Laparoscopic approach of Castleman's disease in the pararenal retroperitoneum: report of two cases

Castleman's disease (CD) is a benign lymphoid neoplasm characterized by massive proliferation of the lymphoid tissue due to an uncertain cause.1 Two clinical types (localized, multicentric) have been described with three histological variants (hyaline-vascular, plasma cell and mixed type). The expected localization is mediastinum and rarely pararenal retroperitoneum.     

Laparoscopic approach of Castleman’s disease in the pararenal retroperitoneum: report of two cases

Castleman＇s disease （CD） is a benign lymphoid neoplasm characterized by massive proliferation of the lymphoid tissue due to an uncertain cause. Two clinical types （localized, multicentric） have been described with three histological variants （hyaline-vascular, plasma cell and mixed type）. The expected localization is mediastinum and rarely pararenal retroperitoneum. We reported two cases of CD in the pararenal retroperitoneum, one was complicated with autoimmune hemolytic anemia.     

Magnetic resonance imaging of bone marrow for TAFRO syndrome

Abstract

We report two cases of TAFRO syndrome, which is characterized by thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly. Magnetic resonance imaging (MRI) of the spine showed a dark medullary pattern in the bone marrow on the T1- and T2-weighted images of both patients. One patient showed complete resolution after treatment. Serial MRIs of the improved patient revealed a transition to a normal marrow pattern on both images, which might represent resolution of the disease.     

Endotracheal castleman disease: A case report

Castleman disease (CD) is an uncommon benign lymphoid hyperplasia with several clinical and morphologic variants associated with distinct outcomes. Pulmonary CD has been reported as a rare extranodal manifestation in the literature. However, CD presenting as an obstructive mass in the airway has not been documented. We report a case of localized hyaline-vascular CD presenting as an endotracheal lesion. The patient was a 50-year-old woman with Marfan syndrome. The lesion caused near-complete airway obstruction with respiratory insufficiency. The patient underwent laser resection, and the diagnosis of CD was supported by comprehensive studies including histopathologic, immunohistochemical, and molecular methods.     

Castleman病肾损害的临床病理分析

目的 探讨Castleman病肾损害的临床病理特点。 方法 对10例Castleman病合并肾损害患者的临床病理资料进行分析。全部病例均接受了淋巴结和肾组织的病理检查,肾活检组织分别进行了光镜、免疫荧光和电镜检查。 结果 10例均为男性,平均年龄（49±14）岁。多数患者有水肿;全部患者均有蛋白尿,尿蛋白量（24 h）为（2.79±3.56）g,其中1例为肾病综合征（NS）;8例有血尿;6例并发急性肾功能不全;4例有高血压。多数有发热、乏力、纳差、体质量下降等。其他异常有贫血、血小板减少、浆膜腔积液、红细胞沉降率增块、高γ球蛋白血症、补体降低、C反应蛋白（CRP）升高、肝脾肿大、甲状腺功能低下等。2例符合POEMS综合征,1例有干燥综合征。10例均表现全身多处淋巴结肿大,以颈部、腋窝及腹股沟淋巴结肿大最常见。淋巴结活检病理类型分别为浆细胞型4例,透明血管型3例,混合型3例。肾活检病理诊断分别为血栓性微血管病5例、新月体性肾小球肾炎2例、肾淀粉样变1例、肾小球微小病变1例、慢性肾小管间质肾病1例。所有病例经过免疫抑制剂或COP方案治疗,病情均明显缓解,淋巴结缩小,蛋白尿减少或转阴,大部分病例肾功能恢复正常。 结论 Castleman病肾损害的临床和病理表现具有多样性,急性肾功能不全发生率较高,常并发全身多系统损害,肾脏病理以血栓性微血管病较多见。对于伴有全身多系统异常的肾脏病患者,有必要进行淋巴结影像学检查和淋巴结活检。     

Plasma HHV‐8 viral load in HHV‐8‐related lymphoproliferative disorders associated with HIV infection

