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目的总结骨髓单个核细胞Coombs(BMMNC-Coombs)试验阳性免疫相关性血细胞减少症(IRP)患者的临床特征。方法回顾性分析天津医科大学总医院血液科1995年1月至2004年3月诊治的72例骨髓BMMNC-Coombs试验阳性的IRP患者的发病诱因、临床表现、血象及骨髓象、自身抗体类型及免疫治疗效果。结果62.5%(45/72)IRP患者发病前有感染、过敏或妊娠等诱因。72.2%(52/72)患者全血细胞减少;91.7%(66/72)患者有网织红细胞不低的大细胞或正细胞贫血;75.0%(54/72)患者有白细胞减少;18.1%(13/72)患者合并发热;95.8%(69/72)患者可见血小板轻、中、重度减少。以皮肤黏膜出血为主。髂骨骨髓增生活跃和明显活跃[68.1%(49/72)],红系比例增高[51.4%(37/72)]或正常[19.4%(14/72)],骨髓淋巴细胞比例与疗效呈负相关;大部分患者[65.9%(27/41)]胸骨骨髓为增生活跃或明显活跃。BMMNC-Coombs试验皆阳性,检出不同的自身抗体类型和组合方式,56.9%(41/72)患者有溶血迹象,但不符合任何种类的溶血性疾病的诊断标准;42.9%(18/42)患者补体C3降低,19.0%(8/42)患者同时或单独存在补体C4降低。给予免疫抑制和促造血治疗后,3年有效率为87.5%(28/32)。结论IRP是一类获得性自身抗体介导的骨髓细胞破坏或抑制性疾病,BMMNC-Coombs试验阳性IRP患者主要表现为网织红细胞和(或)中性粒细胞比例不低的2系或3系血细胞减少,对免疫抑制和促造血治疗反应好。
Objective To summarize the clinical features of patients with positive immune-related cytopenia (IRP) in the bone marrow mononuclear cell Coombs (BMMNC-Coombs) assay. Methods A retrospective analysis of 72 patients with BMMNC-Coombs positive IRP diagnosed and treated by Department of Hematology, Tianjin Medical University General Hospital from January 1995 to March 2004 was made. The incidence, clinical manifestations, blood and bone marrow, autoantibodies and immune treatment effect. Results 62.5% (45/72) patients with IRP had infection, allergy or pregnancy before onset. 72.2% (52/72) of patients had pancytopenia; 91.7% (66/72) of patients had reticulocyte with low or no cell anemia; leukemia in 75.0% (54/72) patients; 18.1% / 72) patients with fever; 95.8% (69/72) of patients showed mild, moderate, severe reduction of platelets. Mainly to the skin and mucous membrane bleeding. Iliopathic bone marrow hyperplasia was active and significantly active [68.1% (49/72)], the proportion of erythroid increased [51.4% (37/72)] or normal [19.4% (14/72)]. The proportion of bone marrow lymphocytes and the therapeutic effect Negative correlation; most patients [65.9% (27/41)] sternal bone marrow hyperplasia was active or significantly active. BMMNC-Coombs test was positive. Different types and combinations of autoantibodies were detected. 56.9% (41/72) patients showed signs of hemolysis but did not meet the diagnostic criteria of any kind of hemolytic disease; 42.9% (18/42) Patients with decreased complement C3, 19.0% (8/42) patients with simultaneous or separate complement C4 decreased. Three years after giving immunosuppressive and hematopoietic treatment, the response rate was 87.5% (28/32). Conclusions IRP is a kind of acquired autoantibody-mediated myelocytic destruction or inhibitory disease. The patients with positive IRP in BMMNC-Coombs test mainly showed 2 or 3 neutrophils with relatively low proportion of reticulocytes and / or neutrophils Cytopenia, immune suppression and promote hematopoietic response to treatment.