• Archives of Craniofacial Surgery
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• v.22 no.4
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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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• v.6 no.1
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• pp.123-130
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• 2002

Posttransplant lymphoproliferative disease (PTLD) represents a diverse lymphoproliferative disorder ranging front nonspecific reactive hyperplasia to malignant immunoblastic sarcoma developed in a setting of immunosuppression following organ or cellular transplantation. It is often associated with Epstein-Barr virus (EBV) infection and high dose immunosuppression. PTLD after renal transplantation was reported at first in adult in Korea in 1997. In children there have been several cases of PTLD after liver transplantation but PTLD after renal transplantation has not been reported. This is a case report of PTLD developed 4 months after renal transplantation in a 9-year-old boy. The major clinical manifestations were fever, multiple lymph nodes enlargement and blood-tinged stool. EBV was detected by in-situ hybridization in the enlarged cervical lymph node and the colonic tissue. Histological examination revealed B-cell lineage. Use of ganciclovir and reduction of the immunosuppression level resulted in complete remission of PTLD. This is the first pediatric case report of PTLD following renal transplantation in Korea. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 123-30)

• 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.

• 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.

• 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.

• Clinical and Experimental Pediatrics
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• v.60 no.3
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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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• v.34 no.1
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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.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.1
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• pp.39-46
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• 2003

Purpose: In a retrospective study for the pediatric patients who underwent liver transplantation in the past 6 years at Samsung Medical Center, the clinical features of 5 patients with posttransplant lymphoproliferative disorder (PTLD) were analyzed. Methods: Between June 1996 and June 2002, 41 pediatric patients underwent liver transplantation. Seven of them died in the postoperative period. Thirty-five including one patient who died of PTLD were finally reviewed. Patients were divided into two groups: high risk group, EBV naive recipients of EBV-positive grafts; low risk group, the patients other than those in high risk group. The authors reviewed age at operation, immunosuppressive agent, postoperative duration until diagnosis, postoperative duration until EBV seroconversion, presence of treatment against rejection, and presenting symptoms of PTLD. Results: Five of 41 patients (12.2%) developed PTLD. All of them belonged to high risk group, and the incidence of PTLD in high risk group was 31.3% (5/16). The mean age at operation was 10.8 months old and the mean duration between operation and diagnosis for PTLD was 9.8 months. Primary EBV infection developed after a median of 6 months after transplantation. One patient was diagnosed as laryngeal and gastrointestinal PTLD and the other four, gastrointestinal PTLD. The following symptoms and signs were seen in the patients: anemia (100%), hypoalbuminemia (100%), fever (80%), diarrhea (80%), gastrointestinal bleeding (80%), and anorexia (60%). Conclusion: PTLD is one of the major complications after pediatric liver transplantation, especially in the group of high-risk recipients. Anemia, hypoalbuminemia, fever, diarrhea and gastrointestinal bleeding were features that are characteristic of PTLD. The common features of PTLD development were: (i) EBV-positive donors placed into EBV naive recipients, (ii) primary EBV infection about 6 months after transplantation, (iii) young age, about 1 year old at operation, and (iv) the requirement for intensive posttransplant immunosuppression.

• 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.