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[与纯红细胞再生障碍相关的慢性T细胞淋巴细胞增殖性疾病]

[Chronic T-cell lymphoproliferative disorder associated with pure red cell aplasia].

作者信息

Chubachi A, Yoshida K, Endo Y, Miura A, Akihama T, Kuwayama A

出版信息

Rinsho Ketsueki. 1989 Jan;30(1):105-10.

PMID:2497262
Abstract

A 42-year-old woman was admitted to our hospital because of easy fatigability in Jan. 1976. Laboratory examination revealed severe macrocytic anemia and slight lymphocytosis. She was diagnosed as having pure red cell aplasia (PRCA). She went into hematological remission 6 weeks following 40 mg/day treatment with prednisolone, but the anemia relapsed frequently when the dosage was lessened. She was then treated with 50 mg/day of cyclophosphamide, 50 mg/day of azathioprine, splenectomy, and methylprednisolone pulse therapy, but the recovery from anemia was temporary after each treatment. Since 1984, peripheral lymphocyte counts were 1-30,000/microliters, and reticulocyte counts were 0. She died of sepsis of Listeria in Sep. 1986. Peripheral lymphocytes had large azurophilic granules and an immunophenotype of OKT3+8+11+Ia1+Leu7+.

摘要

一名42岁女性于1976年1月因易疲劳入院。实验室检查发现严重大细胞性贫血和轻度淋巴细胞增多。她被诊断为纯红细胞再生障碍性贫血(PRCA)。在接受40毫克/天泼尼松龙治疗6周后,她进入血液学缓解期,但当剂量减少时,贫血频繁复发。随后她接受了50毫克/天环磷酰胺、50毫克/天硫唑嘌呤、脾切除术和甲泼尼龙冲击疗法,但每次治疗后贫血的恢复都是暂时的。自1984年以来,外周血淋巴细胞计数为1 - 30000/微升,网织红细胞计数为0。她于1986年9月死于李斯特菌败血症。外周血淋巴细胞有大的嗜天青颗粒,免疫表型为OKT3 + 8 + 11 + Ia1 + Leu7 +。

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