Congenital dyserythropoietic anemia type IV

Congenital dyserythropoietic anemia type IV (CDA IV) is a newly discovered form of CDA (see this term) characterized by ineffective erythropoiesis and hemolysis that leads to severe anemia at birth.

Anemia

A reduction in erythrocytes volume or hemoglobin concentration.


Total: 1

                      


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PMID (PMCID)
29300242
(6092092)
MALE Infant, Newborn
KLF1 E325K-associated Congenital Dyserythropoietic Anemia Type IV: Insights Into the Variable Clinical Severity.
Ravindranath Y, Johnson RM, Goyette G, Buck S, Gadgeel M, Gallagher PG.
J Pediatr Hematol Oncol. 2018;40(6):e405-e409.
We identified a child with KLF1-E325K congenital dyserythropoietic anemia type IV who experienced a severe clinical course, fetal anemia, hydrops fetalis, and postnatal transfusion dependence only partially responsive to splenectomy.