Castleman disease: surgical cure in pediatric patients

Castleman disease is a rare disorder characterized by lymphoid hyperplasia which rarely manifests in children. We present 2 cases which highlight both histologic variants of this disease, and provide suggestions regarding workup and treatment with the goal of making practitioners aware of Castleman disease in the differential diagnosis of a child presenting with vague symptoms.     

多中心性浆细胞型血管滤泡性淋巴结增生1例

患者男性，63岁，因多发的浅表淋巴结进行性肿大2个月，于2004年6月4日入院。患者2004年4月初无意中发现双颈部有杏仁大小的数枚结节，无发热、乏力、盗汗、疼痛等不适，未诊治。后又发现双锁骨上、腋下、右肘部有大小不等的肿物，近1个月生长迅速，直径约1．0cm-2．5cm。并感轻微乏力及体重下降，为进一步诊治而入院。即往体健。     

Castleman病2例

<正>Castleman病(CD)属淋巴增殖性疾病,又称巨大淋巴结增生或血管滤泡性淋巴组织增生,属原因未明的反应性淋巴结病之一。临床较为少见,术前诊断困难,我科收治2例Castleman病,手术切除病灶效果满意。现报告其临床资料,并复习相关文献,总结该病的诊断和治疗经验,分析归纳Castleman病的临床特点及诊疗方法,供临床借鉴。1病例报告例1:女性,49岁,因腹痛伴腹胀1个月入院,间断性右下腹胀痛,伴腹胀,无放射痛,无畏寒发热,无黄疸。PET/CT:右     

Lymphadenopathy associated with IgG4-related disease: Diagnosis & differential diagnosis

IgG4-related sclerosing disease, which now encompasses diverse organ-related disorders with various prior eponymic designations, may also present with solitary or multifocal lymph node enlargement. This review considers the histopathologic features of IgG4 lymphadenopathy (IgG4LAD), which has been subdivided by Cheuk & Chan into 5 microscopic subtypes. Those include variants that are typified by multicentric Castleman disease (MCD)-like changes, follicular hyperplasia, interfollicular lymphoplasmacytic proliferation, progressive transformation of germinal centers, and formation of inflammatory pseudotumor (IPT)-like lesions. All of them demonstrate an excess of IgG4-immunoreactive plasma cells in the inflammatory cell population. Differential diagnostic considerations for IgG4LAD include true MCD, true IPT, luetic lymphadenitis, Rosai-Dorfman disease, and inflammatory myofibroblastic tumor, among others. An interpretative distinction between malignant lymphoma and IgG4LAD is also crucial.     

Efficacy of rituximab in an aggressive form of multicentric Castleman disease associated with immune phenomena

Multicentric Castleman disease (MCD) is an uncommon lymphoproliferative disorder for which the best therapeutic option is not yet well established. Immune-related disorders are rare complications of MCD. We report on an MCD case in a 23-year-old patient with extensive abdominal involvement and associated immune hemolytic anemia and Raynaud phenomenon. He was negative for human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8). After 8 courses of the anti-CD20 monoclonal antibody (rituximab), the patient achieved complete remission. Interestingly, Raynaud phenomenon disappeared under treatment and no new hemolytic events occurred. Anti-CD20 antibody treatment could be an attractive therapeutic approach for MCD, mainly when immune-related disorders are associated.     

Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan

Objectives: To determine the tentative diagnostic criteria and disease severity classification for Castleman disease (CD) and describe the clinical and pathologic features among human herpesvirus 8 (HHV-8) negative idiopathic multicentric CD (iMCD) in the Japanese population.

Methods: We established the working groups for the research of CD in Japan and had meetings to discuss and define the tentative diagnostic criteria and disease severity classification for CD. We subsequently analyzed 142 patients classified into iMCD by using the nationwide Japanese patient registry.

Results: We proposed the preliminary diagnostic criteria and disease severity classification for CD based on our discussion. In addition, we made a proposal for the disease activity score. We identified clinical and pathological features of patients with iMCD diagnosed by these diagnostic criteria. In the disease severity classification, 37, 33 and 30% patients were categorized into mild, moderate and severe diseases, respectively.

Conclusion: This is the first proposal for diagnosis and classification of CD by the Japanese group. Further studies are required to validate whether they can distinguish CD from other inflammatory diseases and to determine their sensitivity and specificity.     

Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi

Introduction

Clinical reports of multicentric Castleman disease (MCD) from sub-Saharan Africa (SSA) are scarce despite high prevalence of HIV and Kaposi sarcoma-associated herpesvirus (KSHV). Our objective is to describe characteristics and survival for HIV-associated MCD patients in Malawi. To our knowledge, this is the first HIV-associated MCD case series from the region.

