RESUMEN
INTRODUCTION: Allogeneic hematopoietic stem cell transplantation (AHSCT) is a curative approach for patients with myelodysplastic syndrome (MDS). METHODS: In this multicenter retrospective study, we analyzed the outcome of adult patients with MDS who underwent AHSCT in Argentina and evaluated the prognostic factors associated with progression-free survival (PFS), overall survival (OS), cumulative incidence (CI) of relapse, and non-relapse mortality (NRM). RESULTS: We analyzed data from 87 adults (median age: 43 years, range 18-66) who underwent SCT after myeloablative (n = 60) or non-myeloablative conditioning (n = 27), and from related (n = 62) or unrelated (n = 25) donors. For all patients, unadjusted 4-year PFS and OS were 37% and 38%, respectively; no significant differences were found between recipients of related or unrelated donors. One-year CI of relapse and NRM were 21% and 20%, respectively. In the multivariate analysis, intermediate disease risk index (DRI) and acute graft versus host disease AGVHD of all grades (I-IV) were independent variables associated with better PFS and lower relapse CI; only intermediate DRI was associated with better OS. CONCLUSIONS: AHSCT is a feasible procedure in Argentina, with more than 30% of the patients achieving long-term survival. Recipients with unrelated donors had at least similar outcome than those with related donors. DRI may be useful to identify patients at higher risk of relapse after transplantation.
Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Síndromes Mielodisplásicos/mortalidad , Síndromes Mielodisplásicos/terapia , Adolescente , Adulto , Anciano , Aloinjertos , Argentina/epidemiología , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
BACKGROUND: Allogeneic hematopoietic stem cell transplantation (AHSCT) represents the only curative treatment for the majority of pediatric patients with Myelodysplastic Syndrome (MDS). We aimed to evaluate overall survival (OS), disease-free survival (DFS), non-relapse mortality (NRM) and relapse incidence in children who underwent AHSCT for MDS in six institutions from Argentina. PROCEDURE: A retrospective analysis of 54 AHSCT was carried out in 52 patients (mean age: 9 years; range: 2-19; 35 males). RESULTS: MDS subtypes were refractory cytopenia of childhood (RCC) (n: 26, 50%), refractory anemia with excess blasts (RAEB) (n: 9, 18%), RAEB in transformation (RAEB-T) (n: 8, 15%) and juvenile myelomonocytic leukemia (JMML) (n: 9, 17%). At time of transplant, seven (13%) patients transformed to acute myeloid leukemia (AML) and two patients with RCC to RAEB. Donors were related in 32 cases (59%) and the stem cells source was: bone marrow (63%), peripheral blood (26%), and umbilical cord blood (11%). Five-year DFS and OS were 50% and 55% respectively; and for patients with JMML, 57% and 67% respectively. Cumulative incidence of NRM and relapse were 27% and 21% respectively. In the multivariate analysis, umbilical cord blood (HR 4.07; P = 0.025) and age ≥ 9 years at transplantation (HR 3.28; P = 0.017) were associated with lower OS; age and graft-versus-host disease (GVHD) had a higher NRM. CONCLUSIONS: In our series, more than half of the patients achieved long term OS with AHSCT. Less toxic conditioning regimens or more intensive GVHD prophylaxis could lead to better results in some children.
Asunto(s)
Enfermedad Injerto contra Huésped/epidemiología , Trasplante de Células Madre Hematopoyéticas , Síndromes Mielodisplásicos/terapia , Recurrencia Local de Neoplasia/epidemiología , Adolescente , Adulto , Argentina/epidemiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Enfermedad Injerto contra Huésped/mortalidad , Humanos , Lactante , Masculino , Síndromes Mielodisplásicos/mortalidad , Recurrencia Local de Neoplasia/mortalidad , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Acondicionamiento Pretrasplante , Trasplante Homólogo , Adulto JovenRESUMEN
Myelodysplastic syndromes (MDS) include a group of heterogeneous hematological disorders with a variable risk of leukemic evolution and short survival. Around 40-50% of patients show abnormal karyotypes that are mostly characterized by monosomies or deletions. Cytogenetic findings are an independent prognostic factor and the International prognostic scoring system (IPSS) differentiates three cytogenetic categories, despite the Intermediate one being heterogeneous. The aim of this study, including 421 Argentinean patients with primary MDS, is to characterize the cytogenetic profile, to test its prognostic value and to compare partial and monosomal karyotypes against other cytogenetic findings. An abnormal karyotype (median survival: 26 months) was observed in 176 patients. The presence of complex karyotypes, number of alterations, and the IPSS cytogenetic groups showed significant differences for predicting outcome. Behavior of patients with isolated deletions (median survival: 49 months) did not differ from those with normal karyotype (56 months, P = 0.654) or Good prognostic findings (43 months, P = 0.371). However, a worse prognosis was observed when another alteration was added (31 months, P = 0.043). Karyotypes with autosomal monosomies (median survival: 16 months) had a prognostic impact similar to other Poor cytogenetic findings (17 months, P = 0.626). In our population classified according to French-American-British (FAB) or World Health Organization (WHO), this new categorization of cytogenetic abnormalities, recognizing three different risk groups, showed an independent prognostic impact and a better discriminating power than the IPSS categories. It can be concluded that all isolate deletions (excluding 7q-) are good prognostic findings and all monosomies (excluding Y chromosome loss) are bad indicators.
