• Radiation Oncology Journal
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• v.31 no.1
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• pp.48-54
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• 2013

Castleman's disease or angiofollicular lymph node hyperplasia is a rare lymphoproliferative disorder. Complete surgical resection was recommended in unicentric Castleman's disease. Radiotherapy was considered alternative therapeutic option. However, there have been consistent favorable responses to radiotherapy. We also experienced two cases of uncentric Castleman's disease salvaged successfully with radiotherapy. This paper described these cases and reviewed the literature about Castleman's disease treated with radiotherapy. Reviewed cases showed that radiotherapy is a successful treatment option in unicentric Castleman's disease. Furthermore, our report confirms the radiotherapy role in uncentric Castleman's disease.

• 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.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 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.

• Journal of Yeungnam Medical Science
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• v.33 no.2
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• pp.112-115
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• 2016

Lymphocytic interstitial pneumonia (LIP) is a rare benign lymphoproliferative disorder characterized by diffuse infiltration of the pulmonary parenchymal interstitium by polyclonal lymphocytes and plasma cells. LIP has been associated with a variety of clinical conditions; such as connective tissue disorders and other immune system abnormalities. Treatment usually involves administration of corticosteroids and other immunosuppressants. We report on a 38-year-old female patient who complained of shortness of breath, dry mouth, and dry eyes for more than 1 month, and was positive for Raynaud's phenomenon. Based on surgical biopsy, she was diagnosed as having LIP accompanied by $Sj{\ddot{o}}gren^{\prime}s$ syndrome. The patient was treated with high-dose steroids followed by maintenance therapy for approximately 2 years, and her condition improved.

• 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.

• Clinics in Shoulder and Elbow
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• v.12 no.1
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• pp.89-93
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• 2009

Purpose: Kimura's disease is an uncommon benign lymphoproliferative inflammatory disorder with an unknown etiology. The recurrence rate after surgical excision is relatively high and renal involvement is its only systemic manifestation. The condition mainly involves the head and neck, and peripheral involvement is extremely rare. Materials and Methods: We encountered the case of a 28-year-old man who had a non-tender mass with mild brownish skin color changes and pruritus around the medial side of the distal arm and elbow. Results: The peripheral blood investigation revealed peripheral eosinophilia and elevated serum IgE levels. Magnetic resonance imaging showed an isointensity signal relative to the muscle on the T1-weighted images and hyperintensity signal relative to the muscle on the T2-weighted images. Conclusion: A marginal resection of the lesion was performed and there was no recurrence at 2 years postoperatively.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.129-138
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• 2023

Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haploinsufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection.

• Journal of Korean Neurosurgical Society
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• v.29 no.10
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• pp.1365-1371
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• 2000

Objectives : Spinal cord compression is a rare presentation of non-Hodgkin's lymphoma(NHL), occurring in 0.1% to 10.2% of patients. Primary spinal extradural NHL, i.e. occurring in the absence of any detectable extraspinal lymphoproliferative disorder, has a potentially favourable outcome if diagnosed and treated early. The authors describe two patients with a clinical picture of acute spinal cord compression as the first presentation of NHL. Methods : The patients were 48-year-old female and 27-year-old female. Both presented with back pain followed by acute paraparesis and voiding difficulty. One patient was diabetic. Plain radiographs of the spine were not specific. Thoracic spine magnetic resonance imaging(MRI) revealed evidence of extradural soft tissue mass extending multiple vertebral segments. Results : The patients underwent emergency laminectomy for decompression and tissue diagnosis. Histological and immunohistochemical study revealed B-cell lymphoma, intermediate grade in both patients. Postoperative staging did not reveal any additional lesions other than extradural manifestation of the malignant lymphoma. Surgery with additional chemotherapy and radiotherapy allowed the clinical improvement of both patients. Conclusion : The authors report two patients with a clinical picture of acute spinal cord compression as the first presentation of NHL, and stress that primary spinal epidural NHL should be a diagnostic consideration in the patient without prior history of malignancy who presents with a prodrome of back pain followed by a rapid neurological deterioration.

• Journal of Korean Neurosurgical Society
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• v.30 no.3
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• pp.395-399
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• 2001

Primary spinal epidural lymphoma(SEL), i.e. occurring in the absence of any detectable extraspinal lymphoproliferative disorder, is an unusual cause of spinal cord compression. The authors report a 48-year-old, diabetic woman presented with back pain followed by acute paraparesis and voiding difficulty. She had been treated with acupunctures on her back before admission, and complete blood count showed leukocytosis with neutrophilia and increased erythrocyte sedimentation rate(ESR). Thoracic spine magnetic resonance imaging(MRI) revealed an epidural mass extending from T5 to T8 with compression of the spinal cord. Emergency decompressive laminectomy was performed with a tentative diagnosis of spinal epidural abscess, but a B-cell lymphoma was final pathologic diagnosis. Further staging showed no other sites of lymphoma, and the spinal lesion was treated by chemotherapy and radiotherapy. The authors stress that primary SEL can mimic spinal epidural abscess(SEA) in the diabetic patient and should be a diagnostic consideration in patients with a syndrome of acute spinal cord compression manifested by a prodrome of back pain and neuroimaging consistent with an epidural compressive lesion, especially in a diabetic.