口服环磷酰胺对CD16+CD56-自然杀伤细胞慢性淋巴细胞增殖性疾病中的库姆斯阴性自身免疫性溶血性贫血有效。

Oral cyclophosphamide was effective for Coombs-negative autoimmune hemolytic anemia in CD16+CD56- chronic lymphoproliferative disorder of NK-cells.

作者信息

Sekiguchi Nodoka, Nishina Sayaka, Kawakami Toru, Sakai Hitoshi, Senoo Noriko, Senoo Yasushi, Ito Toshiro, Saito Hiroshi, Nakazawa Hideyuki, Koizumi Tomonobu, Ishida Fumihiro

机构信息

Department of Comprehensive Cancer Therapy, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.

Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.

出版信息

Int J Hematol. 2017 Jun;105(6):854-858. doi: 10.1007/s12185-016-2170-4. Epub 2016 Dec 27.

DOI:10.1007/s12185-016-2170-4

PMID:28028749

Abstract

An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated. She was diagnosed as having as Coombs-negative autoimmune hemolytic anemia (AIHA). We report the effectiveness of oral cyclophosphamide for Coombs-negative autoimmune hemolytic anemia in CLPD-NK.

摘要

一名84岁女性因贫血被转诊至我院。她的血红蛋白水平为5.8 g/dL，白细胞计数为9400/μL，其中淋巴细胞占82%。鉴于淋巴细胞表型（CD2+、CD3-、CD16+和CD56-）以及全血EBV病毒载量为阴性，我们诊断为NK细胞慢性淋巴细胞增殖性疾病（CLPD-NK）。由于外周血中网织红细胞水平较高且触珠蛋白值较低，我们怀疑存在溶血性贫血。尽管直接抗人球蛋白试验为阴性且无冷凝集现象，但我们检测了她的红细胞结合IgG（RBC-IgG），其水平升高。她被诊断为抗人球蛋白阴性自身免疫性溶血性贫血（AIHA）。我们报告了口服环磷酰胺治疗CLPD-NK中抗人球蛋白阴性自身免疫性溶血性贫血的有效性。