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Cromosomas Humanos/genética , Monosomía , Síndromes Mielodisplásicos/genética , Síndromes Mielodisplásicos/mortalidad , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Argentina/epidemiología , Deleción Cromosómica , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
BACKGROUND: Doxorubicin/bleomycin/vinblastine/dacarbazine (ABVD) plus involved-field radiation therapy (IFRT) is the gold-standard treatment for early and advanced stages of Hodgkin lymphoma (HL). We evaluated the outcomes of patients according to prognosis at diagnosis and over time to determine who achieved complete remission (CR). PATIENTS AND METHODS: Treatment-naive patients under the age of 75 years at all stages of HL were eligible. The favorable group (FG) contained patients with stage IA-IIIA disease without bulky areas who achieved CR after the third cycle of ABVD. They received only IFRT at 25 Gy. Patients in the unfavorable group (UG) exhibited stages IIIB and IV HL. The UG also included all patients with bulky disease and the subset of the FG without CR after 3 cycles of ABVD, ie, slow responders (FGSR). The UG received 6 cycles of ABVD plus IFRT at 30 Gy to bulky areas at diagnosis or to those areas remaining positive after the third cycle of ABVD. RESULTS: In total, 584 patients were evaluable: 285 of them belonged to the FG, and 299 to the UG. Rates of CR were 98% and 85% for the FG and the UG, respectively (P < .001). Sixty patients in the FG received 6 cycles of ABVD because they had not achieved CR after 3 cycles (ie, the FGSR subgroup). The 5-year event-free survival rate was 89% for the FG, 66% for the FGSR, and 72% for the UG (P < .001). The overall survival at 5 years was significantly better for the FG (98%) than for the FGSR (87%) and the UG (88%; P < .001). CONCLUSION: Patients from the FG demonstrated excellent outcomes compared with those from the FGSR and UG, despite receiving less chemotherapy and fewer doses of IFRT.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Enfermedad de Hodgkin/terapia , Adolescente , Adulto , Anciano , Bleomicina/administración & dosificación , Niño , Terapia Combinada , Dacarbazina/administración & dosificación , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Esquema de Medicación , Femenino , Enfermedad de Hodgkin/mortalidad , Enfermedad de Hodgkin/patología , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Radioterapia , Dosificación Radioterapéutica , Inducción de Remisión , Factores de Riesgo , Vinblastina/administración & dosificación , Adulto JovenRESUMEN
La leucemia mieloide crónica (LMC) es un proceso oncohematológico caracterizado por una proliferación clonal que afecta a la célula hematopoyética primitiva. El 95 por ciento de los casos de LMC presenta una alteración citogenética característica conocida como Cromosoma Philadelphia (CR.Ph1). Esta alteración es producto de una traslocación cromosómica 9:22, que da lugar a un gen híbrido BCR/ABL. La terapéutica óptima para el tratamiento de la LMC en FC es un motivo de controversias. Sin embargo el trasplante Alogeneico de Médula Osea (TMO) ha logrado modificar la historia natural de la enfermedad, siendo el único procedimiento terapéutico que logra la curación. Un grupo de 19 pacientes con diagnóstico de LMC recibieron TMO en el Instituto de Trasplante de Médula Osea, Fundación Mainetti entre los años 1994 y 1997. De ellos, 9 fueron de sexo femenino y 10 de sexo masculino. La media de edad fue de 32 años (rango 9-47) 15 de los pacientes estaban en fase crónica (FC) y 4 en fase acelerada (FA). Todos los pacientes al momento del diagnóstico fueron CR.Ph1 positivo y presentaron la traslocación BCR/ABL. El régimen de acondicionamiento consistió en Busulfan y Ciclosfosfamida, con el agregado de Etoposido en los pacientes en FA. La profilaxis de EICH se efectuó con 3 drogas, Ciclosfosfamida, Metrotexato y Metilprednisona en 17 pacientes y con los 2 primeras drogas en 2 pacientes... (TRUNCADO)(AU)
Asunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Trasplante de Médula Ósea , Leucemia Mielógena Crónica BCR-ABL Positiva/terapia , Leucemia Mielógena Crónica BCR-ABL Positiva/complicaciones , Cromosoma Filadelfia , Trasplante Homólogo , Ciclofosfamida/uso terapéutico , Metotrexato/uso terapéutico , Metilprednisolona/uso terapéutico , Quimera por Trasplante , Sobrevivientes , Protocolos ClínicosRESUMEN
La leucemia mieloide crónica (LMC) es un proceso oncohematológico caracterizado por una proliferación clonal que afecta a la célula hematopoyética primitiva. El 95 por ciento de los casos de LMC presenta una alteración citogenética característica conocida como Cromosoma Philadelphia (CR.Ph1). Esta alteración es producto de una traslocación cromosómica 9:22, que da lugar a un gen híbrido BCR/ABL. La terapéutica óptima para el tratamiento de la LMC en FC es un motivo de controversias. Sin embargo el trasplante Alogeneico de Médula Osea (TMO) ha logrado modificar la historia natural de la enfermedad, siendo el único procedimiento terapéutico que logra la curación. Un grupo de 19 pacientes con diagnóstico de LMC recibieron TMO en el Instituto de Trasplante de Médula Osea, Fundación Mainetti entre los años 1994 y 1997. De ellos, 9 fueron de sexo femenino y 10 de sexo masculino. La media de edad fue de 32 años (rango 9-47) 15 de los pacientes estaban en fase crónica (FC) y 4 en fase acelerada (FA). Todos los pacientes al momento del diagnóstico fueron CR.Ph1 positivo y presentaron la traslocación BCR/ABL. El régimen de acondicionamiento consistió en Busulfan y Ciclosfosfamida, con el agregado de Etoposido en los pacientes en FA. La profilaxis de EICH se efectuó con 3 drogas, Ciclosfosfamida, Metrotexato y Metilprednisona en 17 pacientes y con los 2 primeras drogas en 2 pacientes... (TRUNCADO)