• Journal of Chest Surgery
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• v.32 no.3
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• pp.326-329
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• 1999

Pulmonary lymphangioleiomyomatosis is a rare disease occurring almost exclusively in woman of reproductive age. It is characterized by rapid deterioration of respiratory functions and results in death within an average of 10 years. This disease is caused by a progressive proliferation of atypical smooth muscle in the pulmonary lymphatic vessels, blood vessels, and airways leading to the development of distal cystic changes which causes frequent recurrent pneumothoraces. We experienced a case of pulmonary lymphangioleiomyomatosis in a 30-year old woman who had a history of bilateral, recurrent pneumothoraces. The patient underwent lung biopsy through right thoracotomy which revealed the diagnosis of pulmonary lymphangioleiomyomatosis. We report a case with a review of the literature.

• Journal of Dental Rehabilitation and Applied Science
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• v.32 no.2
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• pp.130-134
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• 2016

Malignant lymphomas are neoplasms with diffuse proliferation of neoplastic lymphocytes and their precursor cells. Diffuse large B-cell lymphoma, which is a subtype of non-Hodgkin's lymphomas, rarely occurs in the head and neck area and is especially rare in the maxillary sinus. We report a case of a 76-year-old female patient who was referred to the oral and maxillofacial surgery office for evaluation of a dental abscess as a clinical diagnosis. Laboratory tests revealed no signs of inflammation or infection; therefore, incisional biopsy was performed. The final diagnosis was diffuse large B-cell lymphoma in the maxillary sinus. Here we describe this case with a review of relevant literature.

• Tuberculosis and Respiratory Diseases
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• v.43 no.4
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• pp.613-622
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• 1996

Constrictive bronchiolitis, one of small airway diseases, is very rare and occupies one of the two arms of bronchiolitis obliterans together with proliferative bronchiolitis. Proliferative bronchiolitis, presenting the prototype with bronchiolitis obliterans with organizing pneumonia(BOOP), can be easily taken into diagnostic consideration in terms of relatively rapid clinical course and radiologic presentation as if atypical pneumonia with interstitial and alveolar infiltrations. Meanwhile constrictive bronchiolitis is not only very Tare but also easily overlooked as chronic obstructive pulmonary diseases such as emphysema, because it usually shows normal chest radiographic finding and obstructive pattern in pulmonary function test. In the aspects of the response to treatment, proliferative bronchiolitis showed dramatic response to the corticosteroid while constrictive bronchiolitis is intractable, which is easily explained on the basis of the pathologic characteristics of cicartrical replacement of bronchiolar walls. The bronchiolitis, both proliferative and constrictive, can be associated with diverse conditions such as inhalational injury, postinfectious process, drug of chemical induced reactions, connective tissue diseases, and organ trasplantation. And there is idiopathic type which has no associated condition. There is one explanation that both types of bronchiolitis lie on the same disease spectrum because the different disease pattern can be evoked from the same etiology. In contrast, another explanation is suggested that both types of bronchiolitis are one of nonspecific tissue reaction rather than a disease specific histologic finding because the various types of causes can provoke the same histologic findings. These dilemma remains for further investigation. With literature investigation, the authors report a case of constrictive bronchiolitis proven by open lung biopsy in 47 year old female who was diagnosed as non-Hodgkin's lymphoma and simultaneously had relatively rapid progression of airflow obstruction and showed negative radiographic finding without the rise factors for the development of chronic obstructive lung disease. We consider it as idiopathic because we could not find any relationship between constrictive bronchiolitis and non-Hodgkin's lymphoma on the literature search and it requires further investigation.

• Korean Journal of Veterinary Pathology
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• v.6 no.2
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• pp.101-104
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• 2002

