• Title/Summary/Keyword: Lymphoproliferative disease

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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.

A Case of Multicentric Castleman's Disease Presenting with Follicular Bronchiolitis

  • HwangBo, Yup;Cha, Seung-Ick;Lee, Yong Hoon;Lee, So Yeon;Seo, Hyewon;Oh, Serim;Kim, Minjung;Choi, Sun Ha;Park, Tae In;Shin, Kyung-Min
    • Tuberculosis and Respiratory Diseases
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    • v.74 no.1
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    • pp.23-27
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    • 2013
  • Multicentric Castleman's disease (CD) is a rare atypical lymphoproliferative disorder, which is characterized by various systemic manifestations. Some patients with multicentric CD may have concomitant lung parenchymal lesions, for which lymphoid interstitial pneumonia (LIP) is known to be the most common pathologic finding. Follicular bronchiolitis and LIP are considered to be on the same spectrum of the disease. We describe a case of multicentric CD with pulmonary involvement, which was pathologically proven as follicular bronchiolitis.

A Case of Cervical Malignant Lymphoma Coexisted with Multicentric Castleman's Disease (다발성 캐슬만병과 공존한 경부 악성 림프종 1례)

  • Jang, Gyu Ho;Jung, Young Do;Seo, Youn Tae;Kim, Jeong Kyu
    • Korean Journal of Head & Neck Oncology
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    • v.32 no.2
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    • pp.35-39
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    • 2016
  • Castleman's disease (CD) is an uncommon lymphoproliferative disorder. The disease entity is classified into 2 clinical subtypes, unicentric and multicentric type. Prevalence of lymphoid malignancy in multicentric CD (MCD) is very low. In this case, we report a case of 77 years old woman who developed high fever and swelling in both side of her neck. Neck lymph node biopsy revealed plasma cell hyperplasia. Patient's symptom was subsided after treatment with Dexamethasone. Three months later, multiple lymph node enlargement was developed in abdomen and neck area again. Repeated neck lymph node biopsy confirmed diffuse large B cell lymphoma. The patient started chemotherapy.

Evolving Problem Analyses of Recent Marek's Disease (최근 진화하는 마렉병의 원인 분석)

  • Jang, H.K.;Park, Y.M.;Cha, S.Y.;Park, J.B.
    • Korean Journal of Poultry Science
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    • v.34 no.4
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    • pp.301-318
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    • 2007
  • Marek's disease (MD) is a highly contagious lymphoproliferative disease of poultry caused by the oncogenic herpesvirus designated Marek's disease virus (MDV). MD has a worldwide distribution and is thought to cause an annual loss over US$ one billion to the poultry industry. Originally described as a paralytic disease, today MD is mostly manifested as an acute disease with tumors in multiple visceral organs. MD is controlled essentially by the widespread use of live vaccines administered either in ovo into 18-day-old embryos or into chicks immediately after they hatch. In spite of the success of the vaccines in reducing the losses from the disease in the last 30 years, MDV strains have shown continuous evolution in virulence acquiring the ability to overcome the immune responses induced by the vaccines. During this period, different generations of MD vaccines have been introduced to protect birds from the increasingly virulent MDV strains. However, the virus will be countered each new vaccine strategy with ever more virulent strains. In spite of this concern, currently field problem from MD is likely to be controled by strategy of using bivalent vaccine. But, potential risk factors for outbreak of MD are still remained in this condition. The major factors can be thought that improper handling and incorrect administration of the vaccine, infection prior to establishment of immunity, suppression of immune system by environmental stress and outbreaks of more virulent MDV strain by using vaccine and genetic resistance of host.

Cytologic Features of Ascitic Fluid Complicated by Small Cell Variant T-cell Prolymphocytic Leukemia -A Case Report - (복수를 침범한 소세포형 T-세포 전림프구성 백혈병의 세포소견 -1예 보고-)

  • Han, Jee-Young;Kim, Jin-Soo;Kim, Dong-Hoon;Kim, Lucia;Park, In-Suh;Kim, Joon-Mee;Chu, Young-Chae;Choi, Suk-Jin
    • 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.

Isolated Intracranial Rosai-Dorfman Disease Mimicking Meningioma: A Case Report (뇌수막종으로 오인된 두개 내에만 발생한 Rosai-Dorfman Disease: 증례 보고)

  • Minji Shin;Young Jin Heo;Donghyun Kim;Hae Woong Jeong;Jin Wook Baek;Ha Young Park
    • Journal of the Korean Society of Radiology
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    • v.83 no.3
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    • pp.719-723
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    • 2022
  • Rosai-Dorfman Disease (RDD) is a rare lymphoproliferative disease, and the occurrence of isolated intracranial RDD is extremely rare. Most cases of intracranial RDDs present as dural masses showing homogenous enhancement on MRI, which makes it difficult to differentiate these masses from meningiomas before surgery unless massive cervical lymphadenopathy is observed. We herein report a rare case of isolated intracranial RDD in a 65-year-old male. Brain MRI revealed a well-defined enhancing mass-like lesion involving the right frontal convexity and subtle diffusion restriction. However, only a subtle blush was observed on the preoperative cerebral angiogram. Although instances of isolated intracranial RDD are rare, it should be considered as a potential differential diagnosis when a dural mass with hypovascularity is visualized on the cerebral angiogram.

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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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.

Small Bowel Tumors and Polyposis: How to Approach and Manage? (소장 종양과 용종증: 접근 방법과 관리)

  • Ko, Bong Min
    • The Korean Journal of Gastroenterology
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    • v.72 no.6
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    • pp.277-280
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    • 2018
  • Although small bowel the mainly occupies the most part of the gastrointestinal tract, small intestine tumors are rare, insidious in clinical presentation, and frequently represent a diagnostic and management challenge. Small bowel tumors are generally classified as epithelial, mesenchymal, lymphoproliferative, or metastatic. Familial adenomatous polyposis and Peutz-Jeghers syndrome are the most common inherited intestinal polyposis syndromes. Until the advent of capsule endoscopy (CE) and device-assisted enteroscopy (DAE) coupled with the advances in radiology, physicians had limited diagnostic examination for small bowel examination. CE and new radiologic imaging techniques have made it easier to detect small bowel tumors. DAE allows more diagnosis and deeper reach in small intestine. CT enteroclysis/CT enterography (CTE) provides information about adjacent organs as well as pictures of the intestinal lumen side. Compared to CTE, Magnetic resonance enteroclysis/enterography provides the advantage of soft tissue contrast and multiplane imaging without radiation exposure. Treatment and prognosis are tailored to each histological subtype of tumors.

Pulmonary Nodular Lymphoid Hyperplasia in a 33-Year-Old Woman

  • Park, Ji Ye;Park, Seong Yong;Haam, Seokjin;Jung, Joonho;Koh, Young Wha
    • Journal of Chest Surgery
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    • v.51 no.2
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    • pp.133-137
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    • 2018
  • Pulmonary nodular lymphoid hyperplasia is a reactive lymphoproliferative disease. It is very rare, which means that many aspects of the disease are unknown or have not been proven. Pulmonary nodular lymphoid hyperplasia can be symptomatic or asymptomatic, progressive or not, and solitary or multiple, and a surgical approach is the current treatment of choice. We present a case of pulmonary nodular lymphoid hyperplasia that was visualized as multiple ground glass opacities on a computed tomography (C T) scan, and observed for 1 year because the patient was pregnant. Over this period, the number and extent of the opacities progressed, but no symptoms were reported. A surgical biopsy was done and some remaining lesions regressed on follow-up CT scans, while others progressed, without any appearance of symptoms.