This is a mono‐institutional analysis of the clinical features, immunological and virological findings, and prognostic factors of patients with HIV infection and HHV‐8‐lymphoproliferative disorders. Patients with Multicentric Castleman Disease and HHV‐8‐related lymphoma diagnosed and treated from April 1987 to June 2004 were included in the study. HHV‐8 and HIV plasma viral load, CD4+ count, hematologic parameters, and general wellbeing (performance status) were assessed at the onset of the diseases and analyzed in order to identify possible prognostic factors. Nine patients with Multicentric Castleman disease, and 16 with HHV‐8‐related lymphomas (13 primary effusion lymphomas and 3 solid lymphomas), were diagnosed and treated out of 327 HIV‐related non‐Hodgkin's lymphomas. Four patients with Multicentric Castleman disease received only antiretroviral drugs; 5 HAART plus oral etoposide. Nine patients with primary effusion lymphoma were treated with a CHOP‐like regimen (Cyclophosphamide, Prednisone anthracyclines, Vinca alkaloids, Bleomycin, Etoposide) and HAART; 1 with etoposide and HAART, 1 with HAART alone. The patients with solid lymphoma underwent CHOP‐like chemotherapy. Patients with Multicentric Castleman disease showed lower median values of HHV‐8 viral load and longer overall survival compared with HHV‐8‐related lymphomas. Patients with viral load of HHV‐8, >40,000 cp/ml had a significant shorter overall survival. In the univariate analysis, HHV‐8‐related lymphoma, HHV‐8 viral load >40,000 cp/ml and performance status >2 were associated with an increased risk of death. Multivariate analysis confirmed the diagnosis of lymphoma as an independent predictor of shorter survival. J. Med. Virol. 81:888–896, 2009. © 2009 Wiley‐Liss, Inc.     

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。     

Reversible Cardiomyopathy Associated with Multicentric Castleman Disease: Successful Treatment with Tocilizumab, an Anti—Interleukin 6 Receptor Antibody

Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder characterized by systemic lymphadenopathy and inflammatory symptoms that are associated with the overproduction of interleukin 6 (IL-6). Although several nonlymphoid organs can also be damaged in MCD, only a few cases with cardiac complications have been reported to date. We report a case of congestive heart failure in a female patient with MCD. On admission, her echocardiogram revealed a dilated and diffusely hypokinetic left ventricle. No stenosis was evident in the coronary angiogram. A histopathologic examination of a myocardial biopsy specimen showed mildly hypertrophic myocytes without infiltration of plasma cells or amyloid deposits. Repeated administration of an anti-IL-6 receptor antibody, tocilizumab (formerly known as MRA), gradually improved the ventricular wall motion over 6 months without any additional treatment for heart failure, suggesting the involvement of IL-6 in the pathogenesis of her cardiomyopathy. This report is the first of MCD complicated by heart failure treated successfully with tocilizumab. Administering tocilizumab in cases of MCD with unexplained cardiac dysfunction is worthwhile, because such a complication could be reversible.     

儿童颈、胸部受累的局灶性Castleman病1例报告并文献复习

目的 提高对少见病颈、胸部受累的Castleman病的认识,减少误诊,延长生存时间.方法 报告1例少年颈部、胸部受累的Castleman病,讨论其临床表现、影像学、病理学、鉴别诊断、治疗及预后.结果 颈、胸部受累的多中心Castleman病多为浆细胞型,浆细胞型以淋巴滤泡生发中心间出现成层排列的浆细胞为特征,缺乏或少量存在管壁透明变的毛细血管,约占全部病例的9%.肺门纵隔区多组淋巴结肿大、淋巴细胞性间质性肺炎、胸腔积液,病理学可见淋巴滤泡增生,伴大量浆细胞浸润,外周淋巴结多处肿大,多系统损害.激素或化疗部分缓解.颈、胸部受累的局限性Castlema病多位于纵隔,多为透明血管型,无症状、体征,以淋巴滤泡增生伴生发中心形成和大最管壁透明变的毛细血管存在为特征,约占全部病例的91%.影像学表现为肺门或纵隔部位的块状阴影,病变密度均匀,边缘光滑或有分叶,可单发也可多发,以右上纵隔较多见,肺野多无异常表现,早期手术切除预后好.结论 颈、胸部受累的Castleman病的临床表现多样,易误诊,有单发巨大或多发淋巴结肿大者应警惕此病,诊断关键在于早期多次、多部位外周淋巴结活检和肺组织病理检查.与血管同步显著强化的影像学表现有诊断价值.