Monosomy 7 in Childhood: a Myeloproliferative Disorder
Monosomy 7 in Childhood: a Myeloproliferative Disorder
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童年时期的 7 号单体：一种骨髓增殖性疾病
DOI：
10.1111/j.1365-2141.1981.tb07220.x
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发表时间：
1981
影响因子：
6.5
通讯作者：
Sylvia D. Lawler
中科院分区：
文献类型：
--
作者：
Colin A. Sieff;J. Chessells;B. A. M. Harvey;V. Pickthall;Sylvia D. Lawler
Summary. Between 1976 and 1979 a myeloproliferative disease associated with cells monosomic for chromosome number 7 in the bone marrow was seen in six boys aged 51/2 months to 8 years (median 10 months). Presenting features included hepatosplenomegaly (5/6), respiratory infections (4/6), pallor (2/6) and skin infections (1/6). Haematological features included a leucoerythroblastic anaemia with leucocytosis and thrombocytopenia, and a hyperplastic marrow with a slight excess of blasts. Fetal haemoglobin was normal in four patients and mildly raised in the other two. Neutrophil function tests showed defective chemotaxis with reduced killing, despite a normal NBT test. Cytogenetic analysis of the marrow showed a preponderance of cells with monosomy 7; the blood lymphocytes were cytogenetically normal.
