혈액종양 ; 신경섬유종증 환자에서 종격동과 복강 내 양측을 침범한 총상 신경섬유종 1예

안건희 ( Gun Hee An ),전병연 ( Byung Yeon Jun ),정미향 ( Mi Hyang Jung ),정희경 ( Hee Kyoung Jeong ),진종률 ( Jong Yul Jin ),이기현 ( Kee Hyun Lee ) 대한내과학회 2010 대한내과학회지 Vol.79 No.4

총상 신경섬유종(Plexiform neurofibromatosis)은 슈반세포(Schwann`s cell)에서 발생하며 조직학적으로 양성이고 비피 막성이며 경계가 불명확한 신경초 종양이다. 국내에서 종격동과 복강 내에서 동시에 발견된 신경섬유종증 환자에 대해 보고된 바 없어, 이에 저자들은 문헌고찰과 함께 본 증례를 보고하는 바이다. 환자는 신경섬유종증을 확진시키는 피부병변과 함께 흉부와 복부 CT에서 거대하고 균질한 융합성 종괴가 관찰되었고, 조직검사를 통해 종격동과 복강 내에 동시에 퍼져 있는 총상 신경섬유종임을 진단할 수 있었다. 본 증례에서는 특이증상이 없어 외래에서 추적관찰하기로 하였으나 향후 악성 종양의 가능성이 있으므로 주의깊은 추적관찰이 필요할 것이다. Plexiform neurofibroma is a benign proliferation that arises from the peripheral nerves and represents one of the diagnostic features of neurofibromatosis type I (NF-1). They are commonly found in the gastrointestinal tract, and intrathoracic neurofibroma is relatively uncommon. Ultimately, this tumor grows along the length of any single nerve and may involve multiple fascicles or branches of major nerves. Here, we report a case of multiple-site plexiform neurofibromas in a 40-year-old man previously diagnosed with NF-1. Although he had no perceptible symptoms, contrast-enhanced CT revealed massive diffuse neurofibromas that involved the mediastinum, periportal region, retroperitoneal space, and the mesentery. Histopathological studies of the neck, skin, and intra-abdominal soft tissue showed loose spindle cells and collagen bundles. The microscopic appearance was typical of a plexiform-type neurofibroma. It was decided that we would observe the patient without surgical management, because the neurofibromas were asymptomatic. (Korean J Med 79:443-447, 2010)

종양용해중후군에 의한 급성신부전증의 임상적 양상

박인석(In Seok Park),임규택(Gyu Taek Lim),김성수(Sung Soo Kim),장윤식(Yoon Sik Chang),윤영석(Young Suk Yoon),진종률(Jong Yul Jin),박종원(Jong Won Park),김춘추(Choon Choo Kim),방병기(Byung Kee Bang),김동집(Dong Jip Kim) 대한내과학회 1992 대한내과학회지 Vol.43 No.5

N/A Background: Tumor lysis syndrome (TLS) occurs as a result of the rapid release of intracellular ions and metabolites into the bloodstream which can overwhelm the reanl capacity to excrete and then increase to lifethreatening concentrations. The syndrome is characterized by hyperuricmia, hyperkalemia, hyperphos-phatemia and hypocalcemia. Recently, intensive chemotherapy for hematologic malignancy have introduced and tumor lysis syndorme occurred with increasing tendency. Methods: Renal and metabloic complications of tumor lysis of ten episodes are reviewed in nine patients with various hematologic malignancy during last 2 years. Results: The ten episodes of tumor lysis syndrome had developed in one of malignant lymphoma. five of acute lymphocytic leukemia and four of chronic myelocytic leukemia. The mean serum BUN and creatinine concentration was 68.0 and 4.4 mg/dl, respectively. The mean serum calcium was 5.2 mg/dl, phosphorus 11.5 mg/dl, uric acid 13.9mg/dl. Clinical manifestations of tumor lysis syndrome had developed abruptly within 24 hours after chemotherapy in all episodes. Prophylactic treatment including intravenous hydration, potent diuretics, alkalinization of urine and allopurinol were not always successful in preventing or aborting it. The two major clinical features of tumor lysis syndrome were oligoanuria (6/10) and neurologic symptoms such as carpopedal spasm (5/10). Conclusion: We had experienced ten episodes of tumor lysis syndrome from 9 patients. Except three patients who expired due to unrelated causes, the overall recovery was good. The mean recovery day in three conservative-treated patients and four hemodialysis- Bsekground: Tumor lysis syndrome (TLS) occurs as a result of the rapid release of intracellular ions and metabolites into the bloodstream which can overwhelm the reaol capacity to excrete and then increase to lifethreatening concentrations. The syndrome is characterized by hyperuricmia, hyperkalemia, hyperphos- phatemia and hypocalcemia. Recently, intensive chemotherapy for hematologic malignancy have introduced and tumor lysis syndorme occurred with increasing tendency. Methods: Renal and metabloic complications of tumor lysis of ten episodes are reviewed in nine patients with various hematologic malignancy during last 2 years. Results: The ten episodes of tumor lysis syndrome had developed in one of malignant lymphoma. five of acute lymphocytic leukemia and four of chronic myelocytic leukemia. The mean serum BUN and creatinine concentration was 68.0 and 4.4 mg/dl, respectively. The mean serum calcium was 5.2 mg/dl, phosphorus 11.5 mg/dl, uric acid 13.9mg/dl. Clinical manifestations of tumor lysis syndrome had developed abruptly within 24 hours after chemotherapy in all episodes. Prophylactic treatment including intravenous hydration, potent diuretics, alkalinization of urine and allopurinol were not always successful in preventing or aborting it. The two major clinical features of tumor lysis syndrome were oligoanuria (6/10) and neurologic symptoms such as carpopedal spasm (5/10). Conclusion: We had experienced ten episodes of tumor lysis syndrome from 9 patients. Except three patients who expired due to unrelated causes, the overall recovery was good. The mean recovery day in three conservative-treated patients and four hemodialysistreated patients was 10 and 16 days, respectively.

