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Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen.

Abstract：

:We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the increased fragility of the red cell membrane. On the other hand, the glycerol lysing time was prolonged, and results of the isopropanol test were weakly positive. Despite mimicking the features of hereditary spherocytosis, the results of the genetic analyses verified the second reported family with Hb HK (codon 115, GCC [Ala] --> GAC [Asp]). Splenectomy was effective for the amelioration of hemolysis. Of 7 reported patients with Hb variants at beta-globin codon 115 (Hb Madrid and Hb HK), 5 underwent splenectomy. Because of the variable augmentation of extramedullary hemolysis in dominant beta-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy.

journal_name

Int J Hematol

journal_title

International journal of hematology

authors

Ohga S,Nomura A,Takada H,Kato J,Ideguchi H,Hattori Y,Suda M,Suita S,Hara T

doi

10.1007/BF02983557

subject

Has Abstract

pub_date

2003-11-01 00:00:00

pages

329-34

issue

4

eissn

0925-5710

issn

1865-3774

journal_volume

78

pub_type

杂志文章

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