多中心型Castleman病临床病理分析并文献复习

目的:探讨多中心型Castleman病（multiple Castleman disease,MCD）的临床表现、病理学特征、鉴别诊断、病因及治疗,并分析其预后。方法:对9例多中心型Castleman病的临床资料、病理形态学特征进行分析,并行免疫组化检测及相关文献复习。结果:9例多中心型Castleman病例中,透明血管型5例,浆细胞型2例,透明血管浆细胞混合型2例。免疫组化无特征性表现,但可辅助诊断及鉴别诊断。9例多中心型患者均有完整随访资料,经治疗后其中有1例复发,1例死于心脏疾病,其余患者症状均有所缓解。结论:多中心型Castleman病是一种少见的淋巴组织增生性疾病,临床易与其他疾病相混淆,确诊需依靠病理学检查,准确的临床分型和病理类型对患者的治疗和预后具有重要意义。     

Castleman病转化为霍奇金淋巴瘤一例并文献复习

 目的　分析Castleman病的病因及发病机制、临床及病理特点、诊断、治疗及预后。方法　对1例Castleman病转化为霍奇金淋巴瘤的病例进行分析并进行文献综述。结果　Castleman病是一种以滤泡增生和毛细血管增生为组织学特征的淋巴结良性增生性疾病。根据病理学特点分为透明血管（HV）型、浆细胞（PV）型及兼有两者特点的混合细胞型。其临床表现复杂多变,除淋巴结肿大外尚可有全身表现及多系统受累症状,早期确诊主要靠组织病理学诊断,病理类型不同预后也不同,局灶型（LCD）患者预后良好,可能长期带病生存,多中心型（MCD）具有侵袭性,预后差。结论　Castleman病不同的病理类型其临床表现及预后也不同。     

多中心型 Castleman 病的CT诊断

目的:提高对多中心型Castleman病CT征象的认识.方法:分析3例经手术病理或穿刺活检证实为多中心型Castleman病的CT征象,总结其特征.结果:3例均为多中心分布,病理分型为浆细胞型1例,混合型2例.动态增强扫描病灶均表现轻度强化,延迟期表现为持续强化.结论:多中心型Castleman病的多病灶性分布,轻度强化及分枝状钙化等征象可提示该病的诊断,确诊需要靠穿刺活检及实验室检查.     

Castleman氏病14例报道及文献复习

目的：加强对Castleman氏病（CD）的认识，提高CD的诊治水平。方法：回顾分析14例CD患者临床特征及诊疗情况，并复习文献。结果：14例中临床分型局灶型8例，淋巴结活检、组织病理学均为透明血管型；多中心型6例，其中组织病理学为浆细胞型4倒、混合型2例。14例患者临床表现多样，缺乏特异性，早期多误诊为其他疾病，确诊有赖于组织病理学检查。8例局灶型患者行手术切除，至今无复发；6例多中心型患者予糖皮质激素或联合化疗，病情均有缓解。结论：CD临床表现复杂，淋巴结活检是早期诊断的关键，依据组织病理学和临床分期，制定治疗方案可获较佳疗效。     

多中心透明血管型Castleman病合并肾病综合征一例并文献复习

 【摘要】 目的 探讨多中心透明血管型Castleman病并发肾病综合征的临床表现及病理特点,提高对Castleman病肾脏损害的认识。方法 回顾性分析一例多中心透明血管型Castleman病并发肾病综合征患者的临床资料,结合国内外文献复习对该病的诊治经过、肾脏活检病理进行综合分析。结果 患者为多中心型透明血管型Castleman病且疾病控制良好, 10年后复发且并发肾损害。早期临床表现为蛋白尿和血尿,渐发展为大量蛋白尿、低蛋白血症、高脂血症和水肿。经含有激素的化疗方案3周期治疗,肿大淋巴结缩小,肾病综合征的各项指标渐恢复至正常水平。结论 透明血管型Castleman病引起肾脏损害较少见, 早期临床表现不典型, 应引起临床医师的重视,尽早行肾脏穿刺活检, 以期尽早明确诊断和治疗。     

多中心型Castleman病的CT诊断

目的：提高对多中心型Castleman病CT征象的认识。方法：分析3例经手术病理或穿刺活检证实为多中心型Castleman病的CT征象,总结其特征。结果：3例均为多中心分布,病理分型为浆细胞型1例,混合型2例。动态增强扫描病灶均表现轻度强化,延迟期表现为持续强化。结论：多中心型Castleman病的多病灶性分布,轻度强化及分枝状钙化等征象可提示该病的诊断,确诊需要靠穿刺活检及实验室检查。     

