系统性红斑狼疮患者血液学异常的特点及其临床意义

目的探讨系统性红斑狼疮（SLE）患者血液系统异常的特点及其临床意义。方法236例新诊断的SLE住院患者,其中血红蛋白≤100g／L者设为贫血组;根据白细胞计数分为2组,白细胞计数〈3．0×10^9／L者为WBC1组,白细胞计数3．0～3．9×10^9／L为WBC2组;血小板〈100×10^9／L设定为血小板减少组。无血液学异常者设定为对照组。结果贫血的患者123例,占52．1％。造成贫血的原因主要有：慢性病贫血（ACD）,82例,溶血性贫血（HA）,18例,造血功能异常8例,原因未明,15例。外周血有白细胞下降者73例,占30．9％。血小板下降57例,占24．2％。无血液学异常68例,占28．8％。其中溶血性贫血组、WBC1组和血小板减低组狼疮活动指标明显高于对照组。对72例患者做了骨髓细胞形态学检查,49例是正常骨髓象,10例患者骨髓有不同程度的病态造血,2例表现骨髓巨核细胞缺乏,9例表现为骨髓三系造血增生低下,有2例表现出单纯红系造血低下。结论中国SLE患者贫血比例较高的主要原因可能是慢性病贫血患者的比例明显高于国外。SLE患者溶血性贫血比例较低,但却是SLE疾病活动很重要的指征。白细胞计数〈3．0×10^9／L和血小板下降也与SLE活动有关。部分患者骨髓造血异常是引起较严重血液学改变的原因之一。

Hematological abnormalities in systemic lupus erythematosus and clinical significance thereof: comparative analysis of 236 cases

OBJECTIVE: To investigate the hematological abnormalities and their relationship to the disease activity of systemic lupus erythematosus (SLE). METHODS: The clinical data of 236 SLE patients, 29 males and 207 females with the age of confirmed diagnosis of 33.4, were divided into 3 groups: anemia group, with the hemoglobin (Hgb) < 100 g/L, including 2 subgroups, i.e. subgroup of anemia caused by chronic diseases (ACD) and subgroup of hemolytic anemia; low white blood cell group 1 (Group WBC1) with the WBC count < 4.0 x 10(9)/L, white blood cell group 2 (Group WBC2) with the WBC count 3.0 approximately 3.9 x 10(9)/L, immune thrombopenic purpura group (ITP group) with a platelet count < 100 x 10(9)/L, and control group without hematological changes. 72 patients underwent morphologic characterization of their bone marrow. The hematological data and the relationship thereof to the disease activity in different groups were analyzed. RESULTS: Among the 236 SLE patients 168 (71.18%) had hematological abnormalities and 68 of them (28.82%) without hematological abnormalities. 123 of the 168 patients with hematological abnormalities (52.1%) had anemia, 82 of which (66.7%) had characteristics of anemia caused by chronic diseases, 18 (14.6%) had hemolytic anemia, 8 (6.6%) had hematopoietic abnormalities, and the remaining 15 patients (12%) had anemia caused by unknown reasons. 73 of the 236 SLE patients (30.9%) had a WBC count < 4.0 x 10(9)/L and 57 patients (24.2%) had a platelet count < 100 x 10(9)/L. In the groups with hemolytic anemia, WBC count < 3.0 x 10(9)/L and thrombocytopenia, the complement levels were significant lower, and the levels of C-reactive protein (CRP) and positive anti-dsDNA antibody were significantly higher than those of the controls (all P < 0.05) The rate of positive antiphospholipid antibody of the hemolytic anemic patients and patients with thrombocytopenia were 22.2% and 15.8% respectively, both significantly higher than that of the controls (2.9%, both P < 0.05). 49 of the 72 patients undergoing morphologic characterization of bone marrow had normal cell morphology and a normal appearing bone marrow, 10 had varying degrees of pathologic hematopoietic changes, 2 lacked megakaryocytes, 9 expressed decreased proliferation in all three hematopoietic lineages, and 2 had only a decrease in erythropoiesis. CONCLUSION: The reason of the high proportion of anemia among the SLE patients in China may be the higher proportion of anemia caused by ACD in comparison with that abroad. Although SLE patients have lower rates of hemolytic anemia, HA is an important index of SLE disease activity. Thrombocytopenia and a WBC count < 3.0 x 10(9)/L are related to SLE disease activity. Abnormalities of hemopoiesis by bone marrow is one of the reasons of sever hematological changes in part of the SLE patients.