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致命性传染性单核细胞增多症与病毒相关噬血细胞综合征。

Fatal infectious mononucleosis and virus-associated hemophagocytic syndrome.

作者信息

Mroczek E C, Weisenburger D D, Grierson H L, Markin R, Purtilo D T

出版信息

Arch Pathol Lab Med. 1987 Jun;111(6):530-5.

PMID:3579509
Abstract

A detailed clinicopathologic analysis of 52 cases of fatal infectious mononucleosis was performed. Fever, rash, generalized lymphadenopathy, hepatosplenomegaly, and blood cytopenias were the characteristic findings. Epstein-Barr virus infection was documented in 44 of the 52 patients. A triphasic process evolved in the blood and bone marrow of 43 patients. Early, the leukocyte count was elevated due to numerous atypical lymphoid cells, and the marrow was hyperplastic. Later, severe pancytopenia developed, and the marrow showed extensive infiltration by lymphoid cells with cellular necrosis and histiocytic hemophagocytosis. Terminally, the marrow showed massive necrosis with severe cellular depletion and marked histiocytic hemophagocytosis. The median survival time of the patients was six weeks. Opportunistic infections and/or acute hemorrhage were the major causes of death. We conclude that bone marrow damage secondary to an Epstein-Barr virus-associated hemophagocytic syndrome plays a major role in the death of patients with infectious mononucleosis.

摘要

对52例致命性传染性单核细胞增多症进行了详细的临床病理分析。发热、皮疹、全身淋巴结肿大、肝脾肿大和血细胞减少是其特征性表现。52例患者中有44例记录到爱泼斯坦-巴尔病毒感染。43例患者的血液和骨髓呈现出一个三相过程。早期,由于大量非典型淋巴细胞,白细胞计数升高,骨髓增生。随后,出现严重全血细胞减少,骨髓显示淋巴细胞广泛浸润伴细胞坏死和组织细胞吞噬血细胞现象。末期,骨髓出现大片坏死,细胞严重耗竭,组织细胞吞噬血细胞现象明显。患者的中位生存时间为六周。机会性感染和/或急性出血是主要死因。我们得出结论,爱泼斯坦-巴尔病毒相关噬血细胞综合征继发的骨髓损伤在传染性单核细胞增多症患者的死亡中起主要作用。

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