Department of Pathophysiology, University of Athens, Greece.
Clin Exp Rheumatol. 2013 Jan-Feb;31(1):76-83. Epub 2012 Sep 25.
The aim of this study was to evaluate the aetiology of 'unexplained' cytopenias in patients with autoimmune disorders, as well as to identify parameters that should alert clinicians to the need for bone marrow examination.
During the study period (2005-2010), 110 consecutive patients with an underlying systemic autoimmune disease, excluding Sjogren's syndrome, were referred for haematological consultation and bone marrow examination, due to cytopenias without evident cause including blood loss, haemolysis, nutritional deficiencies and haemoglobin disorders.
Systemic lupus erythaematosus was the most frequent underlying condition (38/110, 34.5%), and anaemia (haemoglobin<12gr/dl) the most common haematologic abnormality (81/110, 74%). Prior to evaluation, more than half of the patients received cytotoxic or immunosuppressive drugs, with methotrexate being the most commonly administrated agent (29/110, 26.4%). Evaluation was informative in 31 (28.2%) of the cases. Twenty-four (21.8%) cases of haematologic clonal disease were diagnosed; 11 myelodysplastic syndromes, 6 lymphoproliferative disorders, 6 plasma cell dyscrasias and one myeloproliferative neoplasm. Seven cases (6.4%) with bone marrow toxicity were also noted. Male gender, serum iron >90 μg/dl, mean corpuscular volume (MCV) >90fl, and serum monoclonal band were significant predictors of specific diagnosis including clonal haematologic disorder or bone marrow toxicity. All other correlations were insignificant.
Clonal haematologic disorders and toxicity are frequent findings in patients with autoimmunity referred for haematologic consultation, owing to otherwise unexplained cytopenias. Patients with high serum iron, high MCV and presence of serum monoclonal band should undergo bone marrow examination to exclude haematologic malignancy or bone marrow toxicity.
本研究旨在评估自身免疫性疾病患者“不明原因”血细胞减少症的病因,并确定一些参数,以便临床医生警惕行骨髓检查的必要性。
在研究期间(2005-2010 年),由于血细胞减少而无明显原因(包括失血、溶血、营养缺乏和血红蛋白疾病),110 例患有系统性自身免疫疾病(不包括干燥综合征)的连续患者被转介进行血液学咨询和骨髓检查。
系统性红斑狼疮是最常见的基础疾病(38/110,34.5%),贫血(血红蛋白<12g/dl)是最常见的血液学异常(81/110,74%)。在评估之前,超过一半的患者接受了细胞毒性或免疫抑制剂治疗,其中甲氨蝶呤是最常用的药物(29/110,26.4%)。31 例(28.2%)的病例评估结果有信息。诊断出 24 例(21.8%)血液学克隆性疾病;11 例骨髓增生异常综合征、6 例淋巴增生性疾病、6 例浆细胞异常和 1 例骨髓增生性肿瘤。还注意到 7 例(6.4%)骨髓毒性病例。男性、血清铁>90μg/dl、平均红细胞体积(MCV)>90fl 和血清单克隆带是特定诊断(包括克隆性血液病或骨髓毒性)的显著预测因子。所有其他相关性均无统计学意义。
自身免疫性疾病患者因不明原因的血细胞减少而转介进行血液学咨询时,常发现克隆性血液病和毒性。血清铁、MCV 高和血清单克隆带阳性的患者应行骨髓检查以排除血液恶性肿瘤或骨髓毒性。