多中心型Castleman病1例报道并文献复习

目的了解Castleman病(CD)的临床特点、诊断及治疗方法。方法对2015年2月我科收治的1例Castleman病进行回顾分析,观察患儿进行6周期CHOP方案(吡柔比星+环磷酰胺+长春地辛+甲泼尼龙)治疗后症状、体征、实验室指标变化。结果通过6周期化疗患儿体温稳定,乏力缓解,关节疼痛消失,淋巴结缩小,CRP、血沉及IgG、IgE水平下降。结论 CD是一种临床罕见的介于良恶性之间的淋巴组织增殖性疾病,化疗可为治疗多中心型Castleman病的有效手段。     

Paraneoplastic syndrome and intrathoracic Castleman disease

We report two cases of intrathoracic Castleman disease presenting with paraneoplastic syndrome. Patient 1 was a 10-year-old girl with short stature. She was found to have delayed bone age, slow growth velocity, and iron-deficiency anemia, which was refractory to treatment. Thrombocytosis and hypergammaglobulinemia were later detected. Chest X-ray revealed a hilar mass. Patient 2 was a 14-year-old boy who had severe cough, progressive mucocutaneous erosion, and dermatitis. Chest X-ray showed a mediastinal mass. Sections of skin biopsy showed findings consistent with pemphigus disease. In each case, the histological diagnosis of Castleman disease was made.     

Iron therapy resistant microcytic anaemia in a 13-year-old girl with Castleman disease

We describe the case history of a 13-year-old girl with chronic fatigue and prolonged microcytic anaemia. She received oral iron since the age of 11 but failed to respond to it. Laboratory studies revealed elevated C-reactive protein and hypergammaglobulinaemia. A large solitary mesenterial lymph node could be demonstrated by ultrasonography and CT. A diagnosis of Castleman disease was suspected and confirmed histologically. After surgical removal of the lymphoma the patient recovered completely. Conclusion Castleman disease should be considered in cases of chronic fatigue, unexplained fever, microcytic anaemia and hypergammaglobulinaemia. Received: 10 February 1996 Accepted: 23 March 1996     

Paediatric Castleman disease: report of seven cases and review of the literature

Castleman disease is a distinct lymphoproliferative disorder of unknown origin. Seven new cases in children are reported here and 76 cases from the paediatric literature are reviewed. The disease has been reported in 46 females and 37 males, their age ranging from 2 months to 17 years. The disease was localized in 72 cases and multicentric in 11 cases. The hyalinovascular type was more frequently encountered (54%) than the plasma cell type (24%). Laboratory abnormalities were more often associated with the plasma cell type and were mainly represented by anaemia and hypergammaglobulinaemia. Treatment of the localized tumour consisted of surgical excision, whereas treatment of the multicentric form was medical, comprising prednisone and other immunosuppressor drugs. The disease in the paediatric population seems to have a more favourable course than in adults. Conclusion The paediatric features of the disease suggest that Castleman disease in this population could represent an earlier form of the pathology or even suggest a benign lymphoproliferative disorder. Received: 14 November 1997 / Accepted in revised form: 9 September 1998     

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.     

Anemia and autoimmunity markers in an adolescent with Castleman disease

We describe herein the case of an adolescent girl with anemia non‐responsive to oral iron, associated with low‐grade fever, diminished appetite and fatigue. A palpable mass below the xiphoid was noted. Laboratory findings were consistent with anemia of inflammation. Direct antiglobulin test was positive without any other evidence of autoimmune anemia. Other autoantibodies, such as anti‐thyroid and anti‐nuclear antibodies, were also positive. After thorough investigation, Castleman disease was the most likely diagnosis on the basis of high serum interleukin (IL)‐6 and the magnetic resonance imaging findings. ¹⁸ F‐FDG positron emission tomography–computed tomography showed a localized hypermetabolic mass, which was resected. Castleman disease of plasma type was identified on histology. Hemogloblin and IL‐6 gradually returned to normal, whereas positive autoantibodies became negative. This case emphasizes the need to investigate thoroughly for the underlying cause of anemia of inflammation and to include Castleman disease in the differential diagnosis, on the measurement of IL‐6.     

