Abstract：
Idiopathic multicentric Castleman disease (iMCD) is a polyclonal lymphoproliferative disorder characterized by constitutional symptoms, generalized lymphadenopathy, cytopenias, and multi-organ dysfunction due to excessive cytokines, notably Interleukin-6. Idiopathic multicentric Castleman disease is often sub-classified into iMCD-TAFRO, which is associated with thrombocytopenia (T), anasarca (A), fever/elevated C-reactive protein (F), renal dysfunction (R), and organomegaly (O), and iMCD not otherwise specified (iMCD-NOS), which is typically associated with thrombocytosis and hypergammaglobulinemia. The diagnosis of iMCD is challenging as consensus clinico-pathological diagnostic criteria were only recently established and include several non-specific lymph node histopathological features. Identification of further clinico-pathological features commonly found in iMCD could contribute to more accurate and timely diagnoses. We set out to characterize bone marrow (BM) histopathological features in iMCD, assess differences between iMCD-TAFRO and iMCD-NOS, and determine if these findings are specific to iMCD. Examination of BM specimens from 24 iMCD patients revealed a high proportion with hypercellularity, megakaryocytic atypia, reticulin fibrosis, and plasmacytosis across patients with both iMCD-NOS and iMCD-TAFRO with significantly more megakaryocytic hyperplasia (p = 0.001) in the iMCD-TAFRO cases. These findings were also consistent with BM findings from 185 published cases of iMCD-NOS and iMCD-TAFRO. However, these findings are relatively nonspecific as they can be seen in various other infectious, malignant, and autoimmune diseases.
摘要：
特发性多中心Castleman病（iMCD）是一种以体质症状为特征的多克隆淋巴增生性疾病，全身淋巴结病，血细胞减少,和多器官功能障碍由于过多的细胞因子，特别是白细胞介素-6。特发性多中心Castleman病通常分为iMCD-TAFRO，与血小板减少症（T）相关，anasarca(A),发热/C反应蛋白（F）升高，肾功能不全(R),和器官肿大（O），和iMCD没有另外指定(iMCD-NOS)，这通常与血小板增多和高球蛋白血症有关。iMCD的诊断具有挑战性，因为最近才建立了共识的临床病理诊断标准，并且包括几种非特异性淋巴结组织病理学特征。识别iMCD中常见的进一步临床病理特征可以有助于更准确和及时的诊断。我们着手表征iMCD中的骨髓（BM）组织病理学特征，评估iMCD-TAFRO和iMCD-NOS之间的差异，并确定这些发现是否特定于iMCD。对来自24例iMCD患者的BM标本的检查显示，细胞数量过多的比例很高，巨核细胞异型性,网状蛋白纤维化，iMCD-NOS和iMCD-TAFRO患者的浆细胞增多，在iMCD-TAFRO病例中，巨核细胞增生明显增多（p=0.001）。这些发现也与185例已发表的iMCD-NOS和iMCD-TAFRO病例的BM发现一致。然而,这些发现是相对非特异性的，因为它们可以在各种其他传染病中看到，恶性,和自身免疫性疾病。
