비소세포 폐암과 동반된 부수종양성 변연계뇌염

신현종 ( Hyun Jong Shin ),김현수 ( Hyun Soo Kim ),임금남 ( Keum Nam Lim ),노유석 ( U Seok Noh ),최정혜 ( Jung Hye Choi ),김인순 ( In Soon Kim ),이영열 ( Young Yeul Lee ),박병배 ( Byeong Bae Park ),박동우 ( Dong Woo Park ) 대한결핵 및 호흡기학회 2007 Tuberculosis and Respiratory Diseases Vol.63 No.4

Paraneoplastic limbic encephalitis is a rare disorder that is characterized by personality changes, irritability, depression, seizures, memory loss and dementia, and is commonly associated with small cell lung cancer. The cause is unknown but it is believed to be an autoimmune disorder that develops secondary to a carcinomatous process. We report a patient with the clinical feature consistent with limbic encephalitis. A 64-year-old women developed disorientation, memory loss and general weakness. She was diagnosed with NSCLC (adenocarcinoma) with a brain metastasis 1 year earlier and was treated with radiation and chemotherapy. Although the lung mass and brain metastatic lesions had improved, the brain T2-weighted MRI showed high signal intensity in the right temporal region. This lesion consisted of with limbic encephalitis and was negative to the other viral and immune markers. The patient`s symptoms did not improve after steroid treatment. Our case demonstrated that a NSCLC (adenocarcinoma) also can be associated with paraneoplastic limbic encephalitis. (Tuberc Respir Dis 2007; 63: 382-386)

치료 불응성 Still's병에서 자가 조혈모세포 채집시 사용된 G-CSF에 의한 급성 악화 1예

이재웅,박성현,왕준광,오호석,최정혜,배상철,이영열,김인순,최일영,안명주 대한조혈모세포이식학회 2003 대한조혈모세포이식학회지 Vol.8 No.2

치료 불응성 자가면역질환에서 고용량 항암화학요법 및 자가 조혈모세포이식술이 시행되고 있으나 조혈모세포 가동화 및 이식술 시 사용되는 G-CSF에 의해 오히려 기존의 자가면역질환의 증세가 악화되었다는 보고가 있다. 저자들은 만성적으로 재발되는 Still's병 환자에서 조혈모세포 가동화를 위해 사용한 G-CSF에 의해 자가면역질환이 급성 악화된 1예를 경험하였기에 보고하는 바이다. High-dose immunosuppressive therapy with autologous stem cell transplantation is an increasingly used treatment for severe refractory autoimmune disorder including multiple sclerosis, rheumatoid arthritis, juvenile chronic arthritis, systemic lupus erythematosus. Although the optimal method of collecting stem cell is not determined, G-CSF-based mobilization is generally considered safe. However, worsening of disease status was reported in autoimmune disease undergoing mobilization with G-CSF. We report a 24-year-old male with refractory Still's disease who developed acute disease flare after administration of G-CSF for stem cell mobilization.

하시모토 병과 동반된 악성 빈혈 1 예

한 호,안명주,최정혜,이창범,이영열,최일영,김인순,이웅수 한양대학교 의과대학 2001 한양의대 학술지 Vol.21 No.2

Pernicious anemia is the most common cause of vitamin B12 deficiency and is caused by autoimmune destruction of parietal cell containing gastric mucosa region. This disease is significantly associated with autoimmune endocrinopathies and anti-receptor autoimmune disease. We report here a case of 26-year old man who was diagnosed pernicious anemia with high level of autoantibody titer (antithyroglobulin antibody 1:25,600 and antimicromosomal antibody 1:6,400) indicating subclinical Hashimoto's thyroiditis. Since other autoimmune diseases are frequently associated with pernicious anemia even without evidence of clinical symptoms, screening test for the presence of autoimmune disease should be evaluated in these patients.

