• 제목/요약/키워드: Lymphoproliferative disorder

검색결과 33건 처리시간 0.027초

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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    • 제9권2호
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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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    • 제43권3호
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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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    • 제64권2호
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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.

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

  • 이정화;고재성;서정기;이남준;서경석;이건욱;강경훈
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제12권2호
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    • pp.199-206
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    • 2009
  • 목 적: 간 이식 소아에서 발생한 PTLD의 경험을 통해 발현 양상, 발병 위험 인자, 진단 방법, 치료 및 예후에 영향을 줄 수 있는 요인들을 알아보았다. 방 법: 1988년 1월부터 2009년 6월까지 서울대학교 어린이병원에서 간 이식을 받은 소아 148명 중 PTLD로 진단된 8명의 환아들을 대상으로 후향적 분석을 하였다. 이식당시 나이, 이식 후 PTLD 증상이 나타날 때 까지의 기간, 임상적 증상, 조직학적 소견, EBV 검사 결과, 치료 및 경과에 대해 조사하였다. 결 과: 간 이식 후 PTLD의 유병률은 5.4%였고, 발생시기는 조기 PTLD가 6명(75%), 후기 PTLD가 2명(25%)이었다. 대상 환아의 간 이식 당시 나이는 평균8.1${\pm}$4.4개월로, 12개월 미만이 7명(87.5%), 12개월 이후가 1명(12.5%)이었다. PTLD 진단 시 주증상은 발열, 설사, 혈변이었고 모두 장이나 장간막 림프절 침범이 있었다. 3명은 개복 수술로 5명은 상부 위장관 내시경이나 대장 내시경을 통한 조직 검사로 진단되었다. 조직학적 진단은 early lesion이 3명, polymorphic PTLD 2명, monomorphic PTLD 1명, Burkitt 림프종 1명, B 세포 림프종이 1명이었다. EBV는 7명에서 양성을 보였다. 치료는 전례에서 1차 치료로 면역억제제를 중지하고 항바이러스제(ganciclovir)를 투여 하였다. 4명의 환아들에서 rituximab을 추가하였고 Burkitt 림프종과 B 세포림프종으로 진단된 환아들은 각각 항암 치료를 병행하였다. B 세포 림프종으로 진단되어 치료 중에 패혈증으로 1명이 사망하였으며 나머지 7명은 호전되었다. 결 론: 1세 미만에 간 이식을 받은 경우가 PTLD 발생의 주요한 위험 인자였으며, EBV viral load의 지속적인 감시와 위장관 내시경을 통한 조직 검사가 PTLD 조기진단에 유용할 것으로 생각한다.

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

  • 노민호;배공근;반명진;박재홍;이승원;박기남;김재욱;고윤우
    • 대한두경부종양학회지
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    • 제31권1호
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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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Prednisolone 투여로 효과적으로 치료된 소아의 전신형 Castleman병 1례 (A Case of Systemic Castleman's Disease in a Child and Successful Treatment with Oral Prednisolone)

  • 구소은;이미정;김정은;허주령;김태형
    • Clinical and Experimental Pediatrics
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    • 제48권4호
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    • pp.443-447
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    • 2005
  • 저자들은 지속되는 발열, 빈혈, 혈소판 감소증, 흉수, 복수 및 간비종대 등의 비특이적인 전신증상을 주소로 내원한 13세 남아를 경부 림프절 조직 검사에서 혼합형의 Castleman병으로 진단한 후 스테로이드와 면역글로불린을 투여하여 호전된 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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

  • 김은아;이종국;김한성
    • Clinical and Experimental Pediatrics
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    • 제46권2호
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    • pp.203-206
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    • 2003
  • 저자는 우연히 발견된 무통성 종괴로 내원한 환아에서 소아과 영역에서 드문 Castleman 씨 병 2례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. 림프절 종창으로 추정되는 무통성의 종괴를 보이는 환아에서는 Castleman 씨 병을 감별 진단으로 반드시 고려해 보아야 할 것이다.

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

  • 손진희;정은하;박혜림
    • 대한세포병리학회지
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    • 제9권2호
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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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2-Chlorodeoxyadenosine에 의해 완전관해가 유도된 모발상 세포백혈병 1예 (A Case of Complete Remission of Hairy Cell Leukemia by 2-Chlorodeoxyadenosine)

  • 김용길;이상진;김민경;이경희;현명수;조희순
    • Journal of Yeungnam Medical Science
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    • 제21권2호
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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
    • 대한두개안면성형외과학회지
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    • 제18권2호
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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.