Billström R, Johansson H, Johansson B, Mitelman F
Department of Medicine, Helsingborg Hospital, Lund, Sweden.
Eur J Haematol. 1995 Jul;55(1):42-8. doi: 10.1111/j.1600-0609.1995.tb00231.x.
It has been recognized in recent years that some patients with myelodysplastic syndromes (MDS) develop immune-mediated complications (IMC), but little is known about the correlations to MDS-specific disease features. In a retrospective study of 82 MDS patients, we identified 10 (12%) with IMC (group A) and compared them to the remaining 72 cases (group B). Group A consisted of 5 patients with biopsy-verified skin vasculitis and 1 case each with temporal arteritis/polymyalgia rheumatica, necrotising panniculitis, Hashimoto's thyroiditis, autoimmune thrombocytopenia, and Sweet's syndrome. Survival times, sex ratio and distribution of MDS subtypes were similar in the two groups. The patients in group A were younger than those in group B (median 66 vs. 76 years, p < 0.01). Four patients (40%) in group A had a history of previous genotoxic therapy for malignant disorders. The bone marrow karyotype was evaluated in 62 patients. Clonal chromosomal abnormalities were found more frequently in Group A than in group B (8/9 vs. 26/53, p = 0.03), and complex karyotypes, i.e., three or more aberrations, were also observed to be more common in group A (3/9 vs. 8/53). The results indicate that IMC preferentially develop in patients with secondary MDS, in younger MDS cases, and in patients with cytogenetic abnormalities.
近年来人们已经认识到，一些骨髓增生异常综合征（MDS）患者会出现免疫介导的并发症（IMC），但对于其与MDS特异性疾病特征之间的相关性却知之甚少。在一项对82例MDS患者的回顾性研究中，我们确定了10例（12%）患有IMC的患者（A组），并将他们与其余72例患者（B组）进行比较。A组包括5例经活检证实的皮肤血管炎患者，以及各1例颞动脉炎/风湿性多肌痛、坏死性脂膜炎、桥本甲状腺炎、自身免疫性血小板减少症和Sweet综合征患者。两组的生存时间、性别比例和MDS亚型分布相似。A组患者比B组患者年轻（中位年龄66岁对76岁，p<0.01）。A组中有4例患者（40%）有既往针对恶性疾病的基因毒性治疗史。对62例患者进行了骨髓核型评估。A组比B组更频繁地发现克隆性染色体异常（8/9对26/53，p=0.03），并且在A组中也观察到复杂核型，即三个或更多畸变更为常见（3/9对8/53）。结果表明，IMC优先发生在继发性MDS患者、年轻的MDS患者以及有细胞遗传学异常的患者中。
