Treatment of Multiple Pulmonary Arteriovenous Fistulas with Therapeutic Embolization in Osler-Rendu-Weber Syndrome / 결핵

Hereditary hemorrhagic telangiectasia(Osler-Rendu-Weber Syndrome) is characterized by telangiectasia of the skin and mucous membranes and intermittent bleeding from vascular abnormalities. About 20% of patients with this is syndrome have pulmonary arteriovenous fistulas. Pulmonary arteriovenous fistula is uncommon malformation which has an abnormal connection between the pulmonary capillary bed, in which venous blind in the pulmonary artery is shunted through the fistula into the pulmonary vein without exposure to alveolar oxygen and result in unoxygenated, desaturated systemic arterial blood, polycythemia, cyanosis and clubbing. Death often results from cerebral abscess and rupture of the malformation with massive hemorrhage. Therapeutic intervention is recommended for all symptomatic patients because of the risk of those serious complications. Treatment options include surgery and transcatheter obliteration with steel coils or detachable balloons. Therapeutic embolization has the advantages that multiple bilateral pulmonary arteriovenous fistulas can be occluded and also that the procedure can be repeated if necessary. Recently we experienced a case of the multiple bilateral pulmonary arteriovenous fistulas associated with telangiectatic change of hepatic artery and multiple angiodysplasia on the gastric mucosa in 41 years old female patient who had mild dyspnea of exertion(NYHA class II), clubbing finger, severe iron deficiency anemia. She was treated with embolization technique using steel coils and iron replacement. After the therapeutic embolization, significant improvement of dyspnea of exertion with disappearance of multiple pulmonary nodule on follow-up simple chest x-ray was noted. During the subsequent six months follow-up period, she bad the improvement of symptoms arid iron deficiency anemia.

Prognostic Factor Analysis of Small Cell Lung Cancer: Appropriateness of Two Staging System / 대한암학회지

PURPOSE: The two staging system, which divides the tumors into limited disease (LD) and extensive disease (ED) has been widely accepted as a major prognostic determinant in small cell lung cancer (SCLC). However this system has provoked several controversial issues in defining stage categories, for instance, ipsilateral pleural effusion as LD or ED. Furthermore, identification of favorable subgroups in the same stage has been recognized as an important factor to determine appropriate treatment strategies. In this study, we performed a retrospective analysis in an attempt to resolve the controversial issues about staging and identify the patient group with favorable prognosis based on this two staging system. MATERIALS AND METHODS: The clinical data of 233 patients with SCLC treated from 1990 to 1996 at Korea Cancer Center Hospital were retrospectively analyzed for this study. All patients were treated with chemotherapy containing cisplatin and/or radiotherapy. The independent prognostic factors for survival were identified by multivariate analysis using Cox's proportional hazards model. RESULTS: Performance status (relative risk of death [RR]:2.89), number of metastasis (RR:2.2), response to treatment (RR:2.2) as well as stage (RR:1.77) were identified as independent prognostic factors for survival in patient with SCLC. The median survival of patients with ipsilateral pleural effusion (13 months) which was categorized as ED was similar to that of patients with contralateral mediastinal or supraclavicular lymph nodes (13.8 months) or other LD patients (13.7 months). This result suggests that ipsilateral pleural effusion should be categorized as LD. In LD, response to treatment was the only independent prognostic factor (RR:2.34) and thoracic radiotherapy moderately improved survival as compared with combination chemotherapy alone (17.7 months vs. 10.4 months, p=0.06). In ED, the patient group with a good performance status (ECOG 0-1), normal range of serum alkaline phophatase, and metastasis less than 2 sites showed significantly prolonged survival, comparing with other ED patients (11.2 months vs. 7.2 months, p=0.0001). CONCLUSION: As a result of survival analysis, we confirmed independent prognostic factors such as stage and performance status in SCLC. We could recommend that LD category include patients with ipsilateral pleural effusion as well as those with contralateral lymphadenopathy. In ED, the survival in patients with favorable prognostic factors was comparable to LD, suggesting this patient group may be a candidate for aggressive therapy.

Analysis of Prognostic Factors and Application of International Prognostic Index Model to Determine the High Risk Group for the Treatment of Aggressive Non - Hodgkin's Lymphoma / 대한내과학회지

OBJECTIVE: Although the therapeutic outcome of aggressive non-Hodgkin's lymphoma (NHL) has been considerably improved by the introduction of combination chemotherapy, many patients still fail to achieve complete response(CR) and/or long-term survival. Because the outcome appears to depend on certain prognostic factors, long term prognosis can be predicted by identification of risk group. And also, the patients in high risk group may benefit from new therapeutic modality. In 1993, the international prognostic index model for aggressive NHL as developed far the purpose of predicting outcome and designing of therapeutic trial. Thus, analysis of prognostic factors was performed to identify independent factors for the end points of CR, overall survival, and disease-free survival. METHODS: From 1989 to 1994, total 340 patients were treated with combination chemotherapy and/or radiotherapy for NHL in Korea Cancer Center Hospital. Among 340, informations on eleven prognostic factors(sex, age, performance status, Ann Arbor stage, serum LDH level, tumor size, number of extranodal disease sites, bone marrow involvement, presence of B symptom, sex, time to CR, and histologic grade) were avaliable for 273 patients. Among these, 221 patients with aggressive NHL(NCI clinical schema) were eligible for the prognostic factor analysis for the response and survival. Also, 186 patients were eligible to determine whether International Prognostic Index Model could be applicable for Korean NHL. RESULTS: One hundred fifty patients(68%, 95% CI 62-74%) achieved a complete remission, 43 patients (20%) a partial remission. With a median follow-up of 3,5 years, overall 3 year survival rate was 6396, and 3 year DFS for the 150 CRs was 72%. In a univariate analysis for the CR and survival, Ann Arbor stage, number of extranadal disease, performance status, presence of B symptoms, presence of BM involvement, serum LDH level and histologic grade were found to be statistically significant prognostic factors. Among them, by multivariate analysis, number of extranodal disease(RR 0.2, 95% CI 0.1-0.7), B Symptoms (RR 0.4, 95% CI 0.2-0.9), and histologic grade(RR 0.2, 95% CI 0.08-0.7) showed to be independent adverse prognostic factors for CR. For disease-free survival, Ann Arbor stage(RR 2.6, 95% CI 1.1-6.4) was independent risk factor. For overall survival, number of extranodal involvement(RR 2, 95% CI 1.3-4) and histologic grade(RR 2, 95% CI 1.2-3.7) were independently significant prognostic factors. With these 2 independent prognostic factors for survival, we could establish a prognastic index model which could separate the high risk patients. However, the usefulness of this model should be confirmed in a larger patient population. The dose intensity of cyclophosphamide, during initial 3 months of treatment, was significantly associated with CR rate and overall survival(p=0.01 and 0.03, respectively). When International Prognostic Index Model was applied to our patients, patients in the lower risk groups had significantly better outcome than patients in the higher risk groups(3 year survival and RR: 77% and 1 for low risk group, 61% and 1.9 for low-intermediate risk group, 50% and 2.2 for high-intermediate risk group, and 25% and 6 for high risk group). CONCLUSION: In this study, we confirmed that features other than the Ann Arbor stage were independently associated with CR and survival, and the International Prognostic Index Model would be an useful tool for the selection of high-risk patients who could be benefited from more aggressive chemotherapy.