• Childhood Kidney Diseases
• /
• 제7권2호
• /
• pp.245-252
• /
• 2003

Posttransplant lymphoproliferative disease(PTLD)는 이식 후 발생하는 림프증식성 질환으로 이식장기의 거부반응을 억제하기 위한 면역억제제의 사용 및 이에 따른 EBV 감염과 연관이 있다고 알려져 있다. 소아 PTLD의 경우 성인에 비해 EBV의 초감염 또는 재활성이 더 많은 것으로 보고되고 있으며 최근 더 강력한 면역억제제들의 개발 및 사용에 따라 발생이 증가하고 있다. 본 증례는 14세 여아로 신이식 44개월 후에 EBV 감염의 증거 없이 지발성 PTLD가 발생하였으며 골수 검사상 B-세포 급성 림프구성 백혈병으로 진단되어 항암화학요법 치료를 시작하였고, 치료 후 완전 관해는 이루어졌으나 심한 중성구 감소증에 따른 패혈성 쇼크로 입원 77일만에 사망하였다.

• 대한두개안면성형외과학회지
• /
• 제22권4호
• /
• pp.199-203
• /
• 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
• /
• 제6권1호
• /
• pp.123-130
• /
• 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.

• Advances in pediatric surgery
• /
• 제19권1호
• /
• pp.14-21
• /
• 2013

Pediatric liver transplantation has evolved into a definite and effective therapeutic modality for various liver diseases in the pediatric patient. During the last 25 years, liver transplant outcomes in Korea have reached international standards and Korea has become the leader in living-donor liver transplantation. This review will present the cumulative outcomes of pediatric liver transplantation performed in Korea and will focus on other issues of interest involving pediatric liver transplant recipients, especially in the field of immunosuppression and post-transplant lymphoproliferative disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제7권1호
• /
• pp.92-97
• /
• 2004

저자들은 간 이식 후 지속적 비기능 항진에 발생된 내과적 치료에 반응하지 않는 림프세포증식성질환의 6세된 남아에서 부분적 비동맥 색전술을 치료의 한 방법으로 선택하였다. 비기능항진의 억제 효과로 면역력 증강을 통해 혈액학적 호전을 유도하고자 하였으며 치료 후 PTLD의 호전을 보인 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• 대한두경부종양학회지
• /
• 제21권1호
• /
• pp.48-52
• /
• 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.

• IMMUNE NETWORK
• /
• 제14권1호
• /
• pp.1-6
• /
• 2014

Epstein-Barr virus (EBV) is etiologically associated with a variety of diseases including lymphoproliferative diseases, lymphomas, carcinomas, and autoimmune diseases. Humans are the only natural host of EBV and limited species of new-world monkeys can be infected with the virus in experimental conditions. Small animal models of EBV infection, required for evaluation of novel therapies and vaccines for EBV-associated diseases, have not been available. Recently the development of severely immunodeficient mouse strains enabled production of humanized mice in which human immune system components are reconstituted and express their normal functions. Humanized mice can serve as infection models for human-specific viruses such as EBV that target cells of the immune system. This review summarizes recent studies by the author's group addressing reproduction of EBV infection and pathogenesis in humanized mice.

• Journal of Yeungnam Medical Science
• /
• 제37권2호
• /
• pp.136-140
• /
• 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.

• Childhood Kidney Diseases
• /
• 제24권2호
• /
• pp.75-82
• /
• 2020

Objectives: We aimed to investigate the incidence, manifestations, and outcomes of malignancy after pediatric kidney transplantation (KT) at our center over 30 years. Methods: We retrospectively reviewed the medical records of 155 patients under 18 years of age who underwent KT between January 1990 and February 2020 at Asan Medical Center. Results: Twelve patients (7.7%) were diagnosed with a malignancy after KT. Malignancy was diagnosed after a mean period of 6.4±5.9 years (median 4.6, range 0.5-20.6 years) after KT. Nine (75.0%) of the 12 cancer patients were diagnosed with post-transplant lymphoproliferative disease (PTLD), and the other three had papillary thyroid cancer, mucoepidermoid cancer of the hard palate, and T-cell acute lymphoblastic leukemia, respectively. PTLD was diagnosed within a mean of 3.7±3.4 years (median 3.7, range 0.5-9.8 years) after KT. Five patients diagnosed with PTLD were cured without recurrence. Three patients with PTLD died from the disease, and one patient with mucoepidermoid cancer from a non-PTLD malignancy died after progression, despite surgical resection and chemotherapy. Three (33.3%) of the nine survivors progressed to end-stage renal disease (ESRD) after completing cancer treatment. No patient with post-transplant malignancy (PTM) experienced critical renal deterioration during cancer treatment. Conclusion: PTLD was the most common PTM, occurring at 5.8% of the pediatric KT patients after KT in our center. Careful follow up is needed particularly considering the risk of PTLD after KT in children.

• Journal of Korean Neurosurgical Society
• /
• 제43권3호
• /
• pp.169-171
• /
• 2008

Giant lymph node hyperplasia (Castleman's disease) is a nonneoplastic lymphoproliferative disorder of unknown etiology that usually occurs in the chest. Its morphological recognition is based on a composition of various histological features. The mass is often asymptomatic, but it can cause nonspecific thoracic symptoms, such as regional pain. This disease can be found wherever lymph nodes are present, but two-thirds of these tumors are found in the chest, along the tracheobronchial tree in the mediastinum or lung hilus. However, we experienced an unusual case of Castleman's disease as a cause of back pain that was localized in the posterior mediastinum bordering the chest wall.