• Journal of Yeungnam Medical Science
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• 제37권2호
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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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• 제13권1호
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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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• 제24권1호
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• pp.44-53
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• 2017

목적: 소아 심장이식 환자에서 림프증식성 질환(posttransplant lymphoproliferative disorder [PTLD]) 발생과 Epstein-Barr virus (EBV) 정량값의 상관관계를 분석하고, PTLD의 발생에 영향을 주는 인자들에 대해서 알아보고자 하였다. 방법: 2006년 1월부터 2015년 3월까지 최근 9년간 서울아산병원 어린이병원에서 심장이식을 받은 18세 미만 환자 중 이식 후 최소 1개월 이상 추적 관찰이 가능한 경우를 대상으로 후향적으로 의무기록을 분석하였다. 결과: 총 40명의 심장이식 환자의 진단 시 나이의 중앙값은 11.5세(범위, 0.3-17.8세)였으며 이 중 3명에서 이식 후 4.3개월, 6.3개월 및 17개월째 PTLD가 발생하였다. 이식 후 혈중 EBV 정량 검사를 시행한 28명 중 최소 1회 이상 EBV 바이러스혈증이 관찰된 경우는 7명이었으며, PTLD로 진단받은 환자 3명이 모두 ${\geq}10,000copies/mL$의 EBV 바이러스혈증이 선행되어 있었다. PTLD 발생군은 PTLD 비발생군에 비해 어린 나이에 이식을 받았으며(P=0.021), 초기 및 최고 혈중 EBV 역가가 더 높은 경향을 보였으나 통계적인 차이는 없었다. 결론: 소아 심장이식 환자에서 이식 당시 어린 연령 및 이식 후 EBV 바이러스혈증 동반 여부가 PTLD 발생과 관련이 있었다. 추후 전향적인 연구를 통해서 소아 심장이식 환자에서 PTLD 발생을 적절히 예측할 수 있는 EBV 정량값의 지표를 구하는 것이 필요하겠다.

• 대한세포병리학회:학술대회논문집
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• 대한세포병리학회 1992년도 제5차 추계학술대회 초록집
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• pp.15.2-16
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• 1992

• Asian Pacific Journal of Cancer Prevention
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• 제14권11호
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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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• 제58권3호
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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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• 제73권2호
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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.

• 대한세포병리학회지
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• 제19권2호
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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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• 제34권12호
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• pp.956-959
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• 2001

이식 후 발생하는 임파구 증식성 질환은 장기 이식의 예가 늘어나면서 증가하는 추세이며 폐 ,심장 이식과 같이 면역 억제 요법의 강도가 심할수록 그 발생률이 높아진다. 특히 임파구 증식성 질환 중 악성 임파종은 치료가 어렵고 예후가 좋지 않아 신속한 진단 및 치료가 필요하다. 본원에서는 1999년 11월 말기 폐기종으로 호흡 곤란이 심해져 가는 37세 A형 여자 환자에 0형 남자 환자의 좌측 폐를 이식하였다. 폐 이식 수술 후 면역 억제를 위해 3제 치료(싸이클로스포린, 아자씨오프런, 프레드니졸론)를 시행하였고, 환자는 이식 후 특별한 문제없이 외래 추적 관찰 중 정기적으로 시행한 가슴 컴퓨터 단층 촬영 상 이식 폐에 비정상적 종괴가 보여 2001년 2월 gun biopsy 시행 후 악성 임파종으로 진단을 받았으며 면역 억제제 감량 후 항암 화학요법 2회 시행하고 경과 관찰 중 갑자기 발생한 이식 폐부종으로 사망하였다.

• 대한두경부종양학회지
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• 제20권2호
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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.