Castleman病6例临床病理分析及文献复习

目的 提高对Castleman病的诊断和治疗水平。方法 报告6例Castleman病,进行分析并复习相关文献。结果 男性4例,女性2例,年龄8岁～54岁。临床分型：局灶型2例,多中心型4例。病理学分型：透明血管型5例,浆细胞型1例。局灶型经手术或术后放疗均治愈。多中心型中3例经干扰素或化疗联合激素治疗后病情稳定,1例出现复发。结论 该病诊断主要依靠病理学确定。手术、化疗、抗病毒治疗可控制其发展。     

Castleman病十例并文献复习

目的 探讨Castleman病的临床病理特点和预后相关因素.方法 对安徽省池州市人民医院10例Castleman病进行回顾性临床病理特征分析,包括临床资料、组织形态学,并复习相关文献.结果 10例患者中,8例男性,2例女性,年龄范围6～74岁.8例单中心型Castleman病(UCD)均为透明血管型,临床症状为淋巴结无痛性肿大,颈部4例,纵隔、肠系膜、颌下、腹膜后各1例,单纯手术切除;2例多中心型Castleman病(MCD)均为浆细胞型,患者全身多处淋巴结肿大伴呼吸系统症状,CHOP方案化疗6个疗程后症状缓解.10例Castleman病随访8～115个月,无复发.结论 Castleman病少见,多为UCD透明血管型,单纯手术可治愈;极少数为MCD浆细胞型,其临床综合治疗方案尚须进一步探讨.     

Castleman病的影像和临床病理分析

[目的]探讨Castleman病的影像和临床病理表现。[方法]回顾分析6例经病理证实的Castleman病患者的影像和临床病理资料。[结果]6例Castleman病局限性3例,均位于胸部;病理分型为透明血管型,CT表现为明显强化的软组织肿块,增强CT值为128～157Hu,平均140Hu,1例肿块内见条状囊变及分隔。弥漫性3例,均位于腹部多发,其中1例伴双侧锁骨上、颈部淋巴结肿大;病理分型均为浆细胞型,超声检查表现为边缘光整的低回声区,肿块周边及内部见异常血流信号。CT表现为强化较明显的多发软组织肿块,增强CT值为95～103Hu,平均98Hu;1例MRI检查表现为:T1WI肿块呈等低信号,T2WI肿块呈高信号,增强扫描肿块强化较明显,呈边缘环状强化。[结论]局限性Castleman病以透明血管型多见,表现为明显强化的软组织肿块,肿块中心簇状分布的分支状钙化为特征;弥漫性Castleman病以浆细胞型多见,主要表现为多发强化软组织肿块,强化程度低于透明血管型,诊断需结合临床和病理资料。     

局灶性Castleman病17例报告并文献复习

背景与目的:Castleman病(Castleman;sdisease,CD)又称血管滤泡性淋巴组织增生或巨大淋巴结增生,是一种少见的原因未明的反应性淋巴结病。本研究报告17例局灶性Castleman病(local Castleman;s disease,LCD)的临床特点和疗效,结合复习相关文献,以提高对LCD的诊治水平。方法:回顾性分析中山大学肿瘤防治中心从1995年8月至2006年7月收治的17例LCD患者临床资料。结果:无临床症状患者14例,有临床症状者3例,淋巴结呈单个或多个聚集,淋巴结最大径1.2～10.4cm,其中11例位于颈部,3例位于纵隔,位于肺部、肠系膜、肾上腺区各1例。其中透明血管型15例,浆细胞型1例,混合型1例,均经术后病理确诊。1例透明血管型CD出现脾大、白蛋白降低(25.6g/L)、球蛋白升高(80.0g/L)、大便潜血( );1例浆细胞型CD出现中度贫血(95.0g/L)、尿蛋白( )、大便潜血( );余15例患者实验室检查结果均在正常范围内。17例患者均行肿物切除术,术后失访2例,余15例随访1～129个月,中位随访时间25个月,现均生存,无肿瘤复发。结论:LCD主要表现为单一部位的淋巴结肿大,以透明血管型为主,多无临床症状和实验室检查异常结果。CT检查对诊断有一定帮助,但确诊仍靠病理。手术切除疗效好,术后可长期生存。     

