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International Journal of Hematology

, Volume 78, Issue 4, pp 329–334 | Cite as

Dominant β-Thalassemia with Hemoglobin Hradec Kralove: Enhanced Hemolysis in the Spleen

  • Shouichi Ohga
  • Akihiko Nomura
  • Hidetoshi Takada
  • Junko Kato
  • Hiroshi Ideguchi
  • Yukio Hattori
  • Masahiro Suda
  • Sachiyo Suita
  • Toshiro Hara
Review Article

Abstract

We describe a 6-year-old girl and her mother with dominant β-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,\(G\underset{\raise0.3em\hbox{$\smash{\scriptscriptstyle-}$}}{C} C[Ala] \to G\underset{\raise0.3em\hbox{$\smash{\scriptscriptstyle-}$}}{A} C[Asp]\)). Splenectomy was effective for the amelioration of hemolysis. Of 7 reported patients with Hb variants at β-globin codon 115 (Hb Madrid and Hb HK), 5 underwent splenectomy. Because of the variable augmentation of extramedullary hemolysis in dominant β-thalassemias, genotyping is necessary for determining the clinical indication of splenectomy.

Key words

Dominant β-thalassemia Hemoglobin Hradec Kralove Red cell fragility Splenectomy 

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Copyright information

© The Japanese Society of Hematology 2003

Authors and Affiliations

  • Shouichi Ohga
    • 1
  • Akihiko Nomura
    • 1
  • Hidetoshi Takada
    • 1
  • Junko Kato
    • 4
  • Hiroshi Ideguchi
    • 4
  • Yukio Hattori
    • 5
  • Masahiro Suda
    • 3
  • Sachiyo Suita
    • 2
  • Toshiro Hara
    • 1
  1. 1.Department of PediatricsGraduate School of Medical Sciences, Kyushu UniversityFukuokaJapan
  2. 2.Department of Pediatric SurgeryGraduate School of Medical Sciences, Kyushu UniversityFukuoka
  3. 3.Division of Clinical LaboratoriesGraduate School of Medical Sciences, Kyushu UniversityFukuoka
  4. 4.Department of Laboratory MedicineFukuoka UniversityFukuoka
  5. 5.Division of Clinical Laboratory SciencesFaculty of Health Sciences, Yamaguchi University School of MedicineYamaguchiJapan

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