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再生障碍危象恢复期患者中与严重低磷血症相关的遗传性球形红细胞增多症

[Hereditary spherocytosis associated with severe hypophosphatemia in patients recovering from aplastic crisis].

作者信息

Sahara N, Tamashima S, Ihara M

机构信息

Department of Hematology, Seirei Hamamatsu General Hospital.

出版信息

Rinsho Ketsueki. 1998 May;39(5):386-91.

PMID:9637890
Abstract

This is a report about two cases of hereditary spherocytosis complicated by severe hypophosphatemia, while recovering from aplastic crisis. Case #1: A 31-year-old male, who had jaundice and splenomegaly since the age of 15 and who has a son diagnosed with hemolytic anemia, was admitted because of fever lymphadenopathy, and jaundice. A diagnosis of hereditary spherocytosis was made based on microspherocytes observed in his peripheral blood smear. After admission, the anemia became more serious for a few days and he was considered suffering from bone marrow aplastic crisis. His serum phosphorus level fell to 0.5 mg/dl on the second day, but it rapidly returned to normal as reticulocyte counts rose. Case #2: A 29-year-old male with known transient jaundice and splenomegaly suffered from fever, anemia and jaundice, but recovered two weeks later. Laboratory examination revealed positive human Parvovirus B19 (HPV-B19) DNA, anti-HPV-19 IgM and IgG-antibody. His serum phosphorus level fell to 1.2 mg/dl on the eighth day, but it rose in the same manner as seen in case #1. The fall in serum phosphorus is probably due to its shift to the erythroblasts during erythroid hyperplasia.

摘要

这是一篇关于两例遗传性球形红细胞增多症并发严重低磷血症的报告,两例均在再生障碍危象恢复期出现此情况。病例1:一名31岁男性,自15岁起出现黄疸和脾肿大,其儿子被诊断为溶血性贫血,该患者因发热、淋巴结病和黄疸入院。根据外周血涂片观察到的小球形红细胞诊断为遗传性球形红细胞增多症。入院后,贫血在数天内加重,考虑为骨髓再生障碍危象。入院第二天其血清磷水平降至0.5mg/dl,但随着网织红细胞计数升高迅速恢复正常。病例2:一名29岁男性,已知有短暂黄疸和脾肿大,出现发热、贫血和黄疸,但两周后康复。实验室检查显示人细小病毒B19(HPV - B19)DNA、抗HPV - 19 IgM和IgG抗体呈阳性。其血清磷水平在第八天降至1.2mg/dl,但与病例1情况相同随后上升。血清磷下降可能是由于在红系增生期间其转移至幼红细胞。

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