颈部淋巴结滤泡树突状细胞肉瘤合并巨大淋巴结增生一例

患者女,36岁。因发现左颈部包块10余年,近2年增大,于2003年6月26日首次入院治疗。之后,又因局部复发于2004年5月、2005年3月和2006年2月入院行手术治疗。并在第3次手术后,进行过不规范的化疗3次(患者不遵医嘱)。4次入院体检时,均在左侧颈部锁骨上触及一约6 cm×     

颈部Castleman病的诊断和治疗

目的：总结颈部Castleman病（Castleman’s disease,CD）的临床与病理特征,探讨其诊断与治疗方法。方法：回顾性分析长沙市第三医院2000年至2012年确诊的5例颈部CD患者的临床资料,包括临床特点、病理诊断、治疗和预后。结果：5例均经病理诊断为单中心型透明血管型CD。均行手术切除,随访12个月以上,仅1例在术后14个月复发,再次行手术切除,随访62个月无复发。结论：颈部CD多为单中心型透明血管型,病理切片检查是其主要诊断方法,手术切除是有效的治疗方法。     

Castleman病临床病理分析并文献复习

目的探讨Castleman病(Castleman’s disease,CD)的临床病理学特征、诊断及鉴别诊断。方法对10例CD进行临床资料分析、病理形态学观察及免疫组化检测,并复习相关文献。结果 10例CD中,8例为单中心型,2例为多中心型;6例为透明血管型,1例为浆细胞型,3例为混合型。免疫表型:所有病例中BCL-6和CD10均阴性,Ki-67增殖指数均≤30%。10例患者中4例有完整的随访资料,其中1例为混合型,3例为透明血管型,手术切除后均痊愈,未复发。结论 CD是一种少见的、病因不明的淋巴组织增生性疾病,术前临床诊断有一定困难。免疫表型特征是否为CD患者免疫功能异常的反映,及其在CD发病机制上起何种作用,仍需进一步深入分析。     

超声诊断右肾上腺区巨淋巴结增殖1例

患者男,49岁,中上腹胀痛伴右侧腰背隐痛1个月。查体:右肾区无叩痛及压痛。实验室检查:血常规及肝肾功能(-),血沉正常。患者无高血压,无血尿。超声检查:肝肾间见96mm×73mm低回声不均质团块,内见5mm强光斑多枚弥漫分布(图1)。肿块与肝脏、右肾间有厚而明亮的包膜,分界清。超声印象:肝肾间实质占位,考虑肾上腺或腹膜后来源,良性病变可能。CT:右肾上腺区见9cm×7cm占位,与右肾紧贴,肿块内见多发钙化斑,增强后肿块动脉期强化明显,持续时间长,门脉期仍为高密度。CT印象:右肾上腺来源肿瘤,性质待定。术中:肝肾间肿块约8cm×7cm×7cm大小,肿块包…     

Aseptic Meningitis-retention Syndrome Associated with Tocilizumab in a Patient with Idiopathic Multicentric Castleman Disease

This is the first report of tocilizumab-associated meningitis-retention syndrome in a patient with idiopathic multicentric Castleman disease. A 57-year-old man presented with headache, nuchal rigidity, impaired consciousness, pyramidal tract signs and urinary retention. A cerebrospinal fluid examination revealed increased cell counts and protein levels. These symptoms were improved by intravenous methylprednisolone. Tocilizumab-associated meningoencephalitis has been reported in patients with rheumatoid arthritis and juvenile idiopathic arthritis but not with multicentric Castleman disease. This case presents evidence of the increased probability of meningitis as a neurological complication of tocilizumab administration.     

Mediterranean fever gene variants modify clinical phenotypes of idiopathic multi‐centric Castleman disease

Four cases of idiopathic multi‐centric Castleman disease (iMCD) reportedly have variants in hereditary autoinflammatory disease‐related genes; however, the frequency and role of these variants in iMCD is still unknown. We therefore investigated such gene variants among patients with iMCD and aimed to reveal the relationship between iMCD and autoinflammatory disease‐related genes. We reviewed 14 Japanese iMCD patients who were recruited between January 2015 and September 2019. All patients met both the Japanese tentative diagnostic criteria for Castleman disease and the international consensus diagnostic criteria for iMCD. We performed genetic analyses for 31 autoinflammatory disease‐related genes by targeted next‐generation sequencing. The MEFV gene variants were observed in 10 of 14 patients with iMCD. Although iMCD had a high percentage of exons 2 or 3 variants of MEFV, comparison of data from healthy Japanese subjects indicated that there was no significant difference in the percentage between healthy Japanese subjects and patients with iMCD. Variants of uncertain significance (VUS) in the TNFRSF1A and CECR1 genes were observed in two of the patients, respectively. We divided patients into two groups—those with MEFV variants (excluding E148Q variants) and those without MEFV variants—and compared the clinical characteristics between these two groups. Patients with MEFV variants, excluding E148Q variants, exhibited a significantly higher likelihood of fever and significantly lower levels of hemoglobin than those lacking MEFV variants. Our results indicated that patients with iMCD tended to have a high frequency of MEFV gene variants and the presence of such variants can affect iMCD clinical phenotypes.     

Focal Castleman disease of the lung: MRI findings

Castleman disease is an uncommon benign disorder mainly affecting mediastinal lymph nodes and rarely visceral organs. The most typical structural finding is hypervascularity which can be well demonstrated both by CT and MRI. We report MR findings of an unusual case of solitary parenchymal lung involvement.     

Castleman disease of the parotid gland in childhood: an unusual entity

Castleman disease (CD) is an uncommon benign condition of uncertain etiology that causes progressive lymph node enlargement. Although the mediastinum is their common location, they also occur in other areas of the body, usually where lymph nodes are normally found. In addition, CD (the hyaline-vascular type) of the parotid gland is very rare, especially in childhood. We report a new case of CD arising from the parotid gland in 9-year-old male child. The clinical course, histopathological findings, radiological findings, and management of CD are reviewed.