Methods

We describe HIV-positive patients with MCD in Lilongwe, and compare them to HIV-associated lymph node Kaposi sarcoma (KS) and non-Hodgkin lymphoma (NHL) patients treated at our centre. All patients were enrolled into a prospective longitudinal cohort study at a national teaching hospital and cancer referral centre serving half of Malawi''s 16 million people. We included adult patients≥18 years of age with HIV-associated MCD (n=6), lymph node KS (n=5) or NHL (n=31) enrolled between 1 June 2013 and 31 January 2015.

Results and discussion

MCD patients had a median age of 42.4 years (range 37.2–51.8). All had diffuse lymphadenopathy and five had hepatosplenomegaly. Concurrent KS was present for one MCD patient, and four had performance status ≥3. MCD patients had lower median haemoglobin (6.4 g/dL, range 3.6–9.3) than KS (11.0 g/dL, range 9.1–12.0, p=0.011) or NHL (11.2 g/dL, range 4.5–15.1, p=0.0007). Median serum albumin was also lower for MCD (2.1 g/dL, range 1.7–3.2) than KS (3.7 g/dL, range 3.2–3.9, p=0.013) or NHL (3.4 g/dL, range 1.8–4.8, p=0.003). All six MCD patients were on antiretroviral therapy (ART) with median CD4 count 208 cells/µL (range 108–1146), and all with HIV RNA <400 copies/mL. Most KS and NHL patients were also on ART, although ART duration was longer for MCD (56.4 months, range 18.2–105.3) than KS (14.2 months, range 6.8–21.9, p=0.039) or NHL (13.8 months, range 0.2–98.8, p=0.017). Survival was poorer for MCD patients than lymph node KS or NHL.

Conclusions

HIV-associated MCD occurs in Malawi, is diagnosed late and is associated with high mortality. Improvements in awareness, diagnostic facilities, treatment and supportive care are needed to address this likely under-recognized public health problem in SSA.     

Castleman病的诊断及治疗进展

?Castleman病（Castleman Disease,CD）又称巨大淋巴结增生,淋巴结错构瘤,良性巨淋巴瘤,血管滤泡淋巴组织增生,淋巴组织肿瘤样增生,是一种原因未明的反应性淋巴结病之一,临床较为少见。1956年Castleman等【1】正式报道一种局限于纵隔的肿瘤样肿块,组织学显示淋巴滤泡及毛细血管明显增生的疾病称为血管滤泡性淋巴结增生(vascular follicular lymphnode hyperplasia) ,1969年Flendring和Schillings【2】提出CD的另一形态学亚型,以浆细胞增生为特征,常伴全身症状。由于本病淋巴结肿大常十分明显,有时直径达10cm以上故又名巨大淋巴结增生（giant lymphnode hyperplasia）。由于多数CD患者的病理及临床表现均缺乏特异性改变,所以CD的诊断标准及分型十分必要。多中心Castleman病（MCD）与单中心Castleman病（UCD）存在显著差异,MCD常出现包括发热、乏力、胸腔积液、腹水等全身系统受累症状较典型,且受累淋巴结广泛,同时可出现肝脾肿大等。MCD主要包括两个亚类：人类疱疹病毒8（human herpesvirus 8,HHV8）相关的MCD【3,4】和特发性多中心Castleman病（idiopathic multicentric Castleman disease,iMCD）,目前尚未发现与iMCD相关的病因【5】。确定CD的分型需进行淋巴结活检、形态学检查等完整的临床评估,且对诊断至关重要。本文主要介绍CD的诊断及治疗进展。     

Castleman病65例诊治分析

目的 总结Castleman病的临床特点和治疗手段.方法 回顾2005-2012年连续收治的65例Castleman病患者的临床资料,总结分析局灶型Castleman病和多中心型Castleman病在临床表现、发病部位、实验室检查及治疗上的不同.结果 本组42例局灶型Castleman病多见于中青年,常为单发肿瘤,表现为无症状的局部淋巴结增大,病理分型以透明血管型(33/42,78.6％)为主,本组42例患者发现44枚肿瘤,以腹膜后腔(10/44,22.7％)、腹腔(10/44,22.7％)等深部多见,通过手术治疗可治愈.本组23例多中心型Castleman病多见于中年,表现为多发淋巴结肿大,无局部淋巴结疼痛及表面破溃,常伴有发热(9/23,39.1％)、贫血(9/23,39.1％)、脾大,实验室检查多见异常,病理分型主要为浆细胞型(14/23,60.9％)和昆合型(5/23,21.7％),本组23例患者发现66枚肿瘤,以颈部(16/66,24.2％)和股沟区(12/66,l8.2％)等浅表部位多见,主要采用CHOP化疗方案或手术联合CHOP化疗方案,本组21例患者获得完全或部分缓解,2例因化疗引起肺部感染死亡,能否耐受长期化疗是影响预后的重要因素.结论 局灶型和多中心型Castleman病临床特点不同,治疗方法也有差异.局灶型Castleman病通过手术可达到治愈目的;多中心型Castleman病则主要采用CHOP化疗方案,多数预后较好.