An Abnormal Haemoglobin (Lepore/Cyprus) Resembling Haemoglobin‐Lepore and its Interaction with Thalassaemia
An Abnormal Haemoglobin (Lepore/Cyprus) Resembling Haemoglobin‐Lepore and its Interaction with Thalassaemia
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类似于血红蛋白-Lepore的异常血红蛋白（Lepore/塞浦路斯）及其与地中海贫血的相互作用
DOI：
10.1111/j.1365-2141.1964.tb00691.x
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发表时间：
1964
影响因子：
6.5
通讯作者：
J. Gillespie
中科院分区：
文献类型：
--
作者：
G. Beaven;W. Gratzer;B. Stevens;E. Shooter;M. Ellis;J. White;J. Gillespie
在本文中，一项研究报告了一个大的土耳其-塞浦路斯家庭群体，延续了四代，其中一方的一个轻微的异常血红蛋白，LeporeiCyprus，发生在几个成员。典型的地中海贫血特征存在于家族的另一边，这两个特征在第四代婴儿中的相互作用引起了严重的临床病症，在14个月大时死亡。Hb-Lepore/ Cyprus与先前描述的轻微异常血红蛋白Hb-Lepore非常相似（Gerald和Diamond，1958 b）。其他两个轻微异常血红蛋白，也非常类似Hb-Lepore已被报道，其中之一与地中海贫血的相互作用已被描述。这四种次要血红蛋白的总体临床和生化表现都非常相似，无论是在性状条件下还是与地中海贫血结合。
IN this paper a study is reported of a large Turkish-Cypriot family group extending over four generations, on one side of which a minor abnormal haemoglobin, LeporeiCyprus, occurs in several members. The typical thalassaemia trait is present on the other side of the famdy, and the interaction of these two features in a fourth-generation infant gave rise to a serious clinical condition, terminating in death at 14 months of age. Hb-Lepore/ Cyprus closely resembles the previously described minor abnormal haemoglobin Hb-Lepore (Gerald and Diamond, 1958b). Two other minor abnormal haemoglobins which also closely resemble Hb-Lepore have since been reported and the interaction of one of these with thalassaemia has been described. The overall clinical and biochemical manifestations of these four minor haemoglobins are all very similar, both in the trait condition and in combination with thalassaemia.