Castleman disease in a child with short stature

We report a 14‐year‐old boy with Castleman disease in this article. He complained of short stature, and his body height was 133.8 cm (<3rd percentile; z score ?4.5). There was marked delay in the appearance of secondary sexual characteristics. He was found to have a remittent fever and a lower mid‐abdominal tumor. Blood test revealed microcytic hypochromic anemia, thrombocytosis, polyclonal hypergammaglobulinemia, hyperfibrinogenemia, and elevated erythrocyte sedimentation rate. The serum IL‐6 and C‐reactive protein levels were increased. The mass was found to be mixed hyaline vascular and plasma cell type of Castleman disease through a pathological examination. Lymph nodes affected by Castleman disease cause overproduction of IL‐6. It decreases IGF‐1, IGFBP‐3 and serum testosterone levels. As a result of tumorectomy, his short stature and delay in the development of secondary sexual characteristics were improved.     

Renal failure in pediatric Castleman disease: Four French cases with thrombotic microangiopathy

Pediatric Castleman disease (CD) is an uncommon and poorly understood disorder of the lymph nodes. Renal failure has not been described in pediatric multicentric CD (MCD). We report four cases, who presented with polyadenopathy, organomegaly, edema and fluid accumulations, high blood pressure, and acute renal failure. In all cases, renal biopsy confirmed diffuse thrombotic microangiopathy. Definitive diagnosis of MCD was made by a biopsy of an affected lymph node located by computer tomography before initiation of corticosteroid therapy. Treatment of CD with corticosteroid therapy and rituximab was rapidly effective without relapse to date.     

Unifocal cervical Castleman disease in two children

Castleman disease (CD) is a benign lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes. Most observations focus on adult patients. In children the disease is rare, usually localized and may be symptomatic or asymptomatic. We reported two children, 7 and 14 years old respectively, affected by unifocal cervical CD of the hyaline vascular type. Conclusion Two cases illustrate that CD may mimic a neoplasm and should be considered in presence of a solitary neck mass with specific features on CT and MRI. Received: 19 November 1996 / Accepted in revised form: 7 April 1997     

Castleman disease in a pediatric liver transplant recipient: a case report and literature review

Castleman disease is a rare hematologic disorder, closely linked to the HHV-8, and most commonly observed in immunocompromised individuals. Thirteen months following a liver transplant for CPS-1 defect, a 15-month-old boy presented with fevers, anemia, and growth retardation. Abdominal CT scan showed splenomegaly and generalized lymphadenopathy. Histology of chest wall lymph nodes revealed a mixed CD3+ T-cell and CD20+ B-cell population with atretic germinal centers consistent with multicentric Castleman disease. Qualitative DNA PCR detected HHV-8 in the resected lymph node and in the blood, supporting the diagnosis. Immunosuppression was tapered, and he was transitioned from tacrolimus to sirolimus. His graft function remained stable, and repeat imaging showed regression of the lymphadenopathy. The child is living one yr after Castleman disease diagnosis with a well-functioning graft. Castleman disease is a potential complication of solid organ transplant and HHV-8 infection. Reduction in immunosuppression and switch to sirolimus may be an effective strategy to treat this condition.     

儿童Castleman病21例临床回顾性分析

目的 分析Castleman病(CD)的临床特点,以期提高临床医师对CD的认识.方法 回顾性分析2010年1月—2020年6月确诊的CD患儿的临床资料,总结归纳患儿的临床特征及诊疗方案、预后.结果 21例CD患儿中,男11例、女10例,中位就诊年龄10.9(5.4～12.1)岁,中位病程为6.0(1.5～16.0)月....     

Multicentric castleman disease in a child with primary immunodeficiency

Multicentric Castleman disease is a rare lymphoproliferative disorder mostly seen in adults with HIV. It presents with fever and systemic symptoms and is extremely uncommon in children. We describe a novel case of multicentric Castleman disease associated with primary immunodeficiency (common variable immunodeficiency) and discuss pathophysiologic mechanisms and recent advances in understanding this disease. Pediatr Blood Cancer. 2010;55:1198–1200. © 2010 Wiley‐Liss, Inc.     

Hydatid disease in children may have an atypical presentation

 Although there is voluminous literature describing various aspects of hydatid disease in children, little attention has been paid to the small group of patients whose symptoms result in atypical presentation. This article addresses this problem, describing the features in ten children aged from 2 to 12 years. The sites of involvement were within a choledochal cyst (1). the pelvic cavity (1), the spleen (1), and transverse mesocolon. Albendazole was efficacious in the treatment of one recurrent case, as well as in preventing recurrence. Accepted: 19 April 1999     

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date.