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免疫相关性全血细胞减少(IRP)或血细胞减少(IRH)症是从骨髓衰竭性疾病中分离出的一种病理机制相对清晰的独立疾病体系,是由于T淋巴细胞调控失衡导致B淋巴细胞数量、亚群、功能异常,进而产生抗骨髓未成熟造血细胞自身抗体并破坏或抑制之,最终导致外周血细胞减少的一类疾病。诊断IRP/IRH关键依据是测得骨髓造血细胞膜结合自身抗体,或经足量肾上腺皮质激素或(和)大剂量静脉丙种球蛋白治疗有效,以及除外检查。目前主要治疗方法为免疫抑制、促造血及对症支持等综合治疗。
Immune-related pancytopenia (IRP) or pancytopenia (IRH) is a relatively clear independent disease system isolated from bone-marrow failure, due to the imbalance of T lymphocytes leading to the number of B lymphocytes, Subpopulation, dysfunction, and then produce anti-myelogenous immature hematopoietic autoantibodies and destroy or inhibit the final result in peripheral blood cells decreased a class of diseases. The key basis for the diagnosis of IRP / IRH is the measurement of bone marrow hematopoietic cell membrane-bound autoantibodies, or treatment with adequate amounts of adrenocorticotropic hormone or (and) high-dose intravenous gamma globulin, as well as exclusion tests. At present the main treatment for immunosuppression, promote hematopoietic and symptomatic support and other comprehensive treatment.