Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patients

Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV‐negative CD patients from four medical centers in southern China. The median age was 37 years. One hundred and twenty‐one patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline‐vascular for 132 patients (71.4%), plasma cell for 50 patients (27%), and mixed type for 3 patients (1.6%). The 5‐year overall survival (OS) of 185 CD cases was 80.3%. All UCD patients underwent surgical excision, whereas the treatment strategies of MCD patients were heterogeneous. The outcome for UCD patients was better than MCD patients, with 5‐year OS rates of 93.6% and 51.2%, respectively. In further analysis of the MCD subgroup, a multivariate analysis using a Cox regression model revealed that age, splenomegaly and pretreatment serum albumin level were independent prognostic factors for OS. This multicenter study comprising the largest sample size to date suggested that MCD is a distinct entity from UCD with a significantly worse outcome. Older age (≥40 years), splenomegaly, and hypoalbuminemia were risk factors for poorer MCD prognosis.     

Castleman病的影像表现与病理对照

目的探讨Castleman病的CT及MRI影像表现,提高对其的认识。方法回顾性分析9例经手术病理证实的Castleman病的CT及MRI资料,并与病理结果对照分析。结果颈部3例,纵隔3例,腹膜后2例,肠系膜1例,临床类型为局限型8例,弥漫型1例,病理分型为透明血管型8例,浆细胞型1例。CT平扫与肌肉相比呈等密度;动态增强CT扫描透明血管型8例,肿块动脉期明显强化,强化程度接近同层面大动脉,静脉期及延迟期均表现为持续强化,5例病灶周围或(和)病灶内可见增粗迂曲血管影。浆细胞型1例表现为肠系膜多个软组织结节,伴有肝脾肿大,呈轻度强化表现。MRI检查3例均为透明血管型,T1WI病灶呈稍低信号,T2WI呈高信号,增强表现与CT近似。结论影像表现与病理分型关系密切;局限性边缘清楚的等密度结节,增强扫描动脉期明显强化,且强化延续时间长,灶周增粗迂曲血管影,分支状或斑点状钙化等为透明血管型相对影像学特点,弥漫浆细胞型无特征性影像学表现,确诊依赖组织病理学。     

Castleman’s disease of the left parotid gland: a case report

Castleman’s disease is a very rare heterogeneous group of lymphoproliferative disorders characterized by non-neoplastic growths. It is unknown about the pathophysiology of the Castleman’s disease. Previous studies demonstrated that Castleman’s disease can be divided into two groups according to clinical classification, including unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). The hyaline vascular type is most common in the head and neck, and is abbreviated as UCD. In the present case, a woman complained that a mass in her parotid gland was growing and it was painless seven months ago. The computed tomography (CT) showed that the superficial lobe of the parotid gland on the left had an elliptical soft tissue density shadow, about 2.5×3.5 cm, with clear boundaries and no obvious abnormalities in the surrounding bone. The CT scan showed no obvious abnormalities in the shape and density of the right parotid and bilateral submandibular glands. After the operation, combined with the results of immunohistochemistry, the final diagnosis was Castleman tumor. The patient recovered smoothly, after the operation and during follow-up. The patient maintained good health without recurrence or metastasis.     

Liver cirrhosis in a child associated with Castleman's disease: A case report

BACKGROUND Castleman's disease(CD)is a lymphoproliferative disorder.TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia,anasarca(generalized edema and pleural effusion),fever(pyrexia),reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes,and organomegaly(such as hepatosplenomegaly and multiple lymphadenopathies);TAFRO syndrome is mainly reported in Japanese patients.To our knowledge,this is the first pediatric case report detailing a CD-associated disorder progressing to cirrhosis.CASE SUMMARY A 10-year old male patient presented with fever and anemia.Six months before hospitalization,he had remarkable abdominal distention.Subsequently,he visited a clinic for a fever that lasted 5 d.The physical findings were marked hepatosplenomegaly and cervical lymphadenopathy.A blood test revealed leukocytosis,microcytic anemia,aspartate aminotransferase-dominant transaminase elevation,high levels of C-reactive protein,polyclonal hypergammaglobulinemia,and high levels of interleukin-6 and vascular endothelial growth factor.Abdominal contrast computed tomography and magnetic resonance imaging suggested cirrhosis,which was confirmed by liver histology.Histological findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles,which is compatible with lymph node histology in TAFRO syndrome.Prednisolone was not effective in reducing the patient’s symptoms;therefore,the patient was prescribed tocilizumab.To date,the patient remains free of fever and continues to receive tocilizumab.CONCLUSION We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.     

