WCIM 2014
30 32nd World Congress of Internal Medicine (October 24-28, 2014)
involvement (27% vs 67%, p=0.004). According to the classifi cation of amyloidosis, increased mortality rates were observed in patients with AL amyloidosis (77% vs 57%, p<0.001), and lower rates in TTR (47% vs 68%, p<0.001) and other types of amyloi- dosis (40% vs 66%, p<0.001). Multivariate logistic regression analysis identifi ed as independent prognostic factors of mortality age (p<0.001), underlying chronic/degen- erative diseases (p <0.001) and renal (p=0.002) and cardiac (p=0.024) involvements.
Conclusions: The mortality rate in patients diagnosed with systemic amyloidosis in internal medicine reached two thirds of cases, with AL amyloidosis showing the high- est rate (80%). The main baseline prognostic factors independently associated with mortality were an older age, underlying chronic/degenerative disease and renal and cardiac involvement.
OS-HEM-09 Hematology
Prevalence and Mortality of Cardiac Involvement in Patients with Amyloidosis of an Institutional Registry Amyloidosis
María Adela AGUIRRE1, María Lourdes POSADAS-MARTINEZ1, Melisa BLOMBERG1, Dorotea Beatriz FANTL2, Diego Hernan GIUNTA1, Fernán GONZALEZ BERNALDO DE QUIRÓZ1, María Florencia GRANDE RATTI1, Gustavo GRELONI3, María Soledad SAEZ4, Patricia Beatríz SORROCHE4, Carlos Federico VARELA3, Gabriel Dario WAISMAN1, Elsa Mercedes NUCIFORA2
Clinical Research Unit, Hospital Italiano De Buenos Aires, Argentina1, Hematology Unit, Hospital Italiano De Buenos Aires, Argentina2, Nephrology Unit, Hospital Italiano De Buenos Aires, Argentina3, Biochemis- try Unit, Hospital Italiano De Buenos Aires, Argentina4
Background: Cardiac involvement is the leading cause of morbidity and mortality of amy- loidosis, especially due to light-chain (AL) and transthyretin, both wild and hereditary types.
Objectives: To estimate the prevalence and mortality of patients with evidence of cardiac involvement in patients diagnosed with amyloidosis.
Methods: Ambispective cohort study, with data obtained from all patients included between 01/2007 and 03/2013 with evidence of amyloidosis in the Institutional Regis- try Amyloidosis of the Hospital Italiano de Buenos Aires. The diagnostic criteria of car- diac amyloidosis were predefi ned. Mortality from cardiac amyloidosis was measured using a combination of active and passive assessment. Survival time was evaluated using the Kaplan-Meier estimator. SPSS 19.0 was used.
Results: The registry included 126 patients and the prevalence of cardiac amyloidosis was 40% (95%, CI:31-49%). Among the 40% (50/126) of patients with confi rmed amy- loidosis and suspected of cardiac involvement, the median age was 72 years (IQR 79-81) and 26% were women (13/50). Suspected diagnosis of cardiac involvement was: clinical 82.4% (42/50), from complementary studies 67% (34/50), and diagnosed from another hospital 6% (3/50). From all patients with suspected cardiac involvement, 47% had con- fi rmed biopsy. Overall mortality of patients with cardiac involvement was 20% (11/50), the median survival was 1782 days of follow up (CI 95% 1365-2199).
Conclusions: The prevalence of cardiac involvement and mortality in patients with amyloidosis was high. Advances in diagnosis of the possible causes of amyloidosis in the future will allow the detection of patients with high risk of death from cardiac causes in our country.
OS-HEM-10 Hematology
The Evidence of Graft-Versus-Tumor Effect in the Real World: Hsct for Refractory Leukemia
Inho KIM1, Youngil KOH1, Sung-Soo YOON1, Seonyang PARK1, Joon Ho MOON2, Sang Kyun SOHN2, June-Won CHEONG3, Yoo Hong MIN3, Hyeon Gyu YI4, Chul Soo KIM4, Sung-Kyu PARK5, Dae-Sik HONG5, Dae-Young KIM6, Jung-Hee LEE6, Je-Hwan LEE6, Kyoo-Hyung LEE6
Seoul National University Hospital, Korea1, Kyungpook National University Hospital, Korea2, Yonsei Uni- versity Severance Hospital, Korea3, Inha University Hospital, Korea4, Soonchunhyang University Hospital, Korea5, Asan Medical Center, Korea6
Background: Chemotherapy-refractory acute leukemia is only salvaged by allogeneic stem cell transplantation(ASCT). We intended to analyze clinical outcome of patients with acute leukemia who received ASCT in chemo-refractory state. With this analysis, we tried to fi nd subset who could be salvaged by ASCT.
