纯红细胞发育不全1例:不同免疫抑制方案的随访。

M Baldus, M Möller, H Walter, H Brass, W Queisser
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引用次数: 2

摘要

一名66岁患者于1992年1月入院,以进一步评估严重正红细胞贫血。血红蛋白(Hb) 3.5 g/dl,网织红细胞计数1%。骨髓显示几乎完全缺乏红细胞前体,因此有利于获得性纯红细胞不全(PRCA)的诊断。开始使用强的松龙进行免疫抑制治疗,但必须补充硫唑嘌呤,因为在最初输入6u红细胞后,Hb进一步迅速下降至3.7 g/dl。然而,在该方案下,Hb再次下降至6.6 g/dl,需要进一步输血。因此,治疗切换到环孢素A (CyA),同时逐渐减少强的松龙。初步诊断4个月后,发现细小病毒B19 IgM抗体阳性。在三种免疫抑制方案的血液学缓解失败后,于1992年7月给予高剂量静脉注射免疫球蛋白(IVIG)。IVIG治疗6周后,血红蛋白峰值浓度为12.3 g/dl,无需进一步输血。青年会在1992年10月逐渐减少。一个月后,由于PRCA复发,重新启动了CyA,但直到1993年1月才成功。此时,由于牙周脓肿,停止了免疫抑制CyA治疗。1993年2月进行了第二次静脉注射,注意到PRCA的第二次缓解,到1993年11月血红蛋白增加到14.5 g/dl。在1994年2月的最后一次随访中,我们的患者血液学仍然完全缓解。
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A case of pure red cell aplasia: follow-up on different immunosuppressive regimens.

A 66-year-old patient was admitted to our hospital in January 1992 for further evaluation of severe normocytic anemia. Hemoglobin (Hb) was 3.5 g/dl, reticulocyte count 1%. Bone marrow showed a nearly complete lack of red cell precursors, thus favoring the diagnosis of acquired pure red cell aplasia (PRCA). Immunosuppressive therapy with prednisolone was started but had to be supplemented with azathioprine because of a further rapid decrease in Hb to 3.7 g/dl after an initial transfusion of 6 U red blood cells. However, with this regimen a renewed decrease in Hb to 6.6 g/dl was noted, and further transfusions were required. Therefore therapy was switched to cyclosporine A (CyA) while tapering off prednisolone. Four months after the initial diagnosis a positive parvovirus B19 IgM antibody was found. After the failure of hematological remission with three immunosuppressive regimens a course of high-dose intravenous immunoglobulins (IVIG) was administered in July 1992. Six weeks after IVIG therapy a peak hemoglobin concentration of 12.3 g/dl was noted, and further transfusion was not required. CyA was tapered off in October 1992. One month later CyA was reinstituted because of a relapse of PRCA but was unsuccessful until January 1993. At this time immunosuppressive CyA therapy was discontinued because of a periodontal abscess. In February 1993 a second IVIG infusion was given, and a second remission of PRCA was noted, showing an increase in hemoglobin up to 14.5 g/dl by November 1993. At the last follow-up visit in February 1994 our patient was still in complete hematological remission.

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