Cutaneous manifestations in relation to immunologic parameters in a cohort of primary myelodysplastic syndrome patients
Cutaneous manifestations in relation to immunologic parameters in a cohort of primary myelodysplastic syndrome patients
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DOI：
10.1111/j.1468-3083.2007.02520.x
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发表时间：
2008-05-01
影响因子：
9.2
通讯作者：
Papadavid, E.
中科院分区：
文献类型：
--
作者：
Dalamaga, M.;Karmaniolas, K.;Papadavid, E.
背景 骨髓增生异常综合征 (MDS) 的皮肤病变可能是特异性的，也可能不是特异性的，并且可能揭示骨髓转化。我们的目的是在 84 名 MDS 患者队列中调查皮肤表现与免疫学参数和 MDS 预后特征的相关性，以阐明其潜在的临床意义。材料和方法我们研究了 84 名新诊断的 MDS 患者队列，以评估诊断时和 1 至 3 年随访期间出现的皮肤表现。我们描述了通过组织学确定的皮肤表现的临床多样性。我们还寻找 MDS 患者组的皮肤表现与 MDS 亚型、免疫学和预后特征之间的任何关联，这些特征突出显示为急性白血病的转变。 结果 21 名患者出现皮肤表现：1 名患者出现皮肤白血病，6 名患者出现与自身免疫性疾病无关的光敏性，3 名结节性痒疹，2 名 Sweet 综合征，6 名白细胞破碎性血管炎，2 名患者与先前存在的复发性多软骨炎相关的瘀斑和紫癜，1 名患者与韦格纳肉芽肿相关的皮下结节，以及 1 名患者与先前存在的系统性红斑狼疮相关的颧骨皮疹和口腔溃疡。根据年龄和性别进行调整后，皮肤检查结果的存在构成了高危 MDS 亚组的重要预测因子（比值比，3.59；95% 置信区间，1.18-10.92）。有皮肤表现的MDS亚组高丙种球蛋白血症明显较高（P=0.03）。结论大多数有皮肤表现的MDS患者属于高危MDS亚组，且存在高丙种球蛋白血症。需要对骨髓增生异常的皮肤病变进行早期活检。
Background Cutaneous lesions in myelodysplastic syndrome (MDS) may be specific or not and may reveal bone marrow transformation. Our purpose was to investigate in a cohort of 84 MDS patients the correlation of cutaneous findings with immunologic parameters and prognostic features of MDS in order to clarify their potential clinical significance.Materials and methods We studied a cohort of 84 newly diagnosed MDS patients in order to assess the cutaneous findings present at the time of diagnosis and during 1 to 3 years of follow-up. We described the clinical variety of cutaneous findings ascertained by histology. We also looked for any association between the group of MDS patients with skin manifestations and MDS subtype, immunologic and prognostic features highlighting transformation to acute leukaemia.Results Twenty-one patients presented cutaneous manifestations: 1 patient developed leukaemia cutis, 6 patients photosensitivity not associated with autoimmune disease, 3 prurigo nodularis, 2 Sweet's syndrome, 6 leucocytoclastic vasculitis, 2 ecchymoses and purpura associated with preexisting relapsing polychondritis, 1 patient subcutaneous nodules associated with Wegener's granulomatosis and 1 patient with malar rash and oral ulcers associated with preexisting systemic lupus erythematosus. Adjusted for age and gender, the presence of skin findings constitutes a significant predictor of the high-risk MDS subgroup (odds ratio, 3.59; 95% confidence interval, 1.18-10.92). Hypergammaglobulinemia was significantly higher in the MDS subgroup with skin manifestations (P = 0.03).Conclusion Most MDS patients with cutaneous manifestations belong to the high-risk MDS subgroup and present hypergammaglobulinemia. Early biopsy of skin lesions in myelodysplasia is indicated.