급성 골수성 백혈병에서 busulfan, thiotepa, 그리고 melphalan 3제 알킬화제제 전처치를 이용한 동종 골수이식

박수정(Soo Jeong Park),민우성(Woo Sung Min),김희제(Hee Je Kim),박은정(Eun Joung Park),엄기성(Ki Seong Eum),엄현석(Hyeon Seok Eum),민창기(Chang Ki Min),김동욱(Dong Wook Kim),이종욱(Jong Wook Lee),진종률(Jong Yul Jin),한치화(Chi Wha Ha 대한내과학회 2001 대한내과학회지 Vol.60 No.2

N/A Background : The purpose of this study was to evaluate the toxicity and efficacy of high-dose chemotherapy with busulfan, thiotepa and melphalan (BTM) as a myeloablative regimen in allogeneic bone marrow transplantation (allo-BMT) for patients with acute myelogenous leukemia (AML). Methods : Twenty-seven patients with AML were enrolled; Sixteen patients had standard risk (SR) diseases (first complete remission (CR1) and de novo AML) and eleven patients had high risk (HR) diseases (second, or subsequent remission, secondary AML, relapsed, or refractory AML, CR marrow with persisting extramedullary manifestation (chloroma), or hypoplastic acute leukemia). The conditioning regimen included busulfan 4 mg/kg/day for a total dose of 12 mg/kg; thiotepa 250 mg/m2/day for a total dose of 500 mg/m2; and melphalan 50 mg/m2/day for a total dose of 100 mg/m2. Cyclosporine A and short-course methotrexate were used for graft-versus-host disease (GVHD) prophylaxis. Results : The median time to recovery a granulocyte count of 0.5 x 109/L was 14 days (range 10∼25 days) and platelet transfusion independence was 30 days (range 12∼49 days). The major regimen-related toxicities were gastrointestinal-related symptoms including oral mucositis, nausea, vomiting, and diarrhea. All patients experienced oral mucositis (≥ grade 1) and the patients with oral mucositis of equal and greater than grade 3 were 44% in SR and 45% in HR. The toxicities associated with lung, skin, heart and brain were minimal. Three (11%) patients had severe or fatal veno-occlusive disease (VOD). There were five treatment-related death (19%) (hepatic VOD with multiorgan failure (n=3), pneumonia and ARDS (n=2)) within the first 100 days after allo-BMT. There was not a significant difference between SR and HR group (p=0.167). The incidence of acute GVHD equal or greater than grade II was less than 10%. The actual survival at 2 year was 70.4%(95% confidence interval (CI), 54.7%∼86.1%)(SR; 81.3% (95% CI; 63.4∼99.1%) vs HR; 54.6% (95% CI; 28.7∼80.4%), p=0.154). After a median follow-up of 630 days, 18 of 27 (67%, 355∼1062 days) patients are alive without evidence of disease. Three of the 27 patients relapsed (SR; 0% vs HR; 55.6% (95% CI; 19.6∼71.3%), p=0.004). Conclusion : The BTM regimen followed by allo-BMT is associated with acceptable toxicity and appears to have significant activity in patients with AML. It should be used with caution in patients with prior hepatopathy or refractory state who have an increased risk of severe VOD. Busulfan, thiotepa, and melphalan is an effective and alternative myeloablative regimen for patients with AML.(Korean J Med 60:156-166, 2001)

악성 임파종 환자에서 폐, 뇌 및 갑상선을 침습한 Aspergillus 감염 1예

김종률,정현,정일건,윤선애,진종률,백상홍,박준철,박석영,김현,이경진,지미경 대한내과학회 1993 대한내과학회지 Vol.44 No.1

저자들은 악성 임파종으로 부신 피질 호르몬과 세포독성 화학용법 치료중 폐, 뇌 및 갑상선에 침습성 Aspergillosis가 발생한 환자에서 Stereotaxic 뇌 생검 및 갑상선 흡입 생검으로 Aspergillus 감염증을 확진하고 Amphotericin B 치료에 좋은 반응을 보인 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. The invasive fungal infections are frequent in patients with neoplastic disease and result in a significant morbidity and mortality. In the last decade, the progress in antineoplastic chemotherapy and the more aggressive approach in the treatment of cancer patients gave rise to increase the number of patients predisposed to invasive fungal infections. With a brief reveiw of literature, we report one case of invasive aspergillosis involving lung, brain and thyroid gland, which was diagnosed by stereotaxic aspiration of brain and needle aspiration of thyroid gland in 21-year-old male patient who had taken the chemotherapeutic agents for the treatment of relapsed malignant lymphoma.

발작성 야간 혈색소뇨증이 동반된 골수이형성 증후군 1례 : 망상적혈구 생존 검사와 Glycosylphosphatidylinositol(GPI) 연관 단백 검사를 이용한 증명

임지향,김용구,김동욱,진종률,한치화,한경자,심상인,김춘추 大韓血液學會 1997 大韓血液學會誌 Vol.32 No.3