The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature

BACKGROUND: Castleman's disease (CD), or angiofollicular lymph node hyperplasia, creates both diagnostic and therapeutic dilemmas for most physicians. For patients with this rare and poorly understood disease, the optimal therapy is unknown. The authors report their experience during the years 1986-1997 with this uncommon clinicopathologic entity. METHODS: Sixteen patients with a histologic diagnosis of CD were identified in the pathology database. Unicentric disease was defined as a solitary mass. Multicentric disease compromised patients with widespread lymphadenectomy. Clinical, radiologic, and laboratory data were analyzed to evaluate treatment response. RESULTS: The study group consisted of 16 patients classified into 3 clinicopathologic groups: hyaline-vascular, plasma cell, and "mixed." Of those patients who underwent complete surgical excision of a unicentric hyaline-vascular CD mass (n = 8), all remain symptom free without clinical or radiographic recurrence. Two patients remain asymptomatic following partial resection or radiation therapy for an unresectable unicentric hyaline-vascular CD mass. Two patients with multicentric hyaline-vascular CD are currently in complete remission following adjuvant therapy. Multicentric plasma cell CD was present in a single patient. This patient (who underwent surgical and systemic therapy) died of disease within 4 months of presentation. Three patients with unicentric hyaline-vascular/plasma cell-CD remain symptom free following either complete resection or observation. CONCLUSIONS: The authors recommend surgical resection for patients with the unicentric variant of CD. Surgical removal of a unicentric mass of hyaline-vascular or hyaline-vascular/plasma cell type is curative. Partial resection, radiotherapy, or observation alone may avoid the need for excessively aggressive therapy. Patients with multicentric disease, either hyaline-vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined.     

Atypical lymphoproliferative disorders: Castleman's disease. Case report and review of the literature

Castleman's disease (CD) is a rare atypical lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with striking vascular proliferations. CD is categorized as being either localized or disseminated and further subdivided into hyaline-vascular, plasma cell, or mixed histopathological patterns. Here we report a case of CD in a 15-year-old girl who presented with a solitary asymptomatic hyaline-vascular mass in the right supraclavicular space. In addition, we discuss the pathogenesis, clinical features and reported co-morbidities of unicentric and multicentric CD and evaluate effective treatment strategies based on the results of lymph node biopsy and careful staging. Surgical excision is curative for the localized variants of CD, either hyaline-vascular or plasma cell type. If complete resection is not possible, partial resection or radiotherapy may be useful to control possible systemic manifestations. Multicentric CD, regardless of the histological subtype, is a more aggressive clinical entity, commonly with a chronic or rapidly fatal course. Patients with multicentric CD do not benefit from surgical treatment and should be candidates for systemic therapy (steroids, combination chemotherapy, novel therapies), although this is still in a fairly experimental phase.     

Retrospective Study of Castleman＇s Disease： A Report of Fourteen Cases and Review of the Literature

OBJECTIVE To enhance the understanding of Castleman＇s disease （CD）, and to improve its diagnosis and management. METHODS Clinical features and related information on diagnosis and treatment of 14 cases of CD were retrospectively analyzed and the literature reviewed. RESULTS Based on the clinical classification, localized CD was found in 8 of the 14 cases. Both the results of lymph node biopsy and histopathology indicated they were a hyaline-vascular type. The multicentric type CD was detected in 6 cases, among which 4 were plasma cell type and 2 mixed type based on histopathologic examination. There were a variety of clinical situations in the 14 cases, with a lack of specificity. They were previously misdiagnosed as other diseases, and final diagnosis depended on a histopathologic examination. The 8 patients with localized CD underwent excision, without recurrence up to now. The 6 patients with multicentric-type CD were treated with glucocorticoids or combined chemotherapy, and all achieved remission. CONCLUSIONS CD has complicated clinical manifestations and is difficult to diagnose. Lymph node biopsy is important for early diagnosis. An optimal curative effect can be achieved with a suitable therapeutic option, based on histopathology and clinical classification.     

A case report of multicentric Castleman's disease with simultaneous Epstein-Barr virus infection.

Castleman's disease is a rare B-cell lymphoproliferative disorder of unknown etiology. In this report we describe a 54-year-old woman with a 10-year history of asymptomatic bilateral, multiple cervical lymph node enlargements. She was not evaluated by lymph node biopsy during this period. She had been well until four months previously. The patient presented with multiple enlarged lymph nodes and systemic symptoms including fever, sweats, weight loss, and anorexia. Two lymph nodes were biopsied, yielding a diagnosis of multicentric Castleman's disease (MCCD) of mixed hyaline-vascular and plasma cell type histology. Serologic studies revealed the simultaneous presence of an acute Epstein-Barr virus (EBV) infection. She experienced an aggressive clinical course with a fatal outcome.     

