• Title/Summary/Keyword: Lymphoproliferative disorder

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Photosensitivity in Hydroa Vacciniforme Is A Photo- Koebner Phenomenon Induced by Infiltration of Epstein-Barr Virus-Infected NK/T Cells

  • Keiji Iwatsuki;Xu, Zi-Gang;Mikio Ohtsuka;Takenobu Yamamoto;Kazuyasu Fujii;Kazuhide Tsuji
    • Journal of Photoscience
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    • v.9 no.2
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    • pp.233-235
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    • 2002
  • Hydroa vacciniforme (HV) is a disease of Epstein-Barr virus (EBV)-associated NK/T cell lymphoproliferative disorder, and patients with severe HV-like eruptions have a high risk to progress to various EBV-related hematological complications. Photosensitivity in HV might be a 'photo-Koebner' phenomenon mediated by infiltration of EBV-infected NK/T cells.

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Giant Lymph Node Hyperplasia (Castleman's Disease) as a Rare Cause of Back Pain

  • Kim, Kyoung-Han;Kim, Sang-Dae;Kim, Se-Hoon;Park, Jung-Yul
    • Journal of Korean Neurosurgical Society
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    • v.43 no.3
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    • pp.169-171
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    • 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.

Pediatric heart transplantation: how to manage problems affecting long-term outcomes?

  • Kim, Young Hwue
    • Clinical and Experimental Pediatrics
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    • v.64 no.2
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    • pp.49-59
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    • 2021
  • Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

Posttransplantation Lymphoproliferative Disorder after Liver Transplantation in Pediatric Patients: Report from a Single-center Over 21 Years (간 이식 소아에서 발생한 이식 후 림프 증식 질환: 단일 기관에서의 21년 경험)

  • Lee, Jung-Hwa;Ko, Jae-Sung;Seo, Jeong-Kee;Yi, Nam-Joon;Suh, Kyung-Suk;Lee, Kuhn-Uk;Kang, Gyeong-Hoon
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.12 no.2
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    • pp.199-206
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    • 2009
  • Purpose: To analyze the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after liver transplantation in children. Methods: From January 1988 to June 2009, we retrospectively reviewed the medical records of 8 PTLD cases among 148 pediatric patients underwent liver transplantation. The age at transplantation, time of presentation after transplantation, clinical manifestations, histologic diagnosis, results of EBV (Epstein-Barr virus) assessments, managements and outcomes of PTLD were investigated. Results: The prevalence of PTLD in liver transplant pediatric recipients was 5.4% (8 of 148). The mean age of patients was 25.4${\pm}$21.3 months (range 10 to 67 months). Seven of 8 patients (87.5%) underwent liver transplantation before 1 year of age. The common clinical presentations were persistent fever (8 of 8, 100%) and bloody diarrhea (6 of 8, 75%). PTLD was diagnosed with gastrointestinal endoscopic biopsies in five patients and surgical biopsies in three. Histologic findings showed early lesion in three patients, polymorphic in two, and monomorphic in three. Burkitt lymphoma and lymphoblastic lymphoma were found in two of 3 monomorphic patients. Seven of 8 patients were found with EBV-positive. Eight patients were treated with dose reduction of immunosuppressants and infusion of ganciclovir. Rituximab was added to four patients. PTLD were successfully managed in all patients except one who died of sepsis during chemotherapy. Conclusion: Major risk factor of PTLD was to undergo liver transplantation before 1 year of age. Continuous monitoring for EBV viral load and gastrointestinal endoscopic biopsy may be useful to early detection of PTLD.

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Synchronous Mucoepidermoid Carcinoma of Parotid Gland and Unicentric Cervical Cathleman's Disease : A Case Report (점액표피양 암종과 동반된 경부 캐슬만 병 1예)

  • Noh, Min Ho;Bae, Kong Geun;Ban, Myung Jin;Park, Jae Hong;Lee, Seung Won;Park, Ki Nam;Kim, Jae Wook;Koh, Yoon Woo
    • Korean Journal of Head & Neck Oncology
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    • v.31 no.1
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    • pp.14-17
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    • 2015
  • Castleman's disease is an uncommon lymphoproliferative disorder. The disorder can be classified based on histological subtype, such as hyaline vascular type, plasma cell type, and mixed type, and can also be clinically divided into either unicentric or multicentric type. Its exact pathophysiology is not clearly identified. The unicentric type is able to be treated by surgical resection. However, there is no standard treatment modlity for the multicentric type. Treatment of multicentric type includes anti-cancer chemotherapy and radiation therapy. Recently, authors have experienced a rare case of unicentric type of Castleman's disease accompanying a mucoepidermoid carcinoma of parotid gland and report a case which is discussed with references.

