Vnitr Lek 1997, 43(10):635-638

[Favorable effect of splenectomy on anemia in 3 siblings with type II congenital dyserythropoietic anemia (HEMPAS). (Ultrastructural changes in erythrocytes after splenectomy)].

L Chrobák, J Spacek

Oddĕlení klinické hematologie LF UK a FN, Hradec Králové.

Congenital dyserythropoietic anemia type II (CDA-type II) (HEMPAS) was reported in three siblings. CDA-type II was associated with marked hepatosplenomegaly and siderosis of both organs. All three sibling developed cholelithiasis with choledocholithiasis and obstructive jaundice in two of them. Anemia showing hemolytic component with sequestration of erythrocytes in the spleen was corrected after splenectomy without the need of blood transfusions during the follow up period of 20, 11 and 11 years. Ultrastructural investigation of the erythrocytes after splenectomy revealed increased number of erythrocytes showing the double membrane phenomenon.

Keywords: Adult; Anemia, Dyserythropoietic, Congenital, blood, ; Cholelithiasis, complications, ; Cholestasis, complications, ; Erythrocytes, ultrastructure, ; Follow-Up Studies; Humans; Male; Splenectomy

Published: October 1, 1997  Show citation