免疫相关性全血细胞减少症39例临床分析

目的 深入了解免疫相关性全血细胞减少症的临床特征,以便与其他血细胞减少症加以鉴别.方法 回顾性分析免疫相关性全血细胞减少症39例的临床资料.结果 本组疾病主要临床特征:三系血细胞减少,网织红细胞和中性粒细胞百分比分别为0.66%～5.76%、46.7%～72.4%,骨髓增生情况多数为活跃,部分明显活跃或减低,粒系血细胞比例均数0.54,红系血细胞比例均数0.35,全片无巨核细胞4例,1～10个8例,>10～35个27例.CD19+、CD5+CD19+B淋巴细胞比率显著高于正常人;通过骨髓单个核细胞抗人球蛋白试验或流式细胞术均可检测到骨髓未成熟造血细胞自身抗体,阳性率分剐为46.2%和89.7%.给予免疫抑制和促造血治疗后,多数患者症状减轻,血细胞不同程度上升.定期随访,6个月后5例患者白细胞及血红蛋白恢复正常,血小板维持在(50～70)×109/L左右.结论 免疫相关性全血细胞减少症是一类抗骨髓未成熟造血细胞自身抗体导致的全血细胞减少性疾病,全面的实验室检查有利于与其他血细胞减少症鉴别.骨髓单个核细胞抗人球蛋白试验、流式细胞术进行外周血B淋巴细胞亚群、骨髓单个核细胞自身抗体检测可为该疾病诊断提供一定的诊断依据,对明确诊断有重要的临床意义.多数病例对免疫抑制和促造血治疗反应好.

Clinical analysis on 39 cases of immunorelated pancytopenia

Objective To know more about the clinical features of immunorelated pancytopenia, for discrimination from other cytopenia diseases. Methods Retrospective analysis was conducted on 39 cases with immunorelated pancytopenia. Results The major clinical features of this group： pancytopenia, the percentage of granulophilocyte or（and） granulocyte in normal level（0.66%-5.76% ,46.7%-72.4%） ;the bone marrow hyperplasia in active level normally, partly in obviously active or degrade state, the average ratio of erythroblastic and grain array were 0.54 and 0.35,respectively,also 4 cases without giant plates,8 cases with 1-10,27 cases with 10-35. The percentage of CD19^＋ ,CD5^＋ CD19^＋B lymphocyte were obviously higher than that of the normal subjects. Through the bone marrow mononuclear cell anti-human-globulin test or flow cytometry, the antibody of nonage bone marrow hematopoietic cell could be detected. The positive rate was 46.2% and 89.7% ,respectively. After the treating of immune suppression and crowded haematogenesis, most patients felt better with different rising level of blood cell. After six month-regular follow-up,leukocytes and haemoglobin recovered to the normal level,and blood platelet kept on （50-70）× 10^9/L in five cases. Conclusion Immunorelated pancytopenia is a kind of pancytopenia disease introduced by the antibody of nonage bone marrow hematopoietic cell, the all round lab tests profit discrimination from other cytopenia. The bone marrow mononuclear cell anti-human-globulin test and the test of the antibody of peripheral blood B lymphocyte subgroup by flow cytometry can offer somewhat diagnosis clue, meanwhile there is an important clinical significance for final diagnosis. A good therapeutic reaction from irnmune suppression and crowded haematogenesis were observed in most cases.