• Journal of Yeungnam Medical Science
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• v.37 no.2
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• pp.136-140
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• 2020

Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.1
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• pp.383-386
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• 2012

Background: Cancer survivors are at increased risk of second cancers. Lymphoproliferative disorders (LPD) are common neoplasms that are primary or subsequent cancers in cases of multiple primary cancer. We here analyzed metachronous or synchronous LPD in multiple primary cancers. Methods: Between 2001 and 2010, LPD were assessed retrospectively in 242 multiple primary cancers patients. Results: Forty nine (20.2%) patients with LPD were detected. Six patients had two LPD where one patient had three LPD. The median age of patients was 60.5 years (range: 28-81). LPD were diagnosed in 29 patients as primary cancer, in 23 patients as second cancer, and in three patients as third cancer in multiple primary cancers. Primary tumor median age was 56 (range: 20-79). Diffuse large B cell lymphoma (n=16), breast cancer (n=9), and lung cancer (n=6) were detected as subsequent cancers. Alklylating agents were used in 19 patients (43.2%) and 20 patients (45.5%) had received radiotherapy for primary cancer treatment. The median follow-up was 70 months (range: 7-284). Second malignancies were detected after a median of 51 months (range: 7-278), and third malignancies with a median of 18 months (range: 6-72). Conclusions: In this study, although breast and lung cancer were the most frequent detected solid cancers in LPD survivors, diffuse large B cell lymphoma was the most frequent detected LPD in multiple primary cancers.

• Pediatric Infection and Vaccine
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• v.24 no.1
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• pp.44-53
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• 2017

Purpose: The aim of this study was to investigate the risk factors for posttransplant lymphoproliferative disorder (PTLD) and to evaluate the association between Epstein-Barr viral load and the development of PTLD in pediatric heart transplant recipients. Methods: We reviewed children aged <18 years who underwent heart transplantation and quantitative analysis of blood Epstein-Barr virus (EBV) viremia at our institute from January 2006 to March 2015. Clinical characteristics and EBV viral loads were compared according to the presence of PTLD. Results: Over 9 consecutive years, a total of 40 heart transplant recipients, were included. Among 28 children with available EBV viral load measurements, seven patients (25%) had EBV viremia only defined as at least one time of ${\geq}457copies/mL$. PTLD occurred in three recipients (7.5%) 4.3, 6.3, and 17.0 months after transplant and all PTLD cases had preceding EBV viremia. The median age at transplant was 5.3 years (range, 0.5 to 6.0 years) in the PTLD group, compared with 11.9 years (range, 0.3 to 17.8 years) in the non-PTLD group (P=0.021). The median values of the peak EBV levels in the PTLD group were 3,452,170 copies/mL (range, 46,750 to 7,622,910 copies/mL); the peak EBV levels in the non-PTLD group were 3,112 copies/mL (range, 2,250 to 103,000 copies/mL). Conclusions: Younger age at transplant and presence of EBV viremia were associated with the development of PTLD in pediatric heart transplant recipients. A prospective study will be required to determine the blood EBV load for predicting the development of PTLD in these patients.

• 대한세포병리학회:학술대회논문집
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• 1992.11a
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• pp.15.2-16
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• 1992

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.11
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• pp.6347-6350
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• 2013

Post-transplant lymphoproliferative disorder (PTLD) is a common complication of therapeutic immunosuppression after organ transplantation. Gene expression profile facilitates the identification of biological difference between Epstein-Barr virus (EBV) positive and negative PTLDs. Previous studies mainly implemented variance/regression analysis without considering unaccounted array specific factors. The aim of this study is to investigate the gene expression difference between EBV positive and negative PTLDs through partial least squares (PLS) based analysis. With a microarray data set from the Gene Expression Omnibus database, we performed PLS based analysis. We acquired 1188 differentially expressed genes. Pathway and Gene Ontology enrichment analysis identified significantly over-representation of dysregulated genes in immune response and cancer related biological processes. Network analysis identified three hub genes with degrees higher than 15, including CREBBP, ATXN1, and PML. Proteins encoded by CREBBP and PML have been reported to be interact with EBV before. Our findings shed light on expression distinction of EBV positive and negative PTLDs with the hope to offer theoretical support for future therapeutic study.

