慢性细小病毒感染模拟骨髓增生异常综合征的儿童亚临床免疫缺陷。

H Hasle, G Kerndrup, B B Jacobsen, E D Heegaard, A Hornsleth, S T Lillevang
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引用次数: 0

摘要

目的:我们报告了一名患有亚临床免疫缺陷的儿童,慢性感染细小病毒导致全血细胞减少和骨髓形态异常,模拟骨髓增生异常综合征(MDS)。患者:一名8岁男孩,表现为严重贫血,中度血小板减少和粒细胞减少。患者表现为高免疫球蛋白M (IgM)免疫缺陷,但对感染的易感性未增加。骨髓细胞增多,伴有粒细胞增生异常和红细胞减少。结果:环孢素联合Ig治疗可使血红蛋白水平暂时恢复正常。几年来,人们一直认为患者患有MDS。由于缺乏特异性抗体和骨髓中没有巨大的原母细胞,细小病毒感染的诊断最初被拒绝。当聚合酶链反应技术可用时,在整个病程中检测到细小病毒DNA。结论:本病例报告扩展了我们对细小病毒感染临床谱的概念,并强调细小病毒必须作为MDS的鉴别诊断。尽管MDS患者血清学结果呈阴性,但我们建议进行细小病毒DNA检测,特别是当伴有免疫异常时。
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Chronic parvovirus infection mimicking myelodysplastic syndrome in a child with subclinical immunodeficiency.

Purpose: We present a report of a child with subclinical immunodeficiency who became chronically infected with parvovirus resulting in pancytopenia and morphologic abnormalities in the bone marrow mimicking myelodysplastic syndrome (MDS).

Patients: An 8-year-old boy presented with severe anemia, moderate thrombocytopenia and granulocytopenia. The patient showed hyper-immunoglobulin M (IgM) immunodeficiency but no increased susceptibility to infections. The bone marrow was hypercellular with dysplastic granulocytopoiesis and erythroblastopenia.

Results: Treatment with cyclosporine and i.v. Ig resulted in temporary normalization of the hemoglobin level. For several years it was assumed that the patient had MDS. A diagnosis of parvovirus infection was initially rejected due to the lack of specific antibodies and the absence of giant pronormoblasts in the bone marrow. When the polymerase chain reaction technique became available, parvovirus DNA was detected from the entire disease course.

Conclusions: This case report expands our conception of the clinical spectrum of parvovirus infection and emphasizes that parvovirus must be considered as a differential diagnosis in MDS. We recommend performing a parvovirus DNA test despite negative serologic findings in patients with MDS, especially when associated with immunologic abnormalities.

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