巨淋巴结增生的临床影像学诊断

目的探讨并总结Castleman病的临床及影像表现。方法回顾性分析我院2012年12月-2015年6月病理证实为Castleman病的8例患者的临床及影像资料,并回顾相关文献,总结其临床及影像学特征。6例进行CT平扫及增强扫描,1例行CT平扫,1例行MRI平扫。结果 8例中,男5例,女3例,年龄23-56岁,中位年龄31.5岁。8例病理类型均为透明血管型,均为单中心型。位于纵膈4例,右前胸壁1例,腹膜后1例,盆腔2例CT平扫均表现为单个类圆形或椭圆形的软组织肿块,边界清晰,2例有散在斑点状钙化,1例有点状钙化,1例有囊变坏死等特征;增强扫描均表现为动脉期明显强化,并持续至静脉期和延迟期,其中1例肿块周围或内部可见增粗的血管影;MRI平扫T1WI为等信号,T2WI为明显高信号。结论 Castleman病较少见且易误诊,常表现为良性肿块特征,部分病灶伴有钙化,了解其临床及影像特征有助于检出本病。     

腹膜后局限型Castleman病的CT及MR表现

目的：分析腹膜后局限型Castleman病的影像学特征,并参考病理分型,提高对该疾病的认识。方法回顾性分析经手术病理证实的10例患者的影像学资料,10例均行CT平扫及增强扫描,5例行MR平扫及增强扫描,分析其影像学表现并总结其特征。结果本组病变病理包括透明血管型8例和浆细胞型2例。CT平扫为等密度病灶,4例伴有钙化,1例伴有囊变,CT增强扫描示7例透明血管型病灶有明显持续强化,1例透明血管型病变与2例浆细胞型病灶呈轻度中度强化。5例行MR扫描,4例为透明血管型,1例为浆细胞型。平扫T1WI呈等或略低信号、T2WI呈高信号,DWI均为较高信号,增强扫描5例均有明显持续强化。结论腹膜后局限型Castleman病以透明血管型居多,影像学表现与病理分型密切相关,透明血管型明显持续强化,且钙化常见,而浆细胞型无明显特异性。     

Castleman病的多层螺旋CT诊断

目的:总结Castleman病的多层螺旋CT(MSCT)表现,以提高对该病的认识和诊断水平。方法:收集经手术病理证实的Castleman病11例,回顾性分析其MSCT平扫和增强表现。结果:颈部1例,胸部6例,腹部4例。6例为局灶型,5例为多中心型。病理分型透明血管型9例,浆细胞型2例。病理上局灶性Castleman病多为透明血管型,平扫表现为较大的软组织密度影,可伴钙化和囊变,增强后中度至明显强化,强化方式与主动脉同步。多中心型Castleman病多为浆细胞型,病灶体积不大,表现为多个软组织结节,增强后轻至中度强化。结论:Castleman病的CT表现与病理类型密切相关,透明血管型的CT表现具有特征性。     

Fatal paraneoplastic pemphigus associated with a mediastinal tumor

Paraneoplastic pemphigus (PNP) is a rare life‐threatening autoimmune bullous skin disease which is an obligate paraneoplasma. A 34‐year‐old woman presented with recalcitrant stomatitis and a generalized lichenoid rash. A diagnosis of PNP was established based on clinical findings, immunofluorescence, histopathology and biochemistry. A localized mediastinal mass was found with CT imaging and excised. The histologic diagnosis was dendritic cell sarcoma. Despite removal of tumor and immunosuppressive therapy, the PNP progressed rapidly and the patient died of septic multiorgan failure.     

自体外周血干细胞移植治疗难治性Castleman病疗效观察并文献复习

目的观察自体外周血干细胞移植治疗难治性Castleman病（Castleman＇s disease,CD）的疗效。方法回顾性分析3例接受自体外周血干细胞移植治疗的难治性CD患者临床资料并复习相关文献。患者均为多中心性CD,中位年龄为42．7（31-57）岁,移植前淋巴结平均最大直径为30mm,预处理2例采用BEAM,1例采用司莫司汀＋依托泊甙＋马法兰。采集物CD34’细胞中位数为7．47（5．51-10．00）×10 6／kg,单个核细胞（MNC）中位数为3．16（2．48-4．01）×10 8／kg。结果患者接受移植前均为难治性CD。所有患者均移植成功,中性粒细胞绝对值（ANC）〉0．5×10 9／L的中位时间为移植后12（11-13）天,血小板〉20×10 9／L为移植后13（12-15）天,移植后症状体征明显好转,淋巴结均消失,平均消失时间为21天。3例患者均完全缓解,至最近随访时间均未复发,中位生存时间超过2年。而联合化疗后疾病进展的患者病死率达55．6％,CR率为16．7％,与自体干细胞移植相比存活率明显下降。结论自体外周血干细胞移植治疗疗效良好,可作为难治性CD的挽救治疗。     

Historical and pathological overview of Castleman disease

Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On the other hand, numerous clinical findings and etiologies make the disease challenging to understand. The origin of the disease is the hyaline vascular-type unicentric Castleman disease (UCD), first reported by Benjamin Castleman et al. in 1954. Although UCD is characterized by localized lesions and lack of symptoms, multicentric Castleman disease (MCD) with multiple lesions and systemic symptoms was reported by Frizzera in 1983. MCD is further divided according to KSHV/HHV8 infection status. In KSHV/HHV8-related MCD, viral infection signals lead to excessive cytokine production, and cause clinical and pathologic abnormalities. Some cases of plasma cell-type KSHV/HHV8-negative MCD can be found in association with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes), which is a paraneoplastic syndrome. The others are idiopathic MCD, which are currently considered a heterogeneous group of diseases with overlapping pathological and clinical features. In this article, we summarize the historical evolution of Castleman disease to help understand the disease concept. We also review the latest ideas and definitions of the subtypes within the MCD spectrum and summarize the histopathological findings.     

Multidisciplinary diagnosis and treatment of recurrent follicular dendritic cell sarcoma in abdomen: A case report

Rationale:Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment.Patient concerns:A 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP.Diagnoses:PNP with FDCS.Interventions:The patient received gamma globulin infusion, took anlotinib, and underwent plasma exchange therapy.Outcomes:The skin lesions recovered and there was no evidence of tumor recurrence.Lessons:The diagnosis and management of PNP with FDCS require close cooperation among surgeons, dermatologists, hematologists, otolaryngologists, oncologists, radiologists, pathologists, and respiratory doctors. The interesting clinical manifestations of this patient provide a multifaceted approach to the investigation of the interactions among FDCS, Castleman disease, and PNP.     

Primary effusion lymphoma metachronous to multicentric Castleman disease in an immunocompetent patient

Human Herpesvirus 8 (HHV8) has been associated with a wide spectrum of B-cell lymphoproliferative disorders, including Primary Effusion Lymphoma, Multicentric Castleman Disease, HHV8-positive Diffuse Large B-cell Lymphoma, not otherwise specified and germinotropic lymphoproliferative disorder. The association of different HHV8-related lymphoproliferative disorders is described in immunodeficient patients. We report a case of Primary Effusion Lymphoma metachronous to Multicentric Castleman Disease in an immunocompetent patient.