• Clinical and Experimental Pediatrics
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• v.58 no.3
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• pp.108-111
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• 2015

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.

• Tuberculosis and Respiratory Diseases
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• v.73 no.2
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• pp.115-121
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• 2012

Pulmonary mucosa-associated lymphoid tissue-derived (MALT) lymphoma is a rare disease. This disorder is considered to be a model of antigen-driven lymphoma, which is driven either by autoantigens or by chronic inflammatory conditions. Low-grade B-cell MALT lymphoma may develop from a nonneoplastic pulmonary lymphoproliferative disorder, such as lymphocytic interstitial pneumonitis (LIP). A recent estimate predicts that less than 5% of LIP patients acquire malignant, low-grade, B-cell lymphoma. In Korea, there has been no previous report of malignant low-grade, B-cell lymphoma, acquired from LIP. Here, we present the case of a patient with LIP that developed into pulmonary MALT lymphoma, six years after diagnosis.

• The Korean Journal of Cytopathology
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• v.19 no.2
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• pp.168-172
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• 2008

T-cell prolymphocytic leukemia (T-PLL) is a rare, mature T-cell lymphoproliferative disorder with a post-thymic mature T-cell phenotype. The disease is characterized by rapidly rising lymphocytosis, lym-phadenopathy, and splenomegaly. The clinical course is usually aggressive and progresses with frequent skin lesions and serous effusions. In 25% of cases, leukemic cells are small and tumor cells may not have a discrete nucleolus under light microscopy. Although the presence of characteristic cytoplasmic protrusions or blebs in tumor cells is a common morphologic finding in the peripheral blood film irrespective of the nuclear features, small cell variants lacking the typical nuclear features can cause diagnostic problems in clinical cytology. Furthermore, the small leukemic cells can share some cytologic findings with lymphocyte-rich serous effusions caused by non-neoplastic reactive lymphocytosis as well as other small lymphocytic lymphoproliferative disorders. Here, we describe the cytological findings of ascitic fluid complicated by small cell variant T-PLL in a 54-year-old man, the cytology of which was initially interpreted as small lymphocytic malignancy such as small lymphocytic lymphoma/chronic lymphocytic leukemia.

• Journal of Chest Surgery
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• v.34 no.12
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• pp.956-959
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• 2001

The Iymphoproliferative disease after the organ transplantation is more commonly seen with the increase according to the increasing number of the organ transplantations and it occurs more frequently in the cases of heart and lung transplantations that needs more aggressive immunosuppression. It demands urgent evaluation and management because of poor prognosis. We transplanted left lung of a man to the woman who suffered from severe dyspnea due to terminal pulmonary emphysema in discrepancy of ABO blood type. Postoperatively, We used triple regimen immunotherapy(cyclosporin, azathioprine, prednisolone) and followed up in the out patient clinic. During the follow up, we found abnormal mass lesion on the transplanted lung and performed gun biopsy. We confirmed malignant lymphoma on the pathollgic examination and two cycled chemotherapy was given after reducing dose of immunosupression. The patient died of sudden onset of pulmonary edema of the transplanted lung.

• Korean Journal of Head & Neck Oncology
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• v.20 no.2
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• pp.194-197
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• 2004

Castleman's disease was first described from Benjamin Castleman in 1956. The disease today is enumerated among lymphoproliferative disorders and has unknown etiology. Although the mediastinum is a their most common location, they also occurs in other areas of the body, usually where lymph nodes are normally found. Castleman's disease of the neck is very rare. We describe a case of Castleman's disease of the neck in a middle aged man, and discuss the clinical presentation, radiological findings, and pathological features.