以血细胞异常为首发的系统性红斑狼疮临床调查研究

目的了解系统性红斑狼疮 (SLE)的血液学变化、临床特点及其对药物治疗的反应。方法对 3 1例以血液系统异常为主要表现的SLE进行骨髓细胞学检查及骨髓活检 ,并与血象等其他临床资料综合分析 ,同时观察糖皮质激素和细胞毒药物治疗后血象的变化。结果SLE骨髓表现从增生明显活跃到增生受抑 ,以至再生障碍。最具意义的是增生受抑 ,但较外周血轻 ,且未见细胞形态异常 ,受抑发生率依次为巨核细胞及血小板 ( 4 5 2 %)、红系 ( 3 8 7%)和粒系 ( 9 7%)。此外尚有呈再生障碍骨髓象、网状吞噬细胞及浆细胞增多、铁染色减少等改变 ,4例骨髓活检未见骨髓纤维化。 3 1例治疗后血细胞减少均得以改善。结论对外周血细胞减少而骨髓细胞形态无异常者应警惕SLE可能 ,缺铁可能是狼疮性贫血的原因之一 ,肝脾、淋巴结增大可能提示骨髓增生。糖皮质激素和免疫抑制剂治疗有效

Diagnosis and treatment of systemic lupus erythematosus presenting abnormalities of hematocytes

Objective To learn the hematological alterations,their clinical significance and response to chemical therapy in systemic lupus erythematosus (SLE). Methods A comprehensive analysis was carried out on bone marrow aspiration and biopsies,blood cells and clinical data in 31 cases of SLE. Meanwhile,were also observed the changes in blood pictures and their reponses to glucocorticoids and cytotoxic drugs. Results Bone marrow findings in SLE were multiform,including obvious proliferation,hyperplasia and hypoplasia.The most valuable finding was hypoplasia of bone marrow,milder than peripheral blood pictures.Moreover,cellular morphologic abnormalities of marrow cells were absent.The prevalence rate of hypoplasia was sequentially megalocaryocyte/ platelet(45 2%),erythrocytic series(38 7%) and granulocytic series(9 7%).Besides,aplastic myelogram,phagocytosis by reticular cells and plasmacytosis were,and decrease of iron staining were found.In 4 bone marrow biopsies,no myolofibrosis was encountered.After the treatment,the hematocytopenia got improved. Conclusions SLE should be highly taken into consideration when peripheral hematocytopenia was present with normal morphology of marrow cells. Iron deficiency might be partial cause for lupus anemia.Hepatosplenomegaly and enlargement of lympho nodes probably suggested that myeloproliferative reaction,a excellent response to steroid and cytotoxic therapies.