遗传性球形红细胞增多症和细小病毒B19感染成人伴髓外造血的再生危象临床病例

A. Jercan, R. Gina, C. Dobrea, D. Coriu, A. Tatic
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摘要

遗传性球形红细胞增多症是一种因红细胞膜缺陷引起的遗传性溶血性贫血,以不同程度的慢性溶血、脾肿大和外周血膜微球形红细胞增多为特征。这些患者可能出现的并发症包括再生危机和髓外造血。在这篇文章中,我们提出了一个42岁的男性遗传性球形红细胞增多症的病例诊断在儿童时期(平均血红蛋白(Hb)值11-12克/分升),其表现为恶化贫血,发烧,寒战,骨和肌肉疼痛。演变为贫血加重(血红蛋白4.2 g/dl),网织红细胞数量减少(血红蛋白0.8%)和胆红素减少(间接胆红素2.7 g/dl)。细小病毒b19 DNA为100.000.000拷贝/ml。计算机断层扫描(CT)显示髓外造血区位于椎旁的下胸和肝脾肿大。感染发作是自限性的,并通过替代治疗得到改善。
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Clinical Case of Aplastic Crisis Associated with Extramedullary Hematopoiesis in an Adult With Hereditary Spherocytosis and Parvovirus B19 Infection
Abstract Hereditary spherocytosis is an inherited hemolytic anemia due to red cell membrane defects, characterised by chronic hemolysis with different severity degrees, splenomegaly and microspherocytosis on the peripheral blood film. Among the possible complications in these patients are aplastic crisis and extramedullary hematopoiesis. In this article we present the case of a 42 years old man with hereditary spherocytosis diagnosed during childhood (average haemoglobin (Hb) value of 11-12 g/dl), which presented with worsening anemia, fever, chills, bone and muscle pain. The evolution was with accentuation of anemia (Hb 4.2 g/dl), decease of reticulocyte number (Ret 0,8%) and bilirubin (indirect bilirubin 2.7 g/dl). ParvovirusB19 DNA was 100.000.000 copies/ml. A computer tomography (CT)scan was performed and showed extramedullary hematopoiesis areas situated paravertebraly in the inferior thorax and hepatosplenomegaly. The infectious episode was self-limited and improved with substitution treatment.
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