• Title/Summary/Keyword: 조직구증식증

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Surgical Treatment of Langerhans Cell Histiocytosis in the Rib - Two cases report - (늑골에 생긴 랑거한스세포 조직구증식증의 외과적 치료-치험 2예-)

  • Kang Do-kyun;Park Jae-min;Han Il-Yong;Jun Hee-jae;Yoon Young-chul;Hwang Yun-ho;Cho Kwang-hyun;Lee Yang-haeng
    • Journal of Chest Surgery
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    • v.38 no.11 s.256
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    • pp.799-802
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    • 2005
  • Langerhans cell histiocytosis (LCH) involves disorders previously referred as 'histiocytosis X' (including eosinophilic granuloma, Letterer-Siwe, and Hand-Schuller syndrome). Its clinical patterns are various and it is a basically benign tumoral condition but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. But, in solitary bone lesion, wide resection with tumor-free margin is required in order to provide the best chance for a cure. In the majority of patients LCH is a osteolytic lesion with a predilection for calvarium and is rarely seen in the skull base and the femur. LCM of rib, especially if solitary, is relatively rare. We report two rare cases of solitary LCH developed in the rib, which were successfully treated by surgical resection.

Fatal Hemophagocytic Lymphohistiocytosis associated with Influenza B (B형 인플루엔자와 관련되어 발생한 혈구포식 림프조직구증식증)

  • Lee, Saem Na;Yoon, Jin Gu;Cho, Chi Hyun;Choi, Chul Won;Choi, Jung Yoon;Cheong, Hee Jin;Kim, Woo Joo
    • The Korean Journal of Medicine
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    • v.91 no.1
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    • pp.88-91
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    • 2016
  • Influenza infection may be complicated by various infectious or non-infectious diseases. Among them, hemophagocytic lymphohistiocytosis (HLH) is an uncommon hyperinflammatory syndrome caused by uncontrolled proliferation and activation of macrophages and lymphocytes, and it is often life threatening. A previously healthy male patient was suspected to have HLH after influenza B infection. The diagnosis was established based on clinical diagnostic criteria suggested in the HLH-2004 trial. Despite prompt antiviral therapy, the patient expired on day 19 of hospitalization. Influenza can thus be complicated by HLH. Due to the non-specific manifestations of HLH, clinical suspicion and early diagnosis are important.

Clinical Characteristics of Pulmonary Histiocytosis X (폐조직구증식증의 임상적 특징)

  • Hwang, Yong-Ii;Park, Gun-Min;Yim, Jae-Joon;Yoo, Chul-Gyu;Lee, Choon-Taek;Kim, Young-Whan;Han, Sung-Koo;Shim, Young-Soo
    • Tuberculosis and Respiratory Diseases
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    • v.51 no.4
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    • pp.346-353
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    • 2001
  • Background : Pulmonary Langerhans cell histiocytosis forms part of a spectrum of diseases that are characterized by a monoclonal proliferation and infiltration of organs by Langerhans cells. Several organ systems may be involved in Langerhans cell histiocytosis, including the lungs, bone, skin, pituitary gland, liver, lymph nodes and thyroid. Pulmonary histiocytosis X represents 2.8% of interstitial lung disease. Here we present the clinical, radiological, therapeutic aspects of pulmonary histiocytosis X. Method : Fourteen cases of biopsy-proven pulmonary histiocytosis X patients who were diagnosed in Seoul National University Hospital during the period from January 1990 to December 1998 were analyzed retrospectively. Result : There were 12 men and 2 women in this study. The initial presenting symptoms were dyspnea, cough, chest pain, which was associated with the pneumothorax, and chest radiography abnormalities. Only 8 patients (57%) were smokers. There were 5 patients with extra-pulmonary histiocytosis (pituitary, bone, skin). Eight patients had received the chemotherapy. There were no mortalities and only one patient experienced an aggravation of symptom during the follow-up period. Conclusion : In contrast to previous reports from other countries, the patients with pulmonary histiocytosis X in this study presented with several different clinical characteristics, such as a male predominance, relatively low smoker's rate, and a better prognosis.

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Epstein-Barr Virus in Nasal Angiocentric Lymphoma with Malignant Histiocytosis-like Hemophagocytic Syndrome (악성조직구증과 유사한 혈구탐식증후군을 동반한 코의 혈관중심위 림프종과 Epstein-Barr 바이러스의 관련성 연구)

  • Han Ji-Youn;Kim Hoon-Kyo;Moon Han-Lim;Seo Eun-Joo;Kwon Hi-Jeong;Park Yeon-Joon;Min Ki-Ouk;Yoon Sei-Cheol;Kim Min-Shik;Cho Seong-Ho;Kim Byung-Kee;Lee Kyung-Shik;Kim Dong-Jip
    • Korean Journal of Head & Neck Oncology
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    • v.13 no.1
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    • pp.9-15
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    • 1997
  • Malignant histiocytosis(MH)-like hemophagocytic syndrome(HS) is a fatal complication of nasal angiocentric lymphoma(AL) and difficult to distinguish from MH. Ten of total 42 patients with nasal AL had HS and 9 of them were initially suspected to have MH. Five patients had HS as initial manifestation, 3 at the time of relapse, and 2 during the clinical remission of lymphoma. Four patients were treated by combination chemotherapy(CHOP) and others had only supportive care. Immunohistochemical study and in situ hybridization were performed on the specimen obtained from 10 patients. The median survival of all patients from HS was 18 days(range 2 - 44 days) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1(CD45RO) and Epstein-Barr virus (EBV) by EBER in situ hybridization. MH-like HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in future.

