Beylot J, Malou M, Doutre M S, Beylot C, Broustet A, Reiffers J, Lacoste D, Turner K
Service de médecine interne, Hôpital Saint-André, Bordeaux.
Rev Med Interne. 1989 Nov-Dec;10(6):509-14. doi: 10.1016/s0248-8663(89)80067-0.
We report 12 cases of leucocytoclastic vasculities associated with myelocytic (7 cases) or lymphocytic (5 cases) blood diseases. The clinical features, laboratory abnormalities and pathological findings are presented. In all cases a drug-induced and/or infective origin could be ruled out, and patients with cryoglobulinaemia were excluded. As in other cases found in the literature, in some patients vasculitis was present several weeks or months before the blood disease was discovered, while in others both conditions developed simultaneously or vasculitis appeared in the course of the blood disease. None of these three possibilities seemed to make the prognosis worse. The pathogenesis of the vasculitis-malignant blood disease association is uncertain. Immune complexes of tumoral origin undoubtedly have some responsibility, but some deficiency of phagocytic cells or chemotactic factors also play a role. Cases such as ours enable the significance of vasculitis to be understood when it occurs in a patient with malignant blood disease, but above all they should prompt investigations for malignant blood disease in patients with cutaneous and/or systemic vasculitis, as is being done in patients with pyoderma gangrenosum or Sweet's syndrome.
我们报告了12例与髓细胞性(7例)或淋巴细胞性(5例)血液疾病相关的白细胞破碎性血管炎。文中呈现了其临床特征、实验室异常及病理表现。所有病例均排除了药物诱导和/或感染性病因,且排除了冷球蛋白血症患者。正如文献中其他病例一样,在一些患者中,血管炎在发现血液疾病数周或数月前就已存在,而在另一些患者中,两种疾病同时发生,或者血管炎在血液疾病病程中出现。这三种情况似乎均未使预后变差。血管炎与恶性血液病关联的发病机制尚不确定。肿瘤来源的免疫复合物无疑有一定作用,但吞噬细胞或趋化因子的某些缺陷也起作用。像我们这样的病例有助于理解血管炎在恶性血液病患者中发生时的意义,但最重要的是,它们应促使对皮肤和/或系统性血管炎患者进行恶性血液病的检查,就如同对坏疽性脓皮病或Sweet综合征患者所做的那样。