Delmer A, Karmochkine M, Le Tourneau A, Bouscary D, Rio B, Perez E, Prudent J, Bonnet P, Audouin J, Diebold J
Service d'Hématologie, Hôtel-Dieu, Paris.
Ann Med Interne (Paris). 1990;141(2):123-8.
Four cases of plasma cell type Castleman's disease (CD) are described. Two patients had localized forms (one mediastinal and the other mesenteric) and presented systemic manifestations associated with hypergammaglobulinemia and severe anemia. In both cases, the lesions were revealed by computerized tomography scans and cures were obtained by the complete surgical removal of the masses, which led to the rapid disappearance of the systemic manifestations. The other 2 patients had the multicentric form of CD and presented more extensive clinical and biological symptoms. One of these developed severe peripheral neuropathy and endocrine anomalies during the late phase of his disease, which led us to discuss the relationship between multicentric CD and the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change) syndrome first described in Japan. Three of our patients presented with hypochromic microcytic anemia too severe to be explained by an inflammatory syndrome alone, and was likely due to several mechanisms. The etiology of CD remains unknown. The histological characteristics of angiofollicular lymph node hyperplasia are among the most important criteria for the diagnosis of localized and multicentric forms of CD, which can easily be made on a lymph node biopsy. However, it must be noted that this lesion can also be observed (but only rarely) in HIV (human immunodeficiency virus) - infected patients. The localized form is always considered to be benign, but, to date, there is no formal argument definitively supporting the malignancy of the multicentric one, in spite of its clinical similarity to a lymphoproliferative syndrome.
本文描述了4例浆细胞型Castleman病（CD）。2例患者为局限性形式（1例为纵隔型，另1例为肠系膜型），并出现与高球蛋白血症和严重贫血相关的全身表现。在这两例中，病变通过计算机断层扫描发现，通过完整手术切除肿块获得治愈，这导致全身表现迅速消失。另外2例患者为多中心型CD，表现出更广泛的临床和生物学症状。其中1例在疾病后期出现严重的周围神经病变和内分泌异常，这使我们首次讨论多中心型CD与日本首次描述的POEMS（多发性神经病变、器官肿大、内分泌病、M蛋白、皮肤改变）综合征之间的关系。我们的3例患者还出现了低色素小细胞性贫血，严重程度无法仅用炎症综合征来解释，可能是由多种机制导致的。CD的病因仍不清楚。血管滤泡性淋巴结增生的组织学特征是诊断局限性和多中心型CD的最重要标准之一，通过淋巴结活检很容易做出诊断。然而，必须注意的是，在人类免疫缺陷病毒（HIV）感染患者中也可观察到这种病变（但非常罕见）。局限性形式一直被认为是良性的，但迄今为止，尽管多中心型CD在临床上与淋巴增殖综合征相似，但尚无确凿证据明确支持其为恶性。
