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[再生障碍危象发生时首次诊断的遗传性球形红细胞增多症;病例报告]

[Hereditary spherocytosis first diagnosed upon the development of aplastic crisis; a case report].

作者信息

Aonuma K, Morohashi F, Nakahata T, Komiyama A, Akabane T

出版信息

Rinsho Ketsueki. 1989 Feb;30(2):266-70.

PMID:2545942
Abstract

We report a Childhood case of hereditary spherocytosis (HS) first diagnosed upon the development of aplastic crisis. A 6-year-old boy presented with fever and anemia. Although there was neither icterus nor splenomegaly at first, mild icterus and splenomegaly gradually developed with improvement of anemia. The diagnosis of HS was made on the basis of the presence of numerous spherocytes on the peripheral smear, increased osmotic fragility and the auto-hemolysis test result. The severe anemia in the early course with a marked decrease in the bone marrow erythroid cells and the absence of icterus and splenomegaly indicate that it was due to aplastic crisis. In the virological study, anti-human parvovirus (HPV) antibody titers were increased: the values of anti-HPV IgM were high and those of anti-HPV IgG were suddenly elevated. We thus considered that this HS case developed aplastic crisis by HPV infection.

摘要

我们报告一例儿童遗传性球形红细胞增多症(HS),首次诊断是在再生障碍性危象发生时。一名6岁男孩出现发热和贫血症状。起初既无黄疸也无脾肿大,但随着贫血症状改善,逐渐出现轻度黄疸和脾肿大。根据外周血涂片上出现大量球形红细胞、渗透脆性增加以及自身溶血试验结果,诊断为HS。病程早期的严重贫血、骨髓红系细胞显著减少以及无黄疸和脾肿大表明这是由再生障碍性危象所致。在病毒学研究中,抗人细小病毒(HPV)抗体滴度升高:抗HPV IgM值高,抗HPV IgG值突然升高。因此,我们认为该HS病例是由HPV感染引发了再生障碍性危象。

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