Sonakul D, Pacharee P, Thakerngpol K
Department of Pathology, Siriraj Hospital, Bangkok, Thailand.
Birth Defects Orig Artic Ser. 1988;23(5B):157-76.
Pathologic findings in six beta-thal major, 58 beta-thal Hb E, and 12 Hb H patients were presented. The causes of death were mainly infection in the first and second decades and cardiac failure in older patients. There was accelerated intramedullary and extramedullary erythropoiesis, with a few cases showing focal fibrosis, some with decreased cellularity and occasional atypia. Iron deposition mirrored that of thal patients in the European and American literature, apart from minimal cardiac siderosis, and the correlation between the amount of iron deposited and dysfunction of the particular organ was not always apparent. Patients with Hb H disease showed a less extensive iron deposition and organ pathology than those with the other two types of thalassemia.
本文展示了6例重型β地中海贫血、58例β地中海贫血-Hb E和12例Hb H病患者的病理结果。死亡原因主要是10至20岁患者的感染以及老年患者的心力衰竭。存在骨髓内和骨髓外红细胞生成加速，少数病例表现为局灶性纤维化，部分病例细胞数量减少且偶见异型性。铁沉积情况与欧美文献中地中海贫血患者的情况相似，仅有轻微的心脏铁沉着，铁沉积量与特定器官功能障碍之间的相关性并不总是明显。与其他两种类型的地中海贫血患者相比，Hb H病患者的铁沉积和器官病理变化范围较小。
