🩸 Chapter 533 · Platelet and Blood Vessel Disorders

Nelson Textbook of Pediatrics 22nd Edition | Platelet disorders: Immune thrombocytopenia (ITP) — most common; TTP/HUS (thrombotic microangiopathies); inherited platelet disorders (Bernard-Soulier, Glanzmann thrombasthenia, Wiskott-Aldrich, TAR syndrome, platelet storage pool defects). Blood vessel disorders: Henoch-Schönlein purpura (IgA vasculitis), Ehlers-Danlos syndrome, scurvy. Management: ITP (observation, corticosteroids, IVIG, rituximab, TPO agonists, splenectomy), TTP (plasma exchange), HUS (supportive), inherited disorders (desmopressin, antifibrinolytics, platelet transfusions).

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📋 30 Clinical Scenarios — Platelet & Blood Vessel Disorders

📇 High‑Yield Review Cards (Platelet & Vessel Disorders)

🩺 Clinical Recognition: Platelet & Blood Vessel Disorders

Select a presentation for diagnostic clues and management.

📋 Stepwise Management of Platelet & Vessel Disorders

🔑 Key Principles — Nelson Chapter 533
• ITP (Immune thrombocytopenia): Most common cause of acute thrombocytopenia in children. Usually post-viral. Management: observation (if mild, no bleeding), corticosteroids (prednisone 2-4 mg/kg/day), IVIG (0.8-1 g/kg), anti-D (Rh-positive, non-splenectomized). TPO agonists (eltrombopag, romiplostim) for chronic ITP.
• TTP (Thrombotic thrombocytopenic purpura): ADAMTS13 deficiency → microangiopathic hemolytic anemia, thrombocytopenia, fever, renal failure, neurologic symptoms. Treatment: plasma exchange (PLEX) + corticosteroids.
• HUS (Hemolytic-uremic syndrome): Shiga toxin-producing E. coli (typical) → supportive care; atypical HUS (complement) → eculizumab.
• Bernard-Soulier syndrome: GPIb/IX/V deficiency → giant platelets, thrombocytopenia, bleeding. Treatment: platelet transfusion.
• Glanzmann thrombasthenia: GPIIb/IIIa deficiency → normal platelet count, severe mucocutaneous bleeding. Treatment: rFVIIa, platelet transfusion.
• Wiskott-Aldrich syndrome: X-linked, microthrombocytopenia, eczema, immunodeficiency. Treatment: HSCT.

    ⚡ Reflex Prompts — Clinical Decisions in Platelet & Vessel Disorders

    📖 Summary: Platelet & Blood Vessel Disorders — Nelson Ch 533