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Diagnostic effectiveness of fine needle aspiration cytology on pediatric cervical lymphadenopathy (소아 경부 림프절 종대의 세침 흡인 세포검사의 진단적 유용성)

  • Byun, Jun Chul;Choe, Byung Kyu;Hwang, Jin-Bok;Kim, Heung Sik;Lee, Sang Sook
    • Clinical and Experimental Pediatrics
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    • v.49 no.2
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    • pp.162-166
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    • 2006
  • Purpose : There are few reports in Korean literature on the diagnostic efficacy of fine needle aspiration cytology(FNAC) for pediatric cervical lymphadenopathy. This study examined the diagnostic value of FNAC on cervical lymphadenopathy in children. Methods : Data from 57 pediatric patients(aged 0.3 to 14 years) who underwent FNAC due to cervical lymphadenopathy between January 2001 and March 2005 was reviewed retrospectively. Results : Reactive cervical lymphadenitis was the most common result of the FNAC(52.5 percent). Malignant disease were revealed in 14 percent of all cases. The sensitivity, specificity was 86 percent and 96 percent, respectively. The positive and negative predictive values for malignant disease was 75 percent and 98 percent, respectively. There were two false-positive cases on FNAC; one case of Langerhans cell histiocytosis, and another with infectious mononucleosis. A false-negative case on FNAC was found to be acute lymphocytic leukemia. In seven cases(12 percent), the final diagnosis was confirmed by an open biopsy after the FNAC. There were four FNAC cases where the specimen was not satisfactory for making a diagnosis(7 percent). There were no serious complications of the FNAC procedure. Conclusion : FNAC had a high diagnostic efficacy for evaluating children with cervical lymphadenopathy.

The Differentiation of Malignant and Benign Musculoskeletal Tumors by F-18 FDG PET/CT Studies - Determination of maxSUV by Analysis of ROC Curve (F-18 FDG PET/CT에서 양성과 악성 근골격 종양의 감별진단 - 수신자 판단특성곡선을 이용한 maxSUV의 절단값 결정)

  • Kong, Eun-Jung;Cho, Ihn-Ho;Chun, Kyung-Ah;Won, Kyu-Chang;Lee, Hyung-Woo;Choi, Jun-Heok;Shin, Duk-Seop
    • Nuclear Medicine and Molecular Imaging
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    • v.41 no.6
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    • pp.553-560
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    • 2007
  • Purpose: We evaluated the standard uptake value (SUV) of F-18 FDG at PET/CT for differentiation of benign from malignant tumor in primary musculoskeletal tumors. Materials and Methods: Forty-six tumors (11 benign and 12 malignant soft tissue tumors, 9 benign and 14 malignant bone tumors) were examined with F-18 FDG PET/CT (Discovery ST, GE) prior to tissue diagnosis. The maxSUV(maximum value of SUV) were calculated and compared between benign and malignant lesions. The lesion analysis was based on the transverse whole body image. The maxSUV with cutoff of 4.1 was used in distinguishing benign from malignant soft tissue tumor and 3.05 was used in bone tumor by ROC curve. Results: There was a statistically significant difference in maxSUV between benign (n=11; maxSUV $3.4{\pm}3.2$) and malignant (n=12; maxSUV $14.8{\pm}12.2$) lesions in soft tissue tumor (p=0.001). Between benign bone tumor (n=9; maxSUV $5.4{\pm}4.0$) and malignant bone tumor (n=14; maxSUV $7.3{\pm}3.2$), there was not a significant difference in maxSUV. The sensitivity and specificity for differentiating malignant from benign soft tissue tumor was 83% and 91%, respectively. There were four false positive malignant bone tumor cases to include fibrous dysplasia, Langerhans-cell histiocytosis (n=2) and osteoid osteoma. Also, one false positive case of malignant soft tissue tumor was nodular fasciitis. Conclusion: The maxSUV was useful for differentiation of benign from malignant lesion in primary soft tissue tumors. In bone tumor, the low maxSUV correlated well with benign lesions but high maxSUV did not always mean malignancy.

Nutritional Assessment and Dietary Management during Anti-Cancer Chemotherapy in Pediatric Oncology Patients (항암 화학요법 중인 소아암 환자의 영양 평가 및 식이 관리)

  • Jang, Se-Ri;Kim, Hee-Sun;Lee, Young-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.2
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    • pp.204-210
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    • 1999
  • Purpose: We assessed the nutritional status and the alterations of oral diets during anti-cancer chemotherapy in pediatric oncology patients. Methods: Twenty children with malignancy were evaluated from day 0 until day 21 of post-chemotherapy. Nutritional status was assessed by body weight and biochemical parameters. The amount and calories of oral diets were assessed and food preference before and during chemotherapy were analysed by questionnelle. Results: 1) The underlying diseases of 20 patients were 11 acute lymphoblastic lekemia, 2 non-Hodgkin's lymphoma, 2 Langerhans cell histiocytosis, 2 Wilm's tumor, 2 brain tumor, 1 rhabdomyosarcoma. 2) There were weight loss during chemotherapy in 8 patients (40.0%), weigt gain in 5 patients (25.0%), and no significant changes in 7 patients (35.0%). 3) Biochemical parameters showed no significant interval changes during chemotherapy except elevation of serum ALT level. 4) The daily caloric intakes of oral diets during chemotherapy were 310~600 Kcal which was much lower than average of daily recommended calory for Korean children. 5) The most favorate food was altered by chemotherapy, from meats to carbonated beverages and unfavorate food was not altered as vegetables. Conclusion: The periodic assessment of nutritional status and dietary supplements according to preferred foods of patients will be required for the optimal nutrition care in cancer patients.

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