[新生儿期表现的家族性噬血细胞性淋巴组织细胞增生症]
[Familial erythrophagocytic lymphohistiocytosis with neonatal presentation].
作者信息
Arregui Sierra A, Arbelo López de Letona A, Quero Jiménez J, Pérez Rodríguez J, Alba Losada J, Acebedo Barberá A
出版信息
An Esp Pediatr. 1982 Aug;17(2):112-8.
PMID:7149477
Abstract
A neonatal case of familial erythrophagocytic lymphohistiocytosis is presented. Evolution was rapidly fatal under a course in which fever, hepatosplenomegaly, pancytopenia, severe coagulation alteration and jaundice were especially significant. Pathology findings after nine days of life, were all conclusive. Diagnostic difficulties of this uncommon entity in its' congenital form only once previously reported, are emphasized.
摘要
本文报告1例家族性噬血细胞性淋巴组织细胞增生症的新生儿病例。在病程中，发热、肝脾肿大、全血细胞减少、严重凝血功能改变和黄疸尤为显著，病情迅速发展并导致死亡。患儿出生9天后的病理检查结果明确。文中强调了这种先天性形式的罕见疾病的诊断困难，此前仅报道过1例。
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