• 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)

• Tuberculosis and Respiratory Diseases
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• v.47 no.1
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• pp.13-25
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• 1999

Background: Ineffective cell-mediated immune response in human tuberculosis is associated with a depressed Thl cytokine response and reduced production of IFN-$\gamma$. Most persons infected with Mycobacterium tuberculosis are healthy tuberculin reactors with protective immunity, but a minority with ineffective immunity develop extensive pulmonary tuberculosis. The cell-mediated immune response is an important aspect of host resistance to mycobacterial infection and is believed to be tightly regulated by a balance between Th1 cytokines including IFN-$\gamma$, IL-12, IL-18, regulated on activation, normal T cell expressed and secreted (RANTES) and Th2 counterparts such as IL-4, monocyte chemoattractant protein-l (MCP-l). Methods: Proliferation and mRNA expression of IFN-$\gamma$, RANTES and MCP-l by RT-PCR in peripheral blood mononuclear cells (PBMCs) in response to in vitro stimulation with mycobacterial antigens were compared in pulmonary tuberculosis patients with cured and treatment failure and in tuberculin-positive and tuberculin-negative healthy subjects. Results: Defective proliferative responsiveness to aqueous TSP antigen was involved with treatment failure tuberculosis patients. Aqueous TSP antigen-induced IFN-$\gamma$ and RANTES mRNA expression was decreased in treatment failure tuberculosis patients compared with healthy tuberculin reactors and cured tuberculosis patients (23.1 % versus 90.0% for IFN-$\gamma$ and 46.2% versus 70.0% versus 46.2% for RANTES). The frequency of MCP-l mRNA expression to aqueous TSP antigen in treatment failure tuberculosis patients was greater than in healthy tuberculin reactors and cured tuberculosis patients (76.9% versus 40.0%). Conclusion: The increasing expression of MCP-1 mRNA in response to aqueous TSP antigen might be predicted to favor Th1 responses and restricted Th1 responses in treatment failure of pulmonary tuberculosis.

• 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.

• Journal of Life Science
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• v.21 no.4
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• pp.542-548
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• 2011

Food-dependent exercise-induced anaphylaxis (FDEIA) is a distinct form of food allergy induced by physical exercise. It is typified by the onset of anaphylaxis during exercise, which is preceded by the ingestion of causal food allergens. Diagnosis of FDEIA is heavily dependent on clinical history. To describe the physiopathological mechanism, etiologic factors, and clinical manifestations, we evaluated the spleen index, proliferation assay of lymphocyte, ROS, ASAS, and cytokines levels in sensitized and exercise-trained mice. One-hundred mice were bred in the animal lab at D and P university under controlled conditions [$22{\pm}2^{\circ}C$, RH 45-55%, and a 12-hour photoperiod]. Animals are 7-weeks-old at the time of study and were fed a standard commercial chow diet from 09:00 to 15:00 over the 8-week study period. The mice were allowed access to distilled deionized water ad libitum. Daily food intake and weekly body gains were routinely recorded throughout the experimental period using computing scale (CAS). Mice were divided into the control group (S; control sensitized, n=25), 30 min swim training group (S30, N=25), 50 min swim training group (S50, N=25), and 80 min swim training group (S80, N=25). The results were as follows: Spleen index showed the highest level in the S80 group compared to other groups; this level was exercise-dependent. In proliferation assay of Med and OVA, the S80 group showed the highest level compared to the other groups; this level also was exercise intensity- dependent. Peritoneal ROS and IL-4 showed a statistically significant difference compared to S; however, there was no significant differences in ROS among S30, 50, and 80. From the results, we concluded that FDEIA is correlated with exercise intensity based on the levels of peritoneal ROS and cytokine profiles.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.32-35
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• 2014

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder of lymph node and extranodal site. Bone involvement is very rare. We report a case of extranodal RDD of the tibia in 32-year old male. The patient presented with pain with no evidence of lymphadenopathy. Clinico-radiologic diagnosis was metastatic carcinoma or Langerhans cell histiocytosis, but, histopathologic examination confirmed the diagnosis with RDD. We performed curettage on the osteolytic lesion of tibia. In South Korea, there was no report about RDD of the extremity and we want to report this case with review of the literature.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1007-1015
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• 2002

Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes were classified into a few, some, and many according to the number of histiocytes comparing with the white cells. Results : The age of thirty patients ranged from two months to 15 years. The median age was 5.6 years with male predominance(2.3 : 1). The most frequent clinical manifestation was fever; others were respiratory symptom, hepatosplenomegaly, lymphadenopathy and skin rash(in order of frequency). Common laboratory findings were leukopenia, thrombocytopenia, anemia and abnormal liver function test. Infection was present in 30 patients; causative organisms were documented in 15 patients, and bacterial infection was more common. Epstein Barr virus was the cause of infection in four patients. Bone marrow examination showed a few(20.0%), some(75.0%) and many(30.0%) hemophagocytic histiocytes. Combination immunochemotherapy including immunoglobulin, steroids and cyclosporine were helpful in 22 out of 30 patients. The mortality rate was high in young patients who showed some to many hemophagocytic histiocytes. Conclusion : Bone marrow examinations and early detection of histiocytes will be helpful in children who have fever, hepatosplenomegaly and abnormal liver function test. Immunochemotherapy were helpful and further investigation will be needed for the detection of the relationship between the bone marrow findings and prognosis.

• 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.

• 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.

• Childhood Kidney Diseases
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• v.7 no.2
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• pp.245-252
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• 2003

Posttransplant lymphoproliferative disease(PTLD) has emerged as a potential life-threatening complication of immunosuppressive therapy after organ transplantation. The occurrence of PTLD is usually associated with an Epstein-Barr virus(EBV) infection in patients who are treated by aggressive immunosuppressive therapy. PTLD is represented by diverse manifestations ranging from reactive lymphoid hyperplasia to high grade malignant lymphoma. This is a case report of a late PTLD in a child. The patient is a 14-year-old girl, who presented as malignant lymphoma 44 months after successful renal transplantation. There was no evidence of EBV infection. On bone marrow study, many neoplastic lymphoid cells were defected. Aggressive chemotherapy for PTLD had resulted in clinical remission. However the patient expired from uncontrolled sepsis and septic shock after 77 days.