透明血管型Castleman 病的CT及MRI表现

目的:探讨透明血管型Castleman 病的CT 及MRI 影像表现,旨在提高对该病的诊断水平。方法:回顾性分析4例经手术病理证实的透明血管型Castleman 病的CT 及MRI 资料,总结其特征。结果:左锁骨上窝1 例,纵隔1例,肠系膜1 例,肝胃间隙1例,4例临床类型均为局限型,4例病理分型均为透明血管型。 CT表现为椭圆形软组织肿块,平扫与肌肉相比呈等密度,动态增强4例肿块动脉期明显强化,静脉期及延时期均表现为持续强化。MRI 检查T1WI 病灶呈等信号,T2WI 呈高信号,增强表现与CT 近似。结论: 透明血管型Castleman 病有一定的CT和MRI影像学特征,具有较高的诊断价值,但需病理性确诊。     

Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric mixed-type Castleman's disease

Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome is a rare multi-systematic disorder of uncertain etiology, if associated with multicentric Castleman's disease, it can lead to a more serious condition. We here presented a case of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome in a 37-year-old male patient who initially presented with progressive lower limb weakness accompanied by pain, low skin temperature, and hyperpigmentation. He was admitted with increasingly serious dyspnea and lower leg edema. Fluid of serous cavities in the patient were also indicated in ultrasonic inspection and X-ray. Furthermore, biopsy of a left axillary lymph node showed mixed hyaline-vascular and plasma cell type of multicentric Castleman's disease. Administration of bortezomib (Velcade) (1.3 mg/m(2) on days 1, 4, 8 and 11 of a 21-day cycle) combined with thalidomide (100 mg/day and 21-day cycle) dramatically improved the condition of this disease. Of note, in our study, combination therapy of bortezomib and thalidomide successfully improved the condition of the patient with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease, suggesting that the combination therapy may be an effective therapeutic strategy for the intractable polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease.     

Fifty years of multicentric Castleman's disease

Benjamin Castleman first described multicentric Castleman's disease (MCD) in a series of cases in 1954. Interest in MCD has grown in recent years following an association with human immunodeficiency virus (HIV) infection. Castleman's disease is separated into localized disease and MCD. The latter is characterized by polylymphadenopathy and multiorgan involvement. Histologically, Castleman's disease is divided into the hyalinized vascular form and a plasma cell variant, the former being more common in localized disease and the latter more common in MCD. MCD is associated with Kaposi's sarcoma herpesvirus (KSHV) infection, which is alternatively termed human herpesvirus 8 (HHV8). This virus encodes a homologue of interleukin 6 (vIL 6), which may mediate some systemic features of MCD. The diagnosis of Castleman's disease is established by biopsy and treatment is often based on published case reports only, as there are no randomized trials of therapy. Surgery has less of a role in MCD than in localized disease, but debulking by splenectomy may be useful to alleviate haematological sequelae. Systemic treatments for MCD have included chemotherapy, anti-herpesvirus treatments to reduce the KSHV viral load, highly active antiretroviral therapy (HAART) to reduce HIV viraemia and latterly monoclonal antibodies against both IL 6 and CD20. The introduction of HAART has altered the natural history of HIV infection; however, its impact on MCD is difficult to ascertain. Optimization and consensus in treatment of these patients remains a target for the future.     

Treatment of multicentric Castleman's disease complicated by the development of non-Hodgkin's lymphoma with high-dose chemotherapy and autologous peripheral stem-cell support

Background: Castleman's disease or angiofollicular lymph node hyperplasia is a rare entity with a localized/unicentric or a generalized/multicentric presentation. Whilie surgery is curable for most localized presentations, there is limited information regarding the optimal management of the multicentric type. The latter type is associated with a poor prognoses and can be associated with the development of lymphoma and infections.Patients and methods: In this report we describe a case of multicentric Castleman's disease who failed steroids and chemotherapy and developed a follicular mixed lymphoma. He was treated with high-dose chemotherapy with autologous stem-cell support and remains disease at four years of follow-up.Conclusions: A long-term durable remission may be possible with high dose chemotherapy with stem-cell support. This treatment modality should be considered an option in the management of multicentric Castleman's disease.