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目的评价系统性红斑狼疮(SLE)患者骨髓增生异常的特性及其与疾病活动度的关系。方法SLE初次诊治患者48例,骨髓增生异常综合征(myelodysplastic syndrome,MDS)患者10例,免疫性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)患者10例。所有患者都因血细胞减少予骨髓穿刺。骨髓涂片Wright-Gimsa染色后在显微镜下观察,同时对比观察10例MDS和ITP患者的骨髓,评价骨髓增生异常的情况。临床和实验室资料通过病历记载获得,SLE疾病的活动性根据SLEDAI标准评估。结果48例SLE患者中白细胞减少12例,贫血43例,血小板减少28例,Coombs’试验阳性17例。31例患者有骨髓发育异常,以派-胡畸形最常见,还有红系和巨核细胞系发育异常。而10例MDS患者至少可以发现一系的病态造血。而ITP患者只是巨核细胞明显增多,出现幼稚产血小板巨核细胞,而粒系和红系没有病态造血。骨髓病态造血的出现和SLE活动明显相关,5例有狼疮性肾炎的患者骨髓均有病态造血。15例复查骨髓的患者中,12例疾病控制良好的患者骨髓病态造血消失。结论血细胞减少是SLE常见的表现,骨髓的各系包括粒系、红系和巨核细胞系均可以有病态造血,疾病控制后病态造血可消失。SLE患者骨髓的病态造血和SLE的严重性相关,可作为评价SLE活动性的指标。同时,对没有染色体异常的骨髓病态造血,诊断MDS时需排除SLE。
Objective To evaluate the characteristics of myelodysplastic syndrome in patients with systemic lupus erythematosus (SLE) and its relationship with disease activity. Methods Forty-eight patients with initial diagnosis and treatment of SLE, 10 patients with myelodysplastic syndrome (MDS) and 10 patients with idiopathic thrombocytopenic purpura (ITP). All patients had bone marrow as a result of cytopenia. BMSCs were stained with Wright-Gimsa and observed under the microscope. Meanwhile, the bone marrow of 10 patients with MDS and ITP were observed to evaluate the myelodysplasia. Clinical and laboratory data were obtained from medical records and the activity of SLE was assessed according to the SLEDAI criteria. Results 48 cases of SLE patients with leukopenia in 12 cases, 43 cases of anemia, thrombocytopenia in 28 cases, Coombs’ test was positive in 17 cases. Thirty-one patients had myelodysplastic abnormality, most often with pie-hu malformation, and abnormal erythroid and megakaryocytic cell lines. The 10 cases of MDS patients can be found at least a series of pathological hematopoiesis. The ITP patients only megakaryocytes significantly increased, there naive platelet megakaryocytes, and granulocyte and erythroid no pathological hematopoietic. Bone marrow morbid hematopoiesis and SLE activity was significantly related to the 5 cases of patients with lupus nephritis have morbid hematopoietic bone marrow. Of the 15 patients who reviewed the bone marrow, 12 patients with well-controlled disease disappeared from the bone marrow. CONCLUSIONS: Cytopenia is a common manifestation of SLE. Pathological hematopoiesis may disappear in all lines of bone marrow, including myeloid, erythroid and megakaryocyte lines, after pathological control. SLE patients with pathological bone marrow morbidity and the severity of SLE can be used as an indicator of SLE activity. At the same time, there is no chromosomal abnormalities of bone marrow morbid hematopoietic, diagnosis of MDS need to rule out SLE.