颈部Castleman病临床报道暨文献回顾

Castleman病（Castleman??s disease,CD）是一种相当罕见和复杂的疾病,本文通过对1例40岁女性发生于右侧颈部胸锁乳突肌内侧Castleman病例的诊治,并复习相关文献,对该病的发病率、临床表现、诊断与鉴别诊断、治疗及预后作回顾性报道。CD发病率较低,既往未见临床统计发病率,并且缺乏特征性的临床表现。临床上分两型：局灶型和多中心型,两者诊断均较为困难,术前CT检查对诊断本病有一定帮助,组织病理学检查或淋巴结活检有益于早期诊断。治疗效果基于组织病理学检查和临床分类。预后因临床分型有所不同,局灶型预后较好,而多中心型预后较差,两者均存在一定复发率。     

Castleman病的影像学诊断

目的探讨Castleman病（CD）的CT及MRI影像表现,提高对其的认识。方法回顾性分析8例经手术及穿刺病理证实CD患者的CT及MRI资料,并与病理结果对照分析。结果 8例CD患者中,6例为局灶型,其中5例为透明血管型、1例为浆细胞型;2例为多中心型,均为浆细胞型。CT检查8例,平扫5例病灶表现为密度均匀的软组织肿块,与肌肉相比呈等密度;3例病灶内有小斑片状低密度。3例伴点、条状钙化。CT增强扫描5例透明血管型动脉期显著强化,强化程度接近同层面血管,门静脉期及延迟期持续强化。其中3例平扫斑片状低密度区,增强后2例呈相对低密度改变,1例呈更高密度。3例浆细胞型中等均匀强化。MRI检查4例,均为透明血管型,T1WI病灶呈稍低信号,T2WI呈高信号。其中1例T2WI中央斑片状亮高信号,增强扫描强化方式与CT一致。结论 CD的影像学表现与临床及病理分型密切相关,局灶型病例多为透明血管型,影像学表现具有一定特征;多中心型病理多为浆细胞型,影像学表现无明显特征,确诊依赖组织病理学。     

Therapeutic options for human herpesvirus-8/Kaposi’s sarcoma-associated herpesvirus-related disorders

Human herpesvirus-8/Kaposi’s sarcoma-associated herpesvirus infection is associated with three proliferative disorders in immunocompromised patients – Kaposi’s sarcoma, primary effusion lymphoma and multicentric Castleman’s disease. These disorders often develop in patients with advanced AIDS who present a number of therapeutic challenges, underscoring the importance of continuing efforts dedicated to basic and clinical research in this field. In the era of highly active antiretroviral therapy, the incidence of AIDS and Kaposi’s sarcoma has considerably decreased, presumably due to enhanced anti-Kaposi’s sarcoma-associated herpesvirus immune responses, whereas the situation with primary effusion lymphoma and multicentric Castleman’s disease is more complex. Based on advances in the understanding of Kaposi’s sarcoma-associated herpesvirus-related disorders and availability of antiretroviral agents, current and future therapeutic approaches will be discussed.     

Iron therapy resistant microcytic anaemia in a 13-year-old girl with Castleman disease

We describe the case history of a 13-year-old girl with chronic fatigue and prolonged microcytic anaemia. She received oral iron since the age of 11 but failed to respond to it. Laboratory studies revealed elevated C-reactive protein and hypergammaglobulinaemia. A large solitary mesenterial lymph node could be demonstrated by ultrasonography and CT. A diagnosis of Castleman disease was suspected and confirmed histologically. After surgical removal of the lymphoma the patient recovered completely. Conclusion Castleman disease should be considered in cases of chronic fatigue, unexplained fever, microcytic anaemia and hypergammaglobulinaemia. Received: 10 February 1996 Accepted: 23 March 1996     

Multicentric castleman disease in a child with primary immunodeficiency

Multicentric Castleman disease is a rare lymphoproliferative disorder mostly seen in adults with HIV. It presents with fever and systemic symptoms and is extremely uncommon in children. We describe a novel case of multicentric Castleman disease associated with primary immunodeficiency (common variable immunodeficiency) and discuss pathophysiologic mechanisms and recent advances in understanding this disease. Pediatr Blood Cancer. 2010;55:1198–1200. © 2010 Wiley‐Liss, Inc.     

Fine-needle aspiration cytology of Castleman disease: case report with review of the literature

Organs involved by Castleman disease (CD) may be investigated by fine-needle aspiration cytology. No specific cytomorphological criteria are currently described for a definitive diagnosis. The cytological features of three fine-needle aspirations from three different lymph nodes of a patient with histologically confirmed CD of the hyaline-vascular type are herein reported, with a review of the literature. The fine-needle aspirations showed branching capillaries associated with fragments of germinal center. Review of the literature yielded 12 other case reports with over half describing similar findings. Because branching hyalinized small blood vessels penetrating follicular germinal center are characteristic of CD of the hyaline-vascular type on histology, this finding in fine-needle aspirates should raise that diagnostic possibility.     

Castleman病8例临床分析

目的探讨Castleman病的临床特征、实验室检查特点及治疗方案。方法回顾性分析8例Castleman病的临床表现、实验室检查、治疗及疗效评价。结果 8例患者中4例首发症状有乏力,3例有低蛋白血症,3例有蛋白尿或者肾损害,2例有感染,1例有肌损害。3例确诊为透明血管型,3例为浆细胞型,2例为混合细胞型。病理学提示淋巴结结构保持完整,滤泡增生明显,血管增生。结论 Castleman病临床表现无特异性,其诊断和分型主要依靠组织病理学,手术、化疗、放疗及生物治疗等多种方法可应用于该病的治疗。     

Predictors of response to anti-IL6 monoclonal antibody therapy (siltuximab) in idiopathic multicentric Castleman disease: secondary analyses of phase II clinical trial data

Siltuximab is the only US Food and Drug Administration-approved treatment for idiopathic multicentric Castleman disease (iMCD), a rare haematological disorder associated with substantial morbidity and mortality. Although siltuximab induces a response in a significant proportion of iMCD patients via interleukin 6 (IL6) neutralization, it is not universally effective. To develop a predictive model of response, we performed an in-depth analysis of 38 baseline laboratory parameters in iMCD patients from the phase II siltuximab trial who met criteria for treatment response or treatment failure. Univariate analyses identified eight baseline laboratory parameters that were significantly different between responders and treatment failures: albumin, immunoglobulin G (IgG), immunoglobulin A, C reactive protein (CRP), fibrinogen, haemoglobin, sodium and triglycerides. Stepwise logistic regression analysis of these candidate parameters identified a top performing model that included fibrinogen, IgG, haemoglobin and CRP. Based on cross-validation of the final multivariate logistic regression model, the model accurately discriminated responders from those who failed treatment (area under the receiver operator characteristic curve 0·86, 95% confidence interval: 0·73–0·95). All four laboratory parameters associated with response to siltuximab have biological relationships with IL6 and acute inflammation. Our model suggests that iMCD patients with laboratory evidence of an inflammatory syndrome are the best candidates for siltuximab therapy.     

Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?

Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV‐8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy. He had severe paraneoplastic pemphigus and was treated with tocilizumab, an anti‐interleukin‐6 receptor monoclonal antibody that has demonstrated good response rates in multicentric Castleman disease but demonstrated no clinical response despite 2 months of treatment. Our report is the first to describe a lack of response to tocilizumab in the rare setting of refractory UCD and discuss potential for distinct disease biology.