닭의 전신장기에 림프종 발생이 특정인 마렉병(Marek's disease; MD)은 림프구성 백혈병(Lymphoid leukosis; LL)과 병리학적 소견이 매우 유사하여 감별이 요구된다. 마렉병 바이러스(Marek's disease virus; MDV)는 질병초기에서 후기에 이르기까지 모낭상피세포에 세포용해성 감염을 지속적으로 일으킨다. 세포용해성 감염이 있는 모낭상피세포는 변성내지 괴사되어 있고 핵내봉입체가 관찰된다. 또한 세포용해성 감염이 있는 모낭상피세포 바로 밑의 진피와 깃털 수질(feather pulp)내의 혈관주위에 림프구 침윤이 관찰된다. 이러한 피부병변은 MD의 특징적인 병변들로서 LL과 감별할 수 있는 결정적인 단서이다. 본 고에서는 MD에 관한 전반적인 것과 특히 MD 진단을 위한 피부병변의 유용성에 대해서 자세히 논하고자 한다. 양계산업에 문제를 야기하고 있는 림프구 증식성 종양은 크게 마렉병(Marek’s disease; MD)과 백혈병(Lymphoid leukosis; LL)이 있다. 이들 질환의 원인체들이 분리되기 전까지는 이들 질병들은 발병부위에 따라 질병명을 붙였다. 즉, 내부장기냐 근육에 발생한 것은 visceral lymphomatosis, 피부에 발생한 것은 skin leukosis, 말초신경의 병변은 poly­n neuritis, neuritis, neurolymphomatosis gallinarum, range paralyis로 불리었다. 또한 눈에 발생한 것은 blindness, gray eye, iritis, uveitis, ocular lymphomatosis라고 불리었다. 1961년에 Biggs는 이러한 림프구 증식성 질병을 마렉병과 백혈병 으로 분류하자는 제안을 하였고, 드디어 1960년대 중반에 림프구 증식성 병변 중의 일부에서 herpesvirus가 분리되어서 Biggs가 제안한 병명인 마렉병을 본격적으로 사용하게 되었다. MD는 마렉병 바이러스(Marek’s disease virus; MDV)가 원 인제로서 닭에 전염성이 강한 염증성에서 종양성의 병변을 내부장기, 피부, 근육, 안구, 중추신경계, 말초신경계 등에 일 으킨다. MDV는 림프종을 유발하기 때문에 처음에는 사람에서 림프종을 유발하는 Epstein-Bar 바이러스와 관련이 있을 것으로 생각되어 gamma-herpesvirus로 분류되었지만, MDV의 게놈 구조와 세포배양에서 빠르게 성장한다는 점 때문에 지금은 alpha-herpesvirus로 재분류 되었다. MDV는 바이러스 중화시험과 한천 겔 침강법에 의해서 3개의 혈청형으로 분류 된다. 혈청형 1은 종양원성 바이러스와 종양원성 바이러스의 계대배양에 의한 약독주가 있다. 혈청형 2는 자연적으로 발 생하는 비종양원성 닭의 MDV이고, 혈정형 3은 바종양원성 칠면조 herpesvirus (HVT)이다. 림프종을 유발하는 MDV 감염은 4개의 과정, 즉 초기 세포용해성 감염, 잠복감염, 후기 세포용해성 감염, 마지막으로 종양화로 나눌 수 있다. 감염의 경로를 보면, 흡입된 MDV는 폐의 대식세포에 감염한 후 전선 장기로 MDV를 전파 시킨다. 특히, 흉선, F냥, 비장 등의 림프구에 초기 세포용해성 감염을 일으키는데, B 림프구가 주로 감염된다. 세포용해성 감염음 방어하기 위해 몰려든 T 림프구가 활성화가 되면, T 세포도 감염되게 된다. 잠복감염은 여러 가지 사이토카인 등 을 포함한 면역반응에 의해서 일어나며, 이 때 잠복감염된 세포는 특히 혈중의 T 세포라고 한다. 혈중 MDV 감염세포 는 피부 모낭상피세포, 선장 등 상피세포 유래의 조직에 MDV를 전파 시켜서 이들 조직에서 후기 세포용해성 감염이 일어나게 한다. 후기 세포용해성 감염을 유발하는 것은 이러한 MDV가 감염된 혈중 림프구라는 증거는 혈중의 세포 외에는 감염성 바이러스가 없기 때문이다. 후기 세포용해성 감염이 있는 후 육안적 혹은 현미경적으로 검출이 가능한 림프종이 여러 장기에서 관찰된다. MDV가 감염되면 병변 발생부위에 따라서 다양한 임상증상이 발생한다. 즉 내부장기에 병변이 있을 경우는 침울, 체중감소, 산란율 저하 등이 발생하며, 신경계는 발생부위별 신경증상이 일어난다. 이러한 장기나 조직에서는 육안적으로 백색에서 회백색의 종양성 병변이 관찰된다. 말초신경에 병변이 발생한 경우에는 특히 좌골신경 및 신경총에서 호발하는데, 이들 조직은 편측성 혹은 양측성으로 종창되어 있다. 안구는 간혹 육안적으로 식별이 가능한 홍채 퇴색 및 증식 병변이 관찰된다. 피부형 MD는 특히 육용계에서 문제가 되고 있으며, 산란계의 내장형 MD가 발생한 경우에도 피부를 자세히 설펴보면 피부형 MD를 간혹 관찰할 수 있다. 피부형 MD는 모낭주위에 원형의 결절형태로 발생하는데, 이들 병변이 커지면 바로 옆의 병변과 융합 되어 큰 결절을 형성하기도 한다. (중략)

