA, TTP, HUS, Wilson)

📇 High‑Yield Review Cards (Extracellular Hemolysis)

🩺 Clinical Recognition: Microangiopathic Hemolytic Anemias & Other Extracellular Factors

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Microangiopathic Hemolytic Anemias

🔑 Key Principles — Nelson Chapter 514
• Schistocytes + thrombocytopenia = thrombotic microangiopathy (TMA).
• TTP (ADAMTS13 deficiency): Plasma exchange (PLEX) + corticosteroids. Rituximab for refractory. Congenital: plasma infusions.
• Typical HUS (Shiga toxin E. coli O157): Supportive care, avoid antibiotics, no PLEX.
• Atypical HUS (complement alternative pathway): Eculizumab (anti-C5).
• Wilson disease: Acute hemolytic anemia can precede liver/neurologic symptoms. Low ceruloplasmin, Kayser-Fleischer rings.
• Kasabach-Merritt syndrome: Giant hemangioma + consumptive coagulopathy + thrombocytopenia. Treat with sirolimus, vincristine, propranolol.

    ⚡ Reflex Prompts — Clinical Decisions in Extracellular Hemolysis

    📖 Summary: Hemolytic Anemias Secondary to Extracellular Factors — Nelson Ch 514