超声对Castleman病的诊断价值研究

目的总结Castleman病(CD)的超声表现及病理学特点,提高对该病的诊断准确率。方法回顾性分析12例经手术病理证实的CD超声声像图特点及病理表现。其中男4例,女8例,年龄19~61岁,平均40岁。结果超声均表现为椭圆形、边界清晰的不均质低回声肿块,其中局灶型10例,均为透明血管型,8例肿块内见细密点线样高回声,2例见散在斑点状粗大强回声;6例肿块内部为Ⅲ级血流信号,4例为Ⅱ级血流信号;7例肿块周边呈环形或半环形血流信号,3例血流信号稀少;6例肿块周围见粗大血管穿入。多中心型2例,1例为透明血管型,肿块内见向周边放射分布的条带状强回声,内部为Ⅱ级血流信号,周围呈半环形血流信号并有粗大血管穿入;1例为浆细胞型,肿块内可见细密点线样高回声,内部为Ⅲ级血流信号,周围血流信号稀少。结论超声显示边界清晰、内部回声不均的低回声肿块,内部血流信号丰富,周边有环形或半环形绕行血流信号,有粗大血管穿入时应考虑到CD可能,内部有强回声钙化者,高度提示透明血管型。     

Human herpesvirus 8‐positive multicentric Castleman disease with germinotropic plasmablastic aggregates: Overlapping spectrum of human herpesvirus 8‐associated lymphoproliferative disorder

The diagnosis of human herpesvirus 8 (HHV8)‐associated lymphoproliferative disorder (LPD) is challenging because of the rarity and extended spectrum of each entity. A 43‐year‐old, human immunodeficiency virus seropositive, Japanese man was referred to our department because of persistent fever, generalized lymphadenopathy, jaundice and anasarca. Biopsy of a left axially lymph node demonstrated relatively preserved nodal structure with multicentric Castleman disease (MCD) features. In the germinal center, there were aggregates of HHV8‐infected plasmablasts that were diffusely positive for CD38, MUM1/IRF4, LCA, IgM and λ; partially positive for CD30, c‐MYC, p53; and negative for CD138, CD20, PAX‐5, κ, CD2, CD3 and CD5. A small number of Epstein–Barr virus encoded small RNA (EBER)‐positive large cells infiltrated in the outer part of the germinal center and the mantle layer, but the cells copositive for EBER and HHV8 were not evident. We diagnosed the patient as HHV8‐positive MCD with germinotropic plasmablastic aggregates, which demonstrated intermediate pathologic features between HHV8‐positive MCD and germinotropic lymphoproliferative disorder. The pathogenesis of each HHV8‐associated LPD differs in cellular origin, host immune status, cytoplasmic immunoglobulin expression, clonality pattern and EBV infection; however, these factors sometimes overlap and induce extended clinical and pathologic presentations.     

合并肾损害的POEMS综合征患者的临床及预后分析

目的 分析合并肾损害的POEMS综合征患者的临床、肾脏病理特点及预后.方法 回顾性分析我院收治的6例POEMS综合征患者的临床表现、实验室检查、肾脏病理检查、治疗及随访等资料.结果 POEMS综合征男性多见,诊断时年龄28～65岁,平均（47.8±13.3）岁,诊断前病程平均4.1年.所有患者均有神经病变、脏器肿大、内分泌异常、皮肤改变,4例患者血M蛋白阳性,1例游离轻链比例异常.POEMS综合征肾损害多表现为轻中度蛋白尿,可合并肾功能不全,肾脏病理免疫荧光多阴性,或为免疫复合物的非特异沉积;光镜下肾小球和肾小动脉内皮细胞增生、肿胀,可合并缺血性肾损伤,透射电镜表现为内皮细胞增生、肿胀,基底膜内疏松层增厚,未见电子致密物.对于POEMS综合征的患者制定个体化治疗方案.随访平均25.8个月,1例合并Castleman病的患者死于感染合并多器官衰竭,其余5例病情稳定.结论 游离轻链比例异常可协助诊断POEMS综合征,本病的肾脏病理有特殊提示,肾活检为协助诊断本病的重要检查手段,单纯POEMS综合征的患者预后较好,合并Castleman病的患者预后相对较差.     

淋巴结内Castleman病临床病理分析

目的：探讨Castleman病的临床病理学特征及诊断、鉴别诊断。方法：通过组织学、免疫组化方法对5例Castleman病进行分析研究,并结合文献加以讨论。结果：3例为透明血管型,表现为增生的淋巴结内散在分布大型的淋巴滤泡,滤泡中心血管壁玻璃样变,其内可见核成空泡状的滤泡树突状细胞,外套层明显增厚,小淋巴细胞呈同心圆状包绕于血管周围,形成特征性的“洋葱皮”样同心圆结构。2例浆细胞型,特点是滤泡内的毛细血管玻璃样变性和“洋葱皮”样改变不明显,滤泡间可见粉染的无定型的嗜酸性物质沉积,并有大量成熟浆细胞弥漫增生。结论：Castleman病是一种特殊类型的淋巴结增生性疾病,诊断时要与反应性滤泡性增生、淋巴瘤等进行鉴别,并注意是否发生淋巴瘤等恶性转化。     

血管滤泡性淋巴结增生症的诊断和综合治疗

疾病的正确诊断和合理治疗是患者和医务工作者共同的心愿。在广东省人民医院肿瘤中心,吴一龙教授指导临床医生应用循证医学理论进行肿瘤的多学科综合治疗,开展临床病例讨论。讨论会上各学科共同围绕一个病例或一个病种进行会诊,临床、病理、B超、放射影像等资料齐全,除相关科室提前准备的中心性发言外,到会人员各抒己见,气氛热烈。参会人员受益匪浅,提高了对疑难病例的诊治水平。为了将他们的诊治经验传播出去,让更多的临床医生获益,我刊开辟“循证病例讨论”栏目,希望广大医务工作者关注此栏目。