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A Case of Systemic Castleman's Disease in a Child and Successful Treatment with Oral Prednisolone (Prednisolone 투여로 효과적으로 치료된 소아의 전신형 Castleman병 1례)

  • Koo, So Eun;Lee, Mee Jeong;Kim, Jeong Eun;Huh, Joo Ryung;Ghim, Taed
    • Clinical and Experimental Pediatrics
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    • v.48 no.4
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    • pp.443-447
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    • 2005
  • Castleman's disease is a rare non-neoplastic lymphoproliferative disorder of unknown etiology. It is divided into three histologic subtypes; hyaline-vascular(HV), plasma cell(PC) type and mixed type (HV-PC). It has two clinical expressions. The localized form, which presents as a slow growing mass, has a relatively benign clinical course. The multicentric form is multilocated and holds significant morbidity. The mainstay of treatment of the localized form is surgical resection. The multicentric form requires medical treatment comprising prednisolone and other immunosuppressor drugs. The disease in children seems to have a more favorable course than in adults. We report a 13-year-old boy with Castleman's disease of multicentric form who was successfully treated with prednisolone and intravenous immunoglobulin.

Two Cases of Castlema's Disease in Childern (소아에 발생한 Castleman's Disease 2례)

  • Kim, Eun Ah;Lee, Chong Guk;Kim, Han Sung
    • Clinical and Experimental Pediatrics
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    • v.46 no.2
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    • pp.203-206
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    • 2003
  • Castleman's disease is an atypical lymphoproliferative disorder of unknown origin. It has three histologic variants(hyaline vascular, plasma-cell, and mixed) and two clinical types(localized and multicentric). Some sufferers have constitutional symptoms and laboratory abnormalities such as anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. The localized form is cured by complete surgical excision whereas the multicentric form is managed by prednisone and other immunosuppressor drugs. The prognosis of the multicentric form is worse than the localized form since malignancies and severe infections may lead to a rapidly fatal outcome. Castleman's disease has been rarely reported at pediatric age in Korea. We experienced two cases of Castleman's disease detected at 3 and 5 years of age. They were presented with painless enlargement of submandibulars and axillary lymph nodes but had no associated symptoms. The lesions were excised and diagnosed as Castleman's disease, and no recurrence was noted during follow-up periods.

Cytologic Findings of Infectious Mononucleosis Lymphadenitis - A Report of Four Cases - (전염성 단핵구중 림프절염의 세침흡인 세포학적 소견 - 4예 보고 -)

  • Sohn, Jin-Hee;Jung, Eun-Ha;Park, Hye-Rim
    • The Korean Journal of Cytopathology
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    • v.9 no.2
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    • pp.227-231
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    • 1998
  • Infectious mononucleosis(IM) Is an acute self-limiting lymphoproliferative disorder associated with infection by the Epstein-Barr Virus(EBV), with the characteristic triad of fever, sore throat, and cervical or generalized lymphadenopathy. And also there are atypical lymphocytes in the peripheral blood. Cytological findings of IM lymphadenitis are characterized by a florid immunoblastic and atypical lymphoid cell proliferation. However, the small number of cases were studied by fineneedle aspiration cytology(FNAC) even though there was a complexity of lymph node pathology. It is important to recognize the reactive pattern of IM that would initiate EBV study and to avoid unnecessary biopsy We studied findings of lymph node FNAC from 4 patients with EBV infection confirmed by EBV-specific serologic studios. All of the cases were positive for viral capsid antigen(VCA) and one case was positive for anti-EBV nuclear antigen(EBNA). Cytologically, all of the cases exhibited high cellularity and atypia with great numbers of large immunoblastic lymphocytes.

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A Case of Complete Remission of Hairy Cell Leukemia by 2-Chlorodeoxyadenosine (2-Chlorodeoxyadenosine에 의해 완전관해가 유도된 모발상 세포백혈병 1예)

  • Kim, Yong-Gil;Lee, Sang-Jin;Kim, Min-Kyung;Lee, Kyung-Hee;Hyun, Myung-Soo;Cho, Hee-Sun
    • Journal of Yeungnam Medical Science
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    • v.21 no.2
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    • pp.237-241
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    • 2004
  • Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder that is characterized by cytopenia, splenomegaly, and mononuclear cells displaying cytoplasmic projections. We experienced a case of hairy cell leukemia that developed in a 38-year-old man. He showed marked splenomegaly without palpable lymphoadenopathy. A complete blood cell count revealed leukopenia ($3300/{\mu}{\ell}$ with 63% of lymphocyte) and the peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow smear revealed abnormal lymphocytes and severe myelofibrosis. Tartrate-resistant acid phosphatase reactivity was strongly positive in the hairy cells. The immunophenotyping results of lymphoid cells were CD5(-), CD10(-), CD19(+), CD25(+), CD103(+), CD20(+), lambda(+). The patient was treated with 2-Chlorodeoxyadenosine at a daily dose of 0.1mg/Kg by a continuous intravenous infusion for 7 days. The patient achieved complete remission.

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Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature

  • Ryu, Wan Cheol;Park, Moon Hyang;Kim, Hoon;Koh, In Chang;Kim, Kyu Nam
    • Archives of Craniofacial Surgery
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    • v.18 no.2
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    • pp.122-127
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    • 2017
  • Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.