Methods: We retrospectively analyzed patients who received ASCT for chemo-refrac- tory acute leukemia. We included patients who received ASCT while the disease was not in complete remission.
Results: A total of 226 patients were analyzed. Disease included acute myeloid leu- kemia (AML, N=156), acute lymphocytic leukemia (ALL, N=52), and acute biphenotypic leukemia (ABL, N=18). Proportion of secondary leukemia was 15.9%. 41.6% of patients were primary chemo-refractory, while 58.4% of patients developed chemo-refractori- ness after achievement of CR at least once. CR was achieved in 79.6% of patients by ASCT. CR rate seemed to be higher in AML (84%) compared to ALL (71%) or ABL (67%) (p=0.058). OS was signifi cantly superior in patients with AML or ABL compared to ALL (p=0.020). Five-years OS rate was more than 20% in patients with AML or ABL, while 5-years OS rate was less than 5% in patients with ALL. Patients who received sibling ASCT had better survival outcome compared to unrelated ASCT or haploidentical ASCT (p<0.001). Primary chemo-refractoriness (p=0.284) and conditioning intensity (p=0.404) did not affect survival outcome. Karyotypic risk grouping had prognostic value in these patients with shorter OS observed in patients with poor risk group com- pared to standard or good risk group (p=0.006).
Conclusions: Among acute leukemias, only AML and ABL are salvaged by ASCT when they are chemo-refractory. However, outcome of primary chemo-refractory disease is not inferior, hence ASCT should be considered in this disease. For ASCT in chemo-re- fractory acute leukemia, sibling ASCT has better outcome than unrelated ASCT.
OS-HEM-11 Hematology
Impact of Stem Cell Dose and Performance Status on Cord Blood Transplantation (CBT) Outcome in Adult Patients with Advanced Hematologic Diseases: 13-Year Experience in Korea
Ji-Won KIM1, Junglim LEE2, So Young CHONG3, Chul Won JUNG4, Yeo-Kyeoung KIM5, Yeung-Chul MUN6, Deog-Yeon JO7, Hong Ghi LEE8, Jong-Ho WON9, Joon Seong PARK10, Jae Hoon LEE11, Joon Ho MOON12, Young Rok DO13, June-Won CHEONG14, Je-Hwan LEE15, Ho-Young YHIM16, Haesook KIM17, Sung-Soo YOON1
Seoul National University Hospital, Korea1, Daegu Fatima Hospital, Korea2, Bundang CHA University, Korea3, Samsung Medical Center, Korea4, Chonnam National University Hwasun Hospital, Korea5, Ewha Womans University Mokdong Hospital, Korea6, Chungnam National University Hospital, Korea7, Konkuk University Hospital, Korea8, Soon Chun Hyang University Hospital, Korea9, Ajou University Hospital, Korea10, Gachon University Gil Hospital, Korea11, Kyungpook National University Hospital, Korea12, Dongsan Medical Center, Korea13, Yonsei University Severance Hospital, Korea14, Asan Medical Center, Korea15, Chonbuk National University Hospital, Korea16, Dana-Farber Cancer Institute, USA17 Background: CBT is an established alternative donor transplantation for treatment of patients with advanced hematologic diseases.
Methods: To assess clinical outcome after CBT and provide future recommendations for alternative donor, we conducted the fi rst retrospective multicenter study of adult CBT in Korea.
Results: Between June 2001 and April 2014, 48 patients (male, 19) were enrolled from 16 hospitals. The median age was 36 years (range, 16-65 years). Forty patients (83.3%) were acute leukemia: 33 (68.8%) in remission and 7 (14.6%) in persistence.
The others were myelodysplastic syndrome (n=3, 6.3%), severe aplastic anemia (n=3, 6.3%), and lymphoma (n=2, 4.2%). Thirty two patients (66.7%) underwent double unit CBT. Twenty two patients (45.8%) underwent myeloablative conditioning and 26 patients (54.2%), reduced-intensity conditioning. Total body irradiation (TBI)- based conditioning regimens were used in 23 patients (47.9%). The median time to neutrophil >500/μL was 20 days among grafted, and 14 patients (29.2%) failed to recover. During follow-up, 8 patients died of disease and non-relapse mortality (NRM)
