• 대한두개안면성형외과학회지
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• 제22권4호
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• pp.199-203
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• 2021

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease characterized by a single mass on the face or upper part of the trunk. It usually presents an asymptomatic and favorable progression, and its histopathologic findings include small and medium-sized lymphoid cells. The authors report a case of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder on the forehead. A 51-year-old man presented with a protruding mass on his forehead that the patient had noted 1 month previously. Surgical excision and a permanent biopsy were performed under local anesthesia. Based on the biopsy results, the mass was diagnosed as a primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was no evidence of recurrence at a 15-month follow-up visit.

• Childhood Kidney Diseases
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• 제6권1호
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• pp.123-130
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• 2002

이식후 발생하는 림프증식성 질환(PTLD)은 양성 다크론성 B림프구 증식증으로부터 악성의 단크론 림프종에 이르기까끼 다양한 임상상을 보이는 질환이다. PTLD는 Epstein-Barr virus(EBV)와 과도한 면역억제와 밀접한 관계가 있다. 국내에서는 1997년 성인에서 신 이식 후 발생한 예가 처음 보고되었으며, 소아에서는 간 이식 후 발생한 예가 있을 뿐 신 이식 후 발생한 증례의 보고는 없어 이 보고가 국내 최초로 신이식 소아에서 발생한 PTLD 증례이다. 환자는 9세 남아로서 긴이식후 4개월후에 PTLD가 발생하였으며 주된 임상 양상은 발열, 림프절 종대와 혈변이었다. 림프절과 대장의 생검조직 에서 EBV in situ hybridization이 양성이고, 조직학적으로 B 세포계열의 PTLD에 합당한 소견이었다. 모든 증상은 면역억제제 감소와 ganciclovir 치료로 완전 관해되었다.

• 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.

• 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.

• 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.

• Clinical and Experimental Pediatrics
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• 제60권3호
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• pp.86-93
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• 2017

Purpose: To evaluate the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after solid organ transplantation (SOT) in children. Methods: We retrospectively reviewed the medical records of 18 patients with PTLD who underwent liver (LT) or kidney transplantation (KT) between January 1995 and December 2014 in Seoul National University Children's Hospital. Results: Eighteen patients (3.9% of pediatric SOTs; LT:KT, 11:7; male to female, 9:9) were diagnosed as having PTLD over the last 2 decades (4.8% for LT and 2.9% for KT). PTLD usually presented with fever or gastrointestinal symptoms in a median period of 7 months after SOT. Eight cases had malignant lesions, and all the patients except one had evidence of Epstein-Barr virus (EBV) involvement, assessed by using in situ hybridization of tumor tissue or EBV viral load quantitation of blood. Remission was achieved in all patients with reduction of immunosuppression and/or rituximab therapy or chemotherapy, although 1 patient had allograft kidney loss and another died from complications of chemotherapy. The first case of PTLD was encountered after the introduction of tacrolimus for pediatric SOT in 2003. The recent increase in PTLD incidence in KT coincided with modification of clinical practice since 2012 to increase the tacrolimus trough level. Conclusion: While the outcome was favorable in that all patients achieved complete remission, some patients still had allograft loss or mortality. To prevent PTLD and improve its outcome, monitoring for EBV infection is essential, which would lead to appropriate modification of immunosuppression and enhanced surveillance for PTLD.

• Journal of Yeungnam Medical Science
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• 제34권1호
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• pp.84-87
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• 2017

Nodular lymphoid hyperplasia (NLH) is a benign lymphoproliferative disease that can affect the lung. Because of its rarity, little is known about the etiology and natural history of NLH. Most cases are usually asymptomatic and found incidentally on imaging studies. Imaging finding of NLH has shown most commonly as a solitary lesion, although multifocal pulmonary nodules may be seen. Surgical resection has proved curative in the cases previously described. We report a rare case of NLH in a 55 year-old man who presented with bilateral multiple pulmonary nodules on chest radiography. Open biopsy was performed from the upper and lower lobe of the left lung. The lesions were pathologically diagnosed as pulmonary NLH. Multifocal residual nodules in both lungs remain stable without spontaneous regression during the 3 years of follow-up.

• 대한두경부종양학회지
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• 제21권1호
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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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권1호
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• pp.39-46
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• 2003

목 적: EBV의 초감염과 면역억제의 강도는 소아 간이식에서 PTLD 발생의 주요 위험인자로 알려져 있다. 삼성서울병원에서 6년간 경험한 PTLD 5례의 임상양상을 분석해보고자 한다. 대 상: 1996년부터 2002년 6월까지 41례의 소아간이식 환아를 대상으로 하였는데 이중 7명이 사망하였고, PTLD로 사망한 1명을 포함한 35명을 대상으로 분석하였다. 방 법: 공여자의 EBV 항체가 양성이고 수혜자가 음성일 경우 고위험군으로, 그 외의 경우를 저위험군으로 정의하였다. 위험군 분류, 면역억제제 종류, 간이식이 행하여진 나이, 수술 후 PTLD 진단까지의 기간, 수술 후 EBV 항체의 양전 시기, 거부반응에 대한 치료 여부, PTLD의 증상들인 발열, 설사, 빈혈, 장출혈 식욕부진 저알부민혈증 등의 발현 여부를 후향적으로 조사하였다. 결 과: 1) PTLD는 전체 소아 간이식 환아 41명 중 5명에서 발생하였다(12.2%). 고위험군은 16명으로 이 중 5명(31.3%)에서 PTLD가 발생하였고 저위험군 19명중에서는 한 명도 발생하지 않았다. 2) 사망례를 제외한 4명은 tacrolimus로 면역억제 치료를 받았으며 수술 당시 나이는 평균 10.8개월이었고 수술 후 PTLD 진단까지 평균 9.8개월이 걸렸 다. EBV 양전 시기는 수술 후 평균 6개월이었다. 3) 사망례를 포함한 5명 중 3명에서 PTLD 진단 이전에 간이식 거부반응이 있었고 스테로이드 pulse 치료 등 면역억제를 증가시키는 치료를 행하였었다. 4) 한 명에서 쉰 목소리 증상이 발견되어 조직검사 상 후두 PTLD와 장 PTLD로 진단하였고 나머지 네 명은 모두 혈변이 있었으며 대장 조직검사 상 장 PTLD로 진단하였다. 5) 빈혈과 저알부민 혈증은 모든 환아에서 나타나고 있었고 발열, 설사, 장출혈이 5명 중 4명에서 보였으며 식욕부진은 5명 중 3명에서 나타났다. 결 론: 10%가 넘는 발병률을 보이는 PTLD는 소아 간이식 후 매우 중요한 합병증으로서 특히 EBV의 고위험군에서 31%에 이르는 발병률을 가지고 있는 바 이들에 대하여 EBV 상태와 면역억제 유지에 대한 철저한 대비가 있어야 할 것으로 생각된다. 특히 빈혈, 저알부민혈증, 발열, 설사, 장출혈 등이 동반할 때에는 PTLD를 의심해 볼 수 있다. PTLD의 공통적인 발병양상으로는 첫째, 공여자가 EBV 양성이고 수혜자가 음성이면서, 둘째, EBV가 수술 후 6개월 경 양전되며, 셋째, 1세 전후에 수술을 시행하고 넷째, 간이식 거부반응을 겪으면서 면역억제의 강도가 높아졌을 경우 등이다.

• 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.