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周期性造血:人类周期性中性粒细胞减少症

Cyclic hematopoiesis: human cyclic neutropenia.

作者信息

Lange R D

出版信息

Exp Hematol. 1983 Jul;11(6):435-51.

PMID:6352296
Abstract

Human cyclic neutropenia is a relatively rare disorder of unknown etiology. Study of patients and animals with the disorder has led to important information regarding the differentiation of blood cells and control mechanisms of hematopoietic regulation. It has a world-wide distribution, occurs in both sexes, and, in about one-fourth of the patients, a family history has been obtained. While usually benign, deaths from overwhelming infections occur. In addition to cycling of neutrophils, in the majority of cases the monocytes cycle and in about one-fifth of the cases eosinophils are elevated. In a small number of patients, cycling of platelets and reticulocytes occurs. Cycles of colony stimulating factor are present. Cycles of bone marrow cells are easily demonstrable. The recent transfer of human cyclic neutropenia following allogenic bone marrow grafting confirms the hypothesis that the disorder is of bone marrow origin. The following subjects are covered in this review article: A. Definition, history, and incidence; B. Etiology, geographic distribution, mode of transmission; C. Symptoms, physical signs, diagnosis, clinical course; D. Clinical laboratory studies; E. Experimental studies; F. Prognosis; G. Treatment. It is felt that human cyclic neutropenia represents a heterogeneous group of disorders and that much remains to be learned about its cause(s).

摘要

人类周期性中性粒细胞减少症是一种病因不明的相对罕见的疾病。对患有该疾病的患者和动物的研究已得出有关血细胞分化和造血调节控制机制的重要信息。它在全球范围内均有分布,男女均可发病,约四分之一的患者有家族病史。虽然通常为良性,但也有因严重感染导致死亡的情况。除了中性粒细胞呈周期性变化外,大多数病例中单核细胞也呈周期性变化,约五分之一的病例中嗜酸性粒细胞增多。少数患者血小板和网织红细胞也呈周期性变化。存在集落刺激因子的周期性变化。骨髓细胞的周期性变化很容易得到证实。同种异体骨髓移植后出现人类周期性中性粒细胞减少症,这证实了该疾病起源于骨髓的假说。本文综述涵盖以下主题:A. 定义、历史和发病率;B. 病因、地理分布、传播方式;C. 症状、体征、诊断、临床病程;D. 临床实验室研究;E. 实验研究;F. 预后;G. 治疗。人们认为人类周期性中性粒细胞减少症代表一组异质性疾病,关于其病因仍有许多有待了解之处。

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