http://chineseinput.net/에서 pinyin(병음)방식으로 중국어를 변환할 수 있습니다.
변환된 중국어를 복사하여 사용하시면 됩니다.
Clinical features of severe ADAMTS13 deficienct- thrombotic thrombocytopenic purpura
( Doyeun Oh ),( In-ho Kim ),( Soo-mee Bang ),( Chul-won Jung ),( Jong-wook Lee ),( Hong Ghi Lee ) 대한내과학회 2015 대한내과학회 추계학술발표논문집 Vol.2015 No.1
Background: Diagnostic and prognostic value of ADAMTS13 activity is controversial. We previously reported the characteristics of severe ADAMTS13 deficiency in thrombotic thrombocytopenic purpura (TTP) and patients with severe ADAMTS13 deficiency (Jang MJ et al, Int J Hematol 2011; 93: 163-9). In this report, we enrolled 95 additional patients from January 2009 to June 2014 and analyzed 161 TTPpatients using same methods. Methods: One-hundred sixty nine patients with TTP from January 2005 to June 2014 were analyzed. ADAMTS13 activity and inhibitors were measured by immunoblotting of degraded von Willebrand factor. Clinical information was retrospectively collected and analyzed. Results: Patients with severe ADAMTS13 deficiency at presentation had lower serum creatinine levels (p<0.0001), lower platelet counts (p<0.0001), and higher total bilirubin levels (p=0.0001) than patients with non-severe ADAMTS13 deficiency. Treatment outcomes did not differ significantly between two groups in response, remission, and mortality rate. After adjusting for clinical and laboratory features, multivariate analysis revealed age over 60 year old is an independent risk factor for TTP-associated mortality (p=0.0249). Conclusions: TTP with severe ADAMTS13 deficiency is a unique subgroup characterized by lower platelet count and relatively good renal function. The severity of ADAMTS13 deficiency at presentation does not have prognostic significance.
방수미,EunKyungCho,CheolwonSuh,Sung-SooYoon,ChuMyungSeong,KyungSamCho,YoonGooKang,SeonyangPark,Myung-JuAhn,YoungSukPark,DoyeunOh,정철원,SamyongKim 대한의학회 2003 Journal of Korean medical science Vol.18 No.5
We conducted a phase II multicenter trial to estimate the response and survival of patients with newly diagnosed multiple myeloma to high dose melphalan therapy followed by autologous peripheral blood stem cell transplantation. Eligible patients who had undergone induction with vincristine, adriamycin and dexamethasone (VAD) should have adequate cardiac, pulmonary and renal function (creatinine <2 mg/dL). Melphalan at 200 mg/m2 was used as a conditioning regimen. Eighty patients were enrolled from 13 centers. The median age of the patients was 53 yr (range; 20 to 68 yr). The initial stage was IA/IIA/IIB/IIIA/IIIB in 3/8/1/54/14 patients, respectively. Beta2-microglobulin, CRP and LDH were increased in 74, 42 and 34% of the patients examined. Cytogenetic data were available in 30 patients, and 6 patients showed numeric or structural abnormalities. Two therapy-related mortalities occurred from infection. Among the 78 evaluable patients, CR/PR/MR/NC/PD were achieved in 48/26/2/1/1patients, respectively. After a median follow-up of 30 months, the median overall and event-free survivals were 66 months (95% CI: 20-112) and 24 months (95% CI: 18-29), respectively. This study verifies the efficacy and feasibility of high dose melphalan therapy with autologous stem cell transplantation in newly diagnosed multiple myeloma.