Savige J A, Chang L, Smith C L, Duggan J C
University Department of Medicine, Austin Hospital, Heidelberg, Victoria, Australia.
Leuk Lymphoma. 1993 Jan;9(1-2):49-54. doi: 10.3109/10428199309148503.
A cutaneous or systemic vasculitis occurs in myelodysplasia as well as in myeloproliferative and lymphoproliferative disorders. The most common lesion is a leucocytoclastic vasculitis, with neurological or joint involvement occurring less often. The vasculitis may appear contemporaneously with or precede the clinical onset of the blood dyscrasia. Occasionally the lesions respond dramatically to the use of steroids but in general, patients with vasculitis have a worse prognosis than those with uncomplicated myelodysplasia. Vasculitis and myelodysplasia appear together too often for the association to be coincidental and the vasculitis in most cases cannot be attributed to intercurrent infections, therapeutic agents or a pre-existing rheumatological disorder. While autoantibodies are frequently present in myelodysplasia, and ANA and anti-neutrophil cytoplasm antibodies (ANCA) are found in other vasculitides, neither of these antibodies is associated with the vasculitis of myelodysplasia. There has however been one report of ANCA in Sweet's syndrome a non-vasculitic skin condition that also occurs in the myelodysplastic syndromes.
皮肤或系统性血管炎可发生于骨髓增生异常综合征以及骨髓增殖性和淋巴增殖性疾病中。最常见的病变是白细胞破碎性血管炎，较少出现神经或关节受累。血管炎可能与血液系统疾病的临床发作同时出现或先于其发作。偶尔，病变对使用类固醇有显著反应，但总体而言，血管炎患者的预后比无并发症的骨髓增生异常综合征患者更差。血管炎和骨髓增生异常综合征同时出现的频率过高，不太可能是巧合，而且在大多数情况下，血管炎不能归因于并发感染、治疗药物或既往存在的风湿性疾病。虽然自身抗体在骨髓增生异常综合征中经常出现，且在其他血管炎中可发现抗核抗体（ANA）和抗中性粒细胞胞浆抗体（ANCA），但这两种抗体均与骨髓增生异常综合征的血管炎无关。然而，有一份报告称在Sweet综合征（一种也发生于骨髓增生异常综合征的非血管炎性皮肤病）中发现了ANCA。
