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白血病转化中骨髓增生异常综合征相关溶血性贫血的消失。

Disappearing myelodysplastic syndrome-associated hemolytic anemia in leukemic transformation.

机构信息

Department of Internal Medicine, Hyogo Prefectural Tsukaguchi Hospital, Hyogo, Japan.

出版信息

Int Arch Allergy Immunol. 2010;152(4):407-12. doi: 10.1159/000288294. Epub 2010 Mar 3.

DOI:10.1159/000288294
PMID:20197683
Abstract

BACKGROUND

Here we report 2 rare cases of acute myeloid leukemia (AML) complicated with hemolytic anemia limited to the myelodysplastic syndrome (MDS) stage, and disappearing in leukemic transformation.

METHODS/RESULTS: A 66-year-old man with MDS-RAEB-2 was admitted to hospital for severe anemia with increased reticulocyte counts. Hemolytic anemia was suspected, and it was ameliorated by methylprednisolone pulse therapy. Although anemia grew worse when steroids were tapered off, later improvement coincided with an increase in myeloblasts in the peripheral blood, i.e. with leukemic transformation. In another case, a 68-year-old man was admitted to hospital when laboratory findings showed a white blood cell count of 24,800/microl with increased myeloblasts (62.5%), leading to the diagnosis of AML with multilineage dysplasia. Following a decrease in blasts due to anti-cancer drugs, supporting the MDS-RAEB-2 status, severe anemia with increased reticulocytes and positive direct antiglobulin test was diagnosed, suggesting the existence of autoimmune hemolytic anemia, which was then ameliorated by steroid therapy.

CONCLUSIONS

The simultaneous loss of autoimmunity and leukemic cell expansion observed in our cases may possibly suggest a common underlying mechanism.

摘要

背景

本研究报告了 2 例罕见的急性髓系白血病(AML)合并仅在骨髓增生异常综合征(MDS)阶段出现的溶血性贫血,且在白血病转化后消失。

方法/结果:一名 66 岁男性患有 MDS-RAEB-2,因严重贫血伴网织红细胞计数升高而入院。疑诊溶血性贫血,给予甲基强的松龙脉冲治疗后病情改善。虽然在逐渐减少类固醇用量时贫血加重,但随后的改善与外周血中原始细胞的增加(即白血病转化)相吻合。另一个病例中,一名 68 岁男性因白细胞计数为 24800/微升,且原始细胞(62.5%)增多而就诊,诊断为伴有多系发育异常的 AML。由于抗癌药物导致白血病细胞减少,支持 MDS-RAEB-2 状态,同时出现严重贫血伴网织红细胞增多和直接抗球蛋白试验阳性,提示存在自身免疫性溶血性贫血,经类固醇治疗后病情改善。

结论

我们的病例中同时观察到自身免疫和白血病细胞扩张的消失,这可能提示存在共同的潜在机制。

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Disappearing myelodysplastic syndrome-associated hemolytic anemia in leukemic transformation.白血病转化中骨髓增生异常综合征相关溶血性贫血的消失。
Int Arch Allergy Immunol. 2010;152(4):407-12. doi: 10.1159/000288294. Epub 2010 Mar 3.
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