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[家族性淋巴细胞组织细胞增多症。2例新生儿期确诊病例的解剖病理学研究]

[Familial lymphohistiocytosis. Anatomopathological study of 2 cases detected neonatally].

作者信息

Labbe A, Dechelotte P, Demeocq F, Lesec G, Gaulme J

出版信息

Arch Fr Pediatr. 1982 Oct;39(8):613-4.

PMID:7159160
Abstract

Two brothers presented with hemophagocytic reticulosis with neonatal onset. Early clinical and biological symptoms included pallor, hepatosplenomegaly and anemia, thrombocytopenia. Evolution was lethal in both cases, at 5 months and 13 days of age, respectively. Diagnosis was confirmed in both by the pathologic findings: diffuse lymphohistiocytic cellular proliferation with hemophagocytosis and atrophy of the lymphoid tissue. The diagnostic difficulties of this disease in the neonatal period are emphasized.

摘要

两兄弟患新生儿期起病的噬血细胞性网状细胞增多症。早期临床和生物学症状包括面色苍白、肝脾肿大、贫血、血小板减少。两例病情均致命,分别于5个月和13日龄时死亡。两例均经病理检查确诊:弥漫性淋巴细胞组织细胞增生伴噬血细胞现象及淋巴组织萎缩。文中强调了该病在新生儿期的诊断困难。

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