관해유도 항암화학요법에 실패한 호지킨병 환자에서 2회의 자가 말초혈액 조혈모세포이식 후 지연되어 완전 관해된 1예

정성진,김일,박환철,오호석,최정혜,이영열,김인순,최일영,안명주 대한조혈모세포이식학회 2003 대한조혈모세포이식학회지 Vol.8 No.1

관해유도 항암화학요법에 실패한 호지킨병 환자에서 2회의 연속된 자가 조혈모세포이식은 하나의 치료 대안으로서 고려될 수 있다. 저자들은 진행된 호지킨병으로 진단된 뒤 기존 항암화학요법에 저항을 보여 자가 말초혈액 조혈모세포이식을 시행하였으나 여전히 부분 관해를 보였고, 재차 자가 말초혈액 조혈모세포이식을 시행하였으나 영상학적 검사에서 부분 관해에 머물렀던 환자가 조혈모세포 이식 3년 후 추적 검사에서는 완전 관해 소견을 보인 1예를 경험하여 이를 보고하는 바이다. The double autologous stem cell transplantation after high-dose therapy has been considered as a therapeutic chance for patients with refractory or relapsing Hodgkin's disease. We report here a 29-year-old patient with Hodgkin's disease (nodular sclerosis, Stage IVB), who was partially responsive to 6 cycles of conventional chemotherapy, achieved only partial remission despite double autologous peripheral blood stem cell transplantation. However, long- term follow-up with 3 years after second stem cell transplantation, the persistently remained tumors on spleen, left ilium, and right scapular area disappeared spontaneously and now the patient has no evidence of disease. Compared with previously reported cases of long-lasting persistent evidences of unresolved disease after allogeneic bone marrow transplantation with subsequent complete response in other hematologic malignancies, an atypical pattern in this case with delayed complete response after autologous hematopoietic stem cell transplantation has not been reported.

