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[家族性噬血细胞性淋巴组织细胞增生症。五例报告及核DNA细胞光度学研究(作者译)]

[Familial erythrophagocytic lymphohistiocytosis. Report of five cases with cytophotometric study of nuclear DNA (author's transl)].

作者信息

Ngendahayo P, Roels H, Benoit Y

出版信息

Ann Pathol. 1982;2(2):127-32.

PMID:7104066
Abstract

The authors report five cases of familial erythrophagocytic lymphohistiocytosis (F.E.L.H.). Two patients are brothers. One case is associated with a striking extramedullary hematopoiesis. A cytophotometric study of nuclear DNA in the histiocytes was performed in all cases. The histiocytes are diploid and there is no aneuploidy. These findings allow the authors to exclude the hypothesis of the tumoral nature of F.E.L.H. The data collected from the literature about the familial incidence of F.E.L.H. militate for an autosomal recessive transmission. The extramedullary hematopoiesis, which has not yet been reported in association with F.E.L.H., is probably due to the intense lymphohistiocytic infiltration of the bone marrow.

摘要

作者报告了5例家族性噬血细胞性淋巴组织细胞增生症(F.E.L.H.)。两名患者为兄弟。1例伴有显著的髓外造血。对所有病例的组织细胞进行了核DNA的细胞光度测定研究。组织细胞为二倍体,无非整倍体。这些发现使作者能够排除F.E.L.H.肿瘤性质的假说。从文献中收集到的关于F.E.L.H.家族发病率的数据支持常染色体隐性遗传。尚未见与F.E.L.H.相关的髓外造血报道,其可能是由于骨髓强烈的淋巴细胞组织细胞浸润所致。

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