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[一名遗传性球形红细胞增多症患者的细小病毒B19相关性噬血细胞综合征]

[Parvovirus B 19 associated hemophagocytic syndrome in a patient with hereditary sperocytosis].

作者信息

Hermann J, Steinbach D, Lengemann J, Zintl F

机构信息

Klinik für Kinder- und Jugendmedizin, Klinikum der Friedrich-Schiller-Universität, Jena.

出版信息

Klin Padiatr. 2003 Sep-Oct;215(5):270-4. doi: 10.1055/s-2003-42662.

Abstract

Infections with parvovirus B 19 can cause aplastic crises with a rapid decline of hemoglobin levels in patients with hereditary spherocytosis. Usually, the symptoms and signs of the actual infection are mild. We here report on an eight year old girl with hereditary spherocytosis who was admitted to hospital with high temperature, headache, impaired consciousness and a profound anemia (Hb 2.9 mmol/l). Since she also developed low leukocyte and platelet counts a hematological malignancy was suspected. The bone marrow aspirate showed only 1 % erythroblasts and macrophages with active hemophagocytosis. The serum ferritin was 1381,4 ng/ml. Both, serology and PCR revealed an active infection with parvovirus B 19. Coagulation analysis suggested a low degree of disseminated intravasal coagulation (low fibrinogen, high D-dimers). We diagnosed a parvovirus B 19 associated hemophagocytic syndrome. With only symptomatic treatment the patient's condition and laboratory findings improved during the course of a few days. In accordance with other reported cases, the prognosis of parvovirus B 19 associated hemophagocytic syndrome seems to be better than in hemophagocytic syndrome of other origin.

摘要

细小病毒B19感染可导致遗传性球形红细胞增多症患者发生再生障碍危象,血红蛋白水平迅速下降。通常,实际感染的症状和体征较轻。我们在此报告一名8岁遗传性球形红细胞增多症女孩,她因高热、头痛、意识障碍和严重贫血(血红蛋白2.9毫摩尔/升)入院。由于她还出现白细胞和血小板计数降低,怀疑患有血液系统恶性肿瘤。骨髓穿刺显示仅有1%的成红细胞和具有活跃噬血细胞作用的巨噬细胞。血清铁蛋白为1381.4纳克/毫升。血清学和聚合酶链反应均显示细小病毒B19活跃感染。凝血分析提示存在低度弥散性血管内凝血(纤维蛋白原降低,D-二聚体升高)。我们诊断为细小病毒B19相关噬血细胞综合征。仅采取对症治疗,患者的病情和实验室检查结果在数天内有所改善。与其他报道的病例一致,细小病毒B19相关噬血细胞综合征的预后似乎比其他原因引起的噬血细胞综合征更好。

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