악성 질환에서의 고용량 화학요법과 자가말초혈액 조혈모세포이식술 : 단일기관 치료 경험

최정혜,안명주,오호석,이웅수,오석중,이영열,최일영,김인순 대한조혈모세포이식학회 2003 대한조혈모세포이식학회지 Vol.8 No.2

연구배경: 고용량 화학요법은 항암제의 투여 용량에 따라 항암효과가 비례하는 용량반응 관계가 있는 악성종양에서 효과를 기대할 수 있으며 현재 급성백혈병, 악성림프종, 다발성골수종 등의 혈액 종양과 유방암, 고환암, 난소암, 신경아세포종 등의 고형암 환자에서 시행되고 있다. 저자들은 고용량화학요법에 이어 자가조혈모세포이식을 시행 받은 악성 종양 환자를 대상으로 치료효과 및 부작용 등의 임상상을 알아보고자 하였다. 방법: 1997년부터 2003년까지 한양대학교 병원에서 자가조혈모세포이식을 시행 받은 악성 종양 환자 31예(남서 : 여성 15 : 16, 중앙연령: 50세, 다발성골수종 14예, 비호지킨림프종 9예, 유방암 4예, 호지킨림프종 3예, 급성골수성백혈병 1예)를 대상으로 후향적 분석을 시행하였다. 결과: 채집된 단핵구의 중앙값은 5.1×10^(8)/kg (범위 1.6~189.3)이었고 CD34양성세포수의 중앙값은 12.0×10^(6)/kg (범위 1.3~75.1)이었다. 말초혈액 과립구가 500/μL 에 도달하는 중앙값은 11일(범위 8~27)이었고 혈소판이 20,000/μL에 도달하는 중앙값은 11일(범위 0~32)이었다. 고용량 항암화학요법 후 21예가 완전관해, 5예가 부분관해에 도달하였다. 생존한 환자의 중앙추적기간은 29.5개월이었고 전체 환자의 2년 무병생존기간은 55.7%이었고 중앙값은 48.2개월이었다. 2년 생존기간은 62.9%이었으며 중앙값에는 도달하지 않았다. 10예의 사망환자 중 7예가 질병의 진행으로 사망하였고, 조기사망 3예(심부전과 신부전 1예, 장파열 1예, 패혈증 1예)가 있었다. 결론: 이 연구는 포함된 환자의 수가 적고 추적기간이 짧은 단점이 있으나, 다발성골수종, 악성림프종 등의 악성 질환에서 고용량 항암화학요법과 자가조혈모세포이식은 안전하고 효과적인 치료임을 추정할 수 있었다. Background: We performed the current study to evaluate the safety and efficacy of high-dose chemotherapy (HDC) with autologous peripheral blood stem cell transplantation (ASCT) in malignant diseases. Methods: Between January 1997 and March 2003, 31 patients (14 multiple myeloma, 9 Non-Hodgkin's lymphoma, 3 Hodgkin's disease, 4 breast cancer, and 1 acute myelogenous leukemia) underwent HDC with ASCT. Stem cells were collected by CS-3000 after mobilization treatment with chemotherapy and granulocyte colony-stimulating factor. The conditioning regimens included high-dose melphalan, BEAM (BCNU, etoposide, cytosine arabinoside, melphalan), BEAC (BCNU, etoposide, cytosine arabinoside, cyclophosphamide), CPB (cyclophosphamide, cisplatin, BCNU) and others. Results: There were 15 male and 16 female patients and median age was 50 years (range: 29~77). The median number of mononuclear and CD34+ cells was 5.1×10^(8)/kg (1.6~189.3) and 12.0×10^(6)/kg (1.3~75.1), respectively. The median time of neutrophil (>500/μL) and platelet (>20,000/μL) engraftment was 11 (8~27) and 11 (0~32) days after ASCT, respectively. Twenty-two patients experienced neutropenic fever. Twenty-one patients achieved complete remission and 5 patients achieved partial remission after HDC with ASCT. The median follow-up duration for surviving patients was 29.5 months. Causes of death included disease progression (7), sepsis (1), bowel perforation (1), and renal failure (1). Median progression free survival (PFS) was 48.2 months and median overall survival (OS) was not reached. Two-year PFS and OS was 55.7% and 62.9%, respectively. Conclusion: Our results suggest that HDC with ASCT was safe and effective modality in patients with various malignant diseases.

제 2형 von Willebrand 병 1예

조윤주,안명주,이영열,최일영,김인순,이웅수 대한내과학회 1997 대한내과학회지 Vol.53 No.3S

저자 등은 내원3주전 맹장염 수술후 과다 출혈이 있어 재수술을 요하였던 21세 남자에서 Ristocetin cofactor activity, RIPA 그리고 혈장의 vWF multimer 분석으로 제 2A형 vWD로 진단하여 가족 검사 및 문헌 고찰과 함께 보고하는 바이다. Von Willebrand's disease is now recognized as the commonest inherited bleeding disorder of humans. Bleeding is caused either by a decrease in the concentration of a normal von Willebrand factor(type 1 and 3) or by the presence in the circulation of a functionally abnormal von Willebrand factor(type 2). Patients with mild manifestations are often difficult to diagnose by standard laboratory criteria, and it is likely that many cases go undiagnosed. A 21 year old male patient was referred to us with a question of a bleeding disorder. He underwent an appendectomy after which he bled excessively from the wound requiring second operation at another hospital. He had a history of frequent epistaxis during childhood. Laboratory data showed a normal platelet count, normal prothrombin time and normal activated partial thromboplastin time. Factor Ⅷ activity was 46% and von Willebrand factor antigen was 43%. Ristocetin cofactor activity was markedly decreased to 10%. Ristocetin induced platelet aggregation showed no aggregation response at Ristocetin concentration of 1.0㎎/dL and 1.2㎎/dL, while control showed normal aggregation response. Plasma vWF multimeric analysis showed absence of intermediate and large sized multimers. Therefore, we recognize this patient as type 2A von Willebrand disease. Family study showed a similar defect in the patient.'s father and brother.

필라델피아 염색체 양성을 보인 급성 골수성 백혈병 1예

안명주,김동선,이재호,이영열,김인순,최일영,조율회,백용균,정낙은,한규섭 大韓血液學會 1987 大韓血液學會誌 Vol.22 No.2