Gherardi R K, Bélec L, Fromont G, Divine M, Malapert D, Gaulard P, Degos J D
Département de Pathologie, Hôpital Henri Mondor, Créteil, France.
Blood. 1994 May 1;83(9):2587-93.
To evaluate a possible implication of cytokines in the pathogenesis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, we studied five consecutive patients with this condition, of which four had sclerotic bone lesions and four had multicentric Castleman's disease. Interleukin-1 beta (IL-1 beta) and IL-6 serum levels were determined in these patients (13 serum samples) and in patients with multiple myeloma (5) and Waldenström's macroglobulinemia (5). In situ hybridization of the relevant mRNAs was performed on lymph node specimens of two patients with POEMS syndrome who had Castleman's disease. Elevated serum levels of IL-1 beta (13/13 samples), and IL-6 (7/13 samples) were found in patients with POEMS syndrome. In the other patients, serum IL-1 beta was undetectable or slightly increased and IL-6 was elevated in a single patient with Waldenström's macroglobulinemia. Abundant IL-1 beta mRNA-producing cells were present in interfollicular spaces in the two tested patients, while IL-6 mRNA-producing cells were rare. We conclude that IL-1 beta and IL-6 serum levels may be chronically elevated in patients with POEMS syndrome, and that lymph node may be one site of IL-1 beta overproduction. These results are in keeping with the hypothesis that cytokines mediate systemic manifestations of POEMS syndrome.
为评估细胞因子在多神经病、器官肿大、内分泌病、M蛋白和皮肤改变(POEMS)综合征发病机制中的潜在作用,我们对连续5例患有该疾病的患者进行了研究,其中4例有硬化性骨病变,4例有多中心性Castleman病。测定了这些患者(13份血清样本)以及多发性骨髓瘤患者(5例)和华氏巨球蛋白血症患者(5例)的白细胞介素-1β(IL-1β)和IL-6血清水平。对2例患有Castleman病的POEMS综合征患者的淋巴结标本进行了相关mRNA的原位杂交。在POEMS综合征患者中发现血清IL-1β水平升高(13/13样本),IL-6水平升高(7/13样本)。在其他患者中,血清IL-1β检测不到或略有升高,在1例华氏巨球蛋白血症患者中IL-6升高。在2例受试患者的滤泡间区存在大量产生IL-1β mRNA的细胞,而产生IL-6 mRNA的细胞很少。我们得出结论,POEMS综合征患者的血清IL-1β和IL-6水平可能长期升高,并且淋巴结可能是IL-1β过度产生的一个部位。这些结果与细胞因子介导POEMS综合征全身表现的假说一致。