• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.203-206
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• 2003

Castleman's disease is an atypical lymphoproliferative disorder of unknown origin. It has three histologic variants(hyaline vascular, plasma-cell, and mixed) and two clinical types(localized and multicentric). Some sufferers have constitutional symptoms and laboratory abnormalities such as anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. The localized form is cured by complete surgical excision whereas the multicentric form is managed by prednisone and other immunosuppressor drugs. The prognosis of the multicentric form is worse than the localized form since malignancies and severe infections may lead to a rapidly fatal outcome. Castleman's disease has been rarely reported at pediatric age in Korea. We experienced two cases of Castleman's disease detected at 3 and 5 years of age. They were presented with painless enlargement of submandibulars and axillary lymph nodes but had no associated symptoms. The lesions were excised and diagnosed as Castleman's disease, and no recurrence was noted during follow-up periods.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.443-447
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• 2005

Castleman's disease is a rare non-neoplastic lymphoproliferative disorder of unknown etiology. It is divided into three histologic subtypes; hyaline-vascular(HV), plasma cell(PC) type and mixed type (HV-PC). It has two clinical expressions. The localized form, which presents as a slow growing mass, has a relatively benign clinical course. The multicentric form is multilocated and holds significant morbidity. The mainstay of treatment of the localized form is surgical resection. The multicentric form requires medical treatment comprising prednisolone and other immunosuppressor drugs. The disease in children seems to have a more favorable course than in adults. We report a 13-year-old boy with Castleman's disease of multicentric form who was successfully treated with prednisolone and intravenous immunoglobulin.

• Journal of Yeungnam Medical Science
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• v.39 no.2
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• pp.153-160
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• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Tuberculosis and Respiratory Diseases
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• v.74 no.1
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• pp.23-27
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• 2013

Multicentric Castleman's disease (CD) is a rare atypical lymphoproliferative disorder, which is characterized by various systemic manifestations. Some patients with multicentric CD may have concomitant lung parenchymal lesions, for which lymphoid interstitial pneumonia (LIP) is known to be the most common pathologic finding. Follicular bronchiolitis and LIP are considered to be on the same spectrum of the disease. We describe a case of multicentric CD with pulmonary involvement, which was pathologically proven as follicular bronchiolitis.

• Korean Journal of Head & Neck Oncology
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• v.21 no.1
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• pp.48-52
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• 2005

Objectives: Castleman's disease(CD) is a lymphoproliferative disorder of unknown etiology. To elucidate the clinicopathologic characteristics of CD, we retrospectively reviewed our experience. Methods: Fifteen patients with CD of the neck and mediastinum were identified. Patients were divided into two groups: group I had an unicentric CD and group II had multicentric CD. The histology of CD was divided into 3 subtypes: hyaline-vascular(HV), plasma cell(PC), and mixed. Results: The study included 12 cases of group I, 3 cases of group II in the clinical aspect and 10 cases with HV, 3 cases with PC, 2 cases with mixed type in the histologic aspect. Of group I patients who underwent complete surgical excision, all are currently free of disease. The clinical course of group II patients was variable. Of two patients with multicentric plasma cell CD who were treated, one remain free from disease and the other had a local recurrence in the neck. One patient with multicentric mixed CD died of disease after 30 months of presentation. Conclusion: Surgical resection is recommend for patients with the unicentric CD, regardless of histologic subtype. Patients with multicentric disease do not benefit from surgical resection and should be candidates for multimodality therapy.

• Journal of Chest Surgery
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• v.20 no.3
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• pp.582-587
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• 1987

Castleman`s disease, giant lymph node hyperplasia, is a rare benign disease. The lesion usually consists of a single node, unassociated with any abnormality of the adjacent lymph nodes or other organs. In the first accounts of giant lymph node hyperplasia of Castleman, the lesion was described as solitary and localized to the mediastinum, which is still the most frequent site of involvement. The disease occurs in all age groups and there is no particular sex preference. It is symptomless and is usually detected on chest films as an incidental finding. On a single involvement, it does not recur after excision, whether total or partial, and the main indication of operation is to rule out more serious tumors. Recently multicentric form appears to be a variant of classic giant lymph node hyperplasia and is associated with significant morbidity and mortality. Histologically, two distinct types have been reported; hyaline-vascular and plasma cell. The hyaline-vascular type of lesion is much more common than the plasma cell type. We report two cases of the hyaline-vascular type of Castleman`s disease.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.74-76
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• 2017

Muscle specific tyrosine kinase (MuSK) myasthenia gravis (MG) is a rare subtype of MG, which is immunologically distinct and differential therapeutic response. Though MG is often associated with other autoimmune disorders or malignancy, concurrence of other disease and MuSK MG has been infrequently reported. We present a patient of MuSK MG associated with multicentric Castelman disease.

• Tuberculosis and Respiratory Diseases
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• v.44 no.3
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• pp.669-676
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• 1997

Castleman's disease is uncommon lymphoproliferative disorder as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia. Multicentric variant of Castleman's disease, plasma cell type has been described that has more generalized lymph node involvement as well as involvement of other organ systems than localized type. Multicentric plasma cell type is frequently accompanied by systemic manifestations, such as weight loss, lowgrade fever and weakness. But the reported cases of pulmonary parenchymal involvement are rare and have almost consisted of hyalinized granuloma adjacent to a bronchus. We report a patient with Castleman's disease of the lung, pathologically proven interstitial pulmonary involvement.

• Korean Journal of Head & Neck Oncology
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• v.32 no.2
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• pp.35-39
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• 2016

Castleman's disease (CD) is an uncommon lymphoproliferative disorder. The disease entity is classified into 2 clinical subtypes, unicentric and multicentric type. Prevalence of lymphoid malignancy in multicentric CD (MCD) is very low. In this case, we report a case of 77 years old woman who developed high fever and swelling in both side of her neck. Neck lymph node biopsy revealed plasma cell hyperplasia. Patient's symptom was subsided after treatment with Dexamethasone. Three months later, multiple lymph node enlargement was developed in abdomen and neck area again. Repeated neck lymph node biopsy confirmed diffuse large B cell lymphoma. The patient started chemotherapy.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.27 no.5
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• pp.468-473
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• 2001

Castleman's disease or angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder. It may be appear as a local or generalized tumor-like condition, usually in chest or abdomen and may involve both lymph nodes and non-nodal tissues. Castleman's disease is an unusal entity which may at times mimic malignancy but is entirely benign in nature. It is topical as it has been noted to occur with AIDS and Kaposi's sarcoma. Careful interpretation of radiogram may help to distinguish Castleman's disease from other tumor condition, such as lymphoma, neurogenic tumor, or even angiofibroma, etc. But exact diagnosis must be made on the basis of histologic confirmation. In addition to histologic features, clinical distinction between the localized and multicentric form is important in selecting appropriate management. Surgical excision of an localized mass is the first choice of treatment. Partial resection, radiotherapy or observation alone may avoid the need for exessively aggressive therapy. Patients with multicentric disease don't benefit by surgical management because of systemic manifestation and rapid deterioration. Thus, antineoplastic agents and steroids may offer an alternative form of therapy. We report a case of female patient with Castleman's disease in oral and maxillofacial region, treated by surgical excision with good results.