摘要
髓系肿瘤(MN)为一组造血干祖细胞生物学功能异常的血液系统恶性肿瘤.越来越多的证据表明,MN患者异常的免疫、造血微环境与恶性克隆性造血干细胞相互作用,促进其疾病的发生、发展.髓系肿瘤伴大颗粒淋巴细胞增殖(Myeloid neoplasms-large granular lymphocyte proliferation,MN-LGLP)为此类疾病中的一种特殊罕见的临床现象,目前国内外对本病的队列研究较少,本研究分析该类患者的临床和实验室特征,探讨影响LGLP对MN患者临床特征和生存的影响.提示MN-LGLP患者更易出现中性粒细胞减少及脾大.LGLP的存在不是影响MN-LGLP患者生存的危险因素.STAG、ASXL1、TET2是MN-LGLP最常见的伴随基因突变,伴有STAG2突变MN-LGLP患者预后较差.
Abstract
Myeloid neoplasms(MNs)belong to a group of hematological malignancies characterized by the abnormal biological functions of hematopoietic stem progenitor cells.The abnormal immune and hematopoietic microenvironment of patients with MN interact with malignant clonal hematopoietic stem cells,promoting the occurrence and development of their diseases.MN large granular lymphocyte proliferation(MN-LGLP)is a special and rare clinical phenomenon in this type of disease.Currently,research on this disease in domestic and international cohorts is limited.This study analyzes the clinical and laboratory characteristics of this type of patient and explores the impact of LGLP on the clinical characteristics and survival of patients with MN.Patients with MN-LGLP are prone to neutropenia and splenomegaly.The presence of LGLP is not a risk factor affecting the survival of patients with MN-LGLP.STAG,ASXL1,and TET2 are the most common accompanying gene mutations in MN-LGLP,and patients with MN-LGLP and STAG2 mutations have poor prognoses.
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