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类固醇反应性周期性中性粒细胞减少症。

Steroid responsive cyclical neutropenia.

作者信息

Young G A, Iland H J, Deveridge S F, Forrest P R, Vincent P C

出版信息

Blut. 1984 Mar;48(3):153-9. doi: 10.1007/BF00320338.

DOI:10.1007/BF00320338
PMID:6697004
Abstract

A 19-year old girl with severe cyclical neutropenia associated with life-threatening infection and who responded dramatically to the administration of oral prednisolone is described. During reduction and eventual cessation of steroid therapy normal or near normal neutrophil counts have been maintained, and there has been parallel improvement in clinical well-being. Prior to therapy and at a time of peak blood neutrophil count low numbers of granulocyte-macrophage progenitor cells (CFU-C) were found in the patient's bone marrow, and her lymphocytes co-cultured with normal marrow failed to show the inhibitory effect normally seen with normal lymphocytes. The findings in this patient are compared with those in the two other cases where cyclical neutropenia has been shown to respond to steroids.

摘要

本文描述了一名19岁患有严重周期性中性粒细胞减少症并伴有危及生命感染的女孩,该女孩对口服泼尼松龙治疗反应显著。在逐渐减少并最终停止类固醇治疗期间,中性粒细胞计数维持在正常或接近正常水平,临床状况也同步改善。治疗前及血中性粒细胞计数峰值时,在患者骨髓中发现少量粒细胞-巨噬细胞祖细胞(CFU-C),且其淋巴细胞与正常骨髓共同培养时未显示出正常淋巴细胞通常所见的抑制作用。将该患者的研究结果与另外两例已证明对类固醇有反应的周期性中性粒细胞减少症病例的结果进行了比较。

相似文献

1
Steroid responsive cyclical neutropenia.类固醇反应性周期性中性粒细胞减少症。
Blut. 1984 Mar;48(3):153-9. doi: 10.1007/BF00320338.
2
Correction of human cyclic neutropenia with prednisolone.
N Engl J Med. 1978 Feb 9;298(6):295-300. doi: 10.1056/NEJM197802092980602.
3
Inhibition of CFU-GM by prostaglandins in a case of chronic T-cell lymphocytosis and neutropenia.前列腺素对一例慢性T细胞淋巴细胞增多症和中性粒细胞减少症患者CFU-GM的抑制作用
Br J Haematol. 1989 Oct;73(2):148-51. doi: 10.1111/j.1365-2141.1989.tb00244.x.
4
Lithium therapy of children with chronic neutropenia.
Am J Med. 1981 May;70(5):1073-7. doi: 10.1016/0002-9343(81)90871-8.
5
A Rare Case of Adult Autoimmune Neutropenia Successfully Treated with Prednisolone.1例成人自身免疫性中性粒细胞减少症经泼尼松龙成功治疗的罕见病例。
Intern Med. 2017;56(11):1415-1419. doi: 10.2169/internalmedicine.56.7619. Epub 2017 Jun 1.
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Levamisole in the treatment of cyclical neutropenia.左旋咪唑治疗周期性中性粒细胞减少症。
Postgrad Med J. 1979;55(642):279-81. doi: 10.1136/pgmj.55.642.279.
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Antithymocyte globulin therapy for pure white cell aplasia.抗胸腺细胞球蛋白治疗纯白细胞再生障碍。
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Leucocyte transfusions from rhG-CSF or prednisolone stimulated donors for treatment of severe infections in immunocompromised neutropenic patients.来自重组人粒细胞集落刺激因子(rhG-CSF)或泼尼松龙刺激供体的白细胞输注用于治疗免疫功能低下的中性粒细胞减少患者的严重感染。
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10
Congenital neutropenia and low serum immunoglobulin A: description and investigation of a large kindred.先天性中性粒细胞减少症与低血清免疫球蛋白A:一个大家族的描述与研究
Thymus. 1988;11(3):185-99.

本文引用的文献

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TREATMENT OF CYCLIC NEUTROPENIA WITH TESTOSTERONE.用睾酮治疗周期性中性粒细胞减少症。
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Cyclic neutropenia; report of a case treated by splenectomy.
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Human cyclic neutropenia: clinical review and long-term follow-up of patients.人类周期性中性粒细胞减少症:患者的临床综述与长期随访
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CFU-C inhibitors in aplastic anaemia.再生障碍性贫血中的集落形成单位 - 粒细胞(CFU-C)抑制剂
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