Congenital Dyserythropoietic Anemia Type IV is a rare form of congenital dyserythropoietic anemia.
Chromosome: 19p13.13
Gene: KLF1 (Kruppel like factor 1)
Inheritance: autosomal dominant
Clinical features:
(1) normocytic anemia from birth, occasionally with hydrops fetalis
(2) dyserythropoiesis
(3) elevated hemoglobin F
(4) mild hemolysis with elevated serum LDH and decreased serum haptoglobin
(5) hepatomegaly and splenomegaly
(6) bi- or multi-nucleated erythroblasts
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Specialty: Hematology Oncology, Clinical Laboratory, Nutrition, Gastroenterology