小儿Constant-Spring型血红蛋白H病31例报告

HEMOGLOBIN CONSTANT-SPRING TYPE IN HEMOGLOBIN H DISEASE IN CHILDREN: A report of 3l cases

血红蛋白Constant-Spring型血红蛋白H病(简称CS型HbH病)是一种特殊类型的血红蛋白H病,国外1971年首先由Paul于牙买加的Constant Spring地区华侨血红蛋白H病患者中发现,国内秦文斌等报告6例。

Thirty-one cases of Hemoglobin Con-stant-Spring type in Hemoglobin disease (CStype Hb H disease) Confirmed by hemoglobinanalysis from 1978 to 1987 are riported.Except 2 cases from Guangxi province, 29cases were from Guangdong. They were allof Han nationality. The male was 19 andfemale 12. In most cases the anemic symp-toms began at the age under 3 years old. Themajor clinical feature presented was hemoly-tic anemia. In the majority of cases, theirgrowth and development were retarded, andmild thalassemic faces could be seen. Hepat-omegaly and Splenomegaly were present in allof the patients. Hemoglobin electrophoresis: The concentra-tions of Hb H were 2.10-18.89% (mean9.97±4.79%). all were associated with HbCS (mean 3.13±1.20%), 27 cases were assoc-iated with Hb Bart's disease,1 case was ass-ociated with Hb K. Heinz bodies and Hb Hinclusion bodies were found in all cases. In 25families, 8 of the cases of CS type Hb H diseasewere found in siblings Splenectomy was per-formed in 3 cases of CS type Hb H disease.