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Hematology: thalassemia, hemophilia, ITP in children

Pediatrics · MBBS

Study notes

A child — severe anemia, frontal bossing; thalassemia major; transfusions + chelation. Thalassemia major (beta — severe anemia — 6mo, hepatosplenomegaly, chipmunk facies: Hb electrophoresis — HbF high; treat — regular transfusions — Hb >9 + iron chelation — desferrioxamine/deferiprone; curative — BMT; prevent — carrier screening + prenatal): hemophilia (X-linked — factor VIII — A, IX — B: hemarthrosis, prolonged bleeding — aPTT prolonged; treat — factor replacement; desmopressin — mild A; prevent — avoid IM/NSAIDs). ITP (immune — well child — petechiae, low platelets: steroids/IVIG if bleeding; most remit spontaneously; chronic — rituximab/splenectomy). The chipmunk face is thalassemia — transfuse and chelate.

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