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[难治性贫血伴原始细胞增多(RAEB),在明显全血细胞减少之前HbF水平较高]

[Refractory anemia with excess of blasts (RAEB) with a high level of HbF that preceded apparent pancytopenia].

作者信息

Matsuda A, Kusumoto S, Ito K, Yokoo H, Maruyama T, Jinnai I, Harano T, Watanabe K, Hirashima K

机构信息

Department of Internal Medicine, Social Insurance Saitama Chuo Hospital, Japan.

出版信息

Rinsho Ketsueki. 1996 Jan;37(1):60-4.

PMID:8683869
Abstract

A 76-year-old male was kept under observation for idiopathic interstitial peumonitis in our hospital from August 1992. Laboratory data revealed a slightly high level of HbF (2.7%) but normal values of other hematological examination. The level of HbF increased slowly, and in April 1994, pancytopenia appeared for the first time. Bone marrow was normocellular with myelodysplasia and 9% blasts. Cytogenetic analysis revealed 46, XY, del (20) (q11;q13). He was diagnosed as having myelodysplastic syndrome (MDS), refractory anemia with excess of blasts. At diagnosis of MDS, the level of HbF was 20.0%. He developed acute myelocytic leukemia 3 months later. It has been reported that approximately 40% of patients with MDS have higher HbF levels than normal, which is considered to be functional abnormality of the MDS clone. It is suggested that the MDS clone had already increased in this patient at presentation, 32 months before pancytopenia appeared.

摘要

一名76岁男性自1992年8月起在我院因特发性间质性肺炎接受观察。实验室检查数据显示HbF水平略高(2.7%),但其他血液学检查结果正常。HbF水平缓慢上升,1994年4月首次出现全血细胞减少。骨髓细胞数量正常,伴有骨髓发育异常,原始细胞占9%。细胞遗传学分析显示核型为46, XY, del (20) (q11;q13)。他被诊断为骨髓增生异常综合征(MDS),即伴有过多原始细胞的难治性贫血。在诊断为MDS时,HbF水平为20.0%。3个月后他发展为急性髓细胞白血病。据报道,约40%的MDS患者HbF水平高于正常,这被认为是MDS克隆的功能异常。提示在该患者出现全血细胞减少前32个月就诊时,MDS克隆就已经增多。

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