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.24-30
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• 2017

Gorham-Stout disease is a rare disorder characterized by lymphovascular proliferation and destruction of osseous matrix. The etiology of this condition remains poorly understood. Chylothorax as a consequence of lymphatic leakage in thoracic cage may cause a severe life-threatening complication, accompanying respiratory difficulty. Currently, there is no standard management for this extremely rare condition. Here we describe a patient affected by Gorham-Stout disease successfully managed by the combined treatment of mTOR inhibitor and beta-blocker. A previously healthy 11-year-old female developed dyspnea and chest pain with a massive pleural effusion. The ligation of right thoracic duct and bilateral pleurodesis temporarily decreased her pleural effusion, which was aggravated repetitively and required frequent admission and tube thoracotomies. Along with bilateral pleural adhesiolysis with thoracotomy, the combined treatment of oral beta-blocker and mTOR inhibitor was commenced. After 1 month of oral medication, her pleural effusion was not increased and she was free of respiratory difficulty on room air without chest tubes. Over eleven months of treatment, no serious adverse reaction was noted and her condition has been stable with no further admission required.

• 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)

• Korean Journal of Clinical Laboratory Science
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• v.54 no.1
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• pp.68-72
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• 2022

Angioimmunoblastic T-cell lymphoma (AITL) is a lymphoproliferative disorder of mature T follicular helper cells. Atypical lymphoid cells were observed in the bone marrow of an 80-year-old woman, and the flow cytometric determined immunophenotypes of B-cells were unusual, that is, CD19+, CD20-, and CD22- with lambda light chain restriction. Initially, we suspected BM involvement of B-cell lymphoma based on the presence of abnormal B-cells. However, the patient was diagnosed with AITL involving BM. A re-analysis of flow cytometric immunophenotyping revealed a minor, aberrant T-cell population, and the lambda light chain restriction observed by surface staining was considered non-specific binding. This case demonstrates B-cells in patients with EBV-positive T-cell lymphoma may exhibit immunophenotypes resembling those of plasma cells, and that proliferation of abnormal B-cells or plasma cells could also potentially mask underlying T-cell lymphoma. A more integrated approach is required for accurate 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.1
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• pp.1-9
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• 2009

Purpose: Lymphonodular hyperplasia of the colon (LNHC) is a rare finding in children and its significance as a pathologic finding is unclear. The aim of this study was to investigate the clinical significance of LNHC by analyzing clinical and histopathologic findings in children with LNHC. Methods: We analyzed data from 38 patients who were confirmed to have LNHC by colonoscopy. We checked age, birth history, past history, family history, and clinical symptoms. A hematologic exam, stool exam, and image studies were performed and biopsy specimens were examined by a pathologist. All patients were asked to have short- and long-term follow-up. Results: The mean age of the patients was 12.5${\pm}$14.4 months. All patients presented with complaints of bloody stool. They appeared healthy and the hematologic findings were within a normal range, with the exception of one case. There was no other identified source of bleeding. On histologic exam, 36 patients (94.7%) had lymphoid follicles and 34 patients (84.5%) fulfilled the criteria of allergic colitis. Regardless of diet modification and presence of residual symptom, there was no recurrence of bloody stool through long-term follow-up in all patients. Conclusion: LNHC is more common in infants who are affected by allergic colitis, but it can appear even after infancy. LNHC should be regarded as the etiology when there are any other causes of rectal bleeding, especially in healthy children. We suggest that LNHC has a benign course regardless of diet modification and it might not require excessive concerns.

• The Journal of the Korean bone and joint tumor society
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• v.12 no.1
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• pp.78-82
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• 2006

Kimura's disease is uncommon and chronic inflammatory disease of unknown etiology. It manifests as solitary or multiple subcutaneous nodule, primarily located on the cervical area with local adenopathies and salivary gland hypertrophy. Its clinical course is benign nature. Histologic findings are hyperplastic lymphoid tissue, inflammatory infiltrates rich in eosinophils and proliferations of capillary venules. Treatment options are steroid therapy, radiation therapy and surgical excision. We report two cases of Kimura's disease on medial aspect of upper arm.