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1.
Hematology ; 25(1): 366-371, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33095117

RESUMO

OBJECTIVE: To describe chronic lymphocytic leukemia (CLL) treatment patterns and patient outcomes in Latin America. METHODS: This chart review study (NCT02559583; 2008-2015)evaluated time to progression (TTP) and overall survival (OS) outcomes among patients with CLL who initiate done (n = 261) to two (n = 96) lines of therapy (LOT) since diagnosis. Differences in TTP and OS were assessed by Kaplan-Meier analysis, with a log-rank test for statistical significance. Association between therapeutic regimen and risk for disease progression or death was estimated using Cox proportional hazard regression. RESULTS: The most commonly prescribed therapies in both LOTs were chlorambucil-, followed by fludarabine- and cyclophosphamide (C)/CHOP-based therapies. Chlorambucil- and C/CHOP-based therapies were largely prescribed to elderly patients (≥65 years) while fludarabine-based therapy was predominantly used by younger patients (≤65 years). In LOT1, relative to chlorambucil-administered patients, those prescribed fludarabine-based therapies had lower risk of disease progression (hazard ratio [HR] and 95% confidence interval [CI] 0.32 [0.19-0.54]), whereas C/CHOP-prescribed patients had higher risk (HR 95%CI 1.88 [1.17-3.04]). Similar results were observed in LOT2. There was no difference in OS between treatments in both LOTs. DISCUSSION: Novel therapies such as kinase inhibitors were rarely prescribed in LOT1 or LOT2in Latin America. The greater TTP observed forfludarabine-based therapies could be attributed to the fact that fludarabine-based therapies are predominantly administered to young and healthy patients. CONCLUSION: Chlorambucil-based therapy, which has limited benefits, is frequently prescribed in Latin America. Prescribing novel agents for fludarabine-based therapy-ineligible patients with CLL is the need of the hour. Trial registration: ClinicalTrials.gov identifier: NCT02559583.

2.
Transpl Infect Dis ; : e13350, 2020 Jun 05.
Artigo em Inglês | MEDLINE | ID: mdl-32500925

RESUMO

Adult T-cell lymphoma is an aggressive and poor prognosis HTLV1-associated lymphoma. There is no standard treatment, but it is known that intensive chemotherapy regimens are necessary, with or without concomitant antiretroviral therapy, plus consolidation with allogeneic stem cell transplantation. Our case report shows a favorable outcome after 2 cycles of chemotherapy and allogeneic stem cell transplantation without antiretroviral agents, achieving complete remission, and a negative proviral load.

3.
Medicina (B Aires) ; 80(1): 81-83, 2020.
Artigo em Espanhol | MEDLINE | ID: mdl-32044744

RESUMO

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.


Assuntos
Neoplasias do Íleo/patologia , Ileíte/patologia , Linfoma/patologia , Síndrome de Wiskott-Aldrich/patologia , Adulto , Biópsia , Diagnóstico Diferencial , Humanos , Neoplasias do Íleo/diagnóstico , Ileíte/diagnóstico , Imuno-Histoquímica , Linfoma/diagnóstico , Masculino , Síndrome de Wiskott-Aldrich/diagnóstico
4.
Medicina (B.Aires) ; 80(1): 81-83, feb. 2020. ilus
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1125040

RESUMO

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.


Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

5.
J Glob Oncol ; 5: 1-19, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31774711

RESUMO

PURPOSE: Limited information is available on multiple myeloma (MM), chronic lymphocytic leukemia (CLL), and non-Hodgkin lymphoma (NHL) management in Latin America. The primary objective of the Hemato-Oncology Latin America (HOLA) study was to describe patient characteristics and treatment patterns of Latin American patients with MM, CLL, and NHL. METHODS: This study was a multicenter, retrospective, medical chart review of patients with MM, CLL, and NHL in Latin America identified between January 1, 2006, and December 31, 2015. Included were adults with at least 1 year of follow-up (except in cases of death within 1 year of diagnosis) treated at 30 oncology hospitals (Argentina, 5; Brazil, 9; Chile, 1; Colombia, 5; Mexico, 6; Panama/Guatemala, 4). RESULTS: Of 5,140 patients, 2,967 (57.7%) had NHL, 1,518 (29.5%) MM, and 655 (12.7%) CLL. Median follow-up was 2.2 years for MM, 3.0 years for CLL, and 2.2 years for NHL, and approximately 26% died during the study observation period. Most patients had at least one comorbidity at diagnosis. The most frequent induction regimen was thalidomide-based chemotherapy for MM and chlorambucil with or without prednisone for CLL. Most patients with NHL had diffuse large B-cell lymphoma (DLBCL; 49.1%) or follicular lymphoma (FL; 19.5%). The majority of patients with DLBCL or FL received rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. CONCLUSION: The HOLA study generated an unprecedented level of high-quality, real-world evidence on characteristics and treatment patterns of patients with hematologic malignancies. Regional disparities in patient characteristics may reflect differences in ethnoracial identity and level of access to care. These data provide needed real-world evidence to understand the disease landscape in Latin America and may be used to inform clinical and health policy decision making.


Assuntos
Leucemia Linfocítica Crônica de Células B/epidemiologia , Linfoma não Hodgkin/epidemiologia , Mieloma Múltiplo/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Humanos , América Latina/epidemiologia , Pessoa de Meia-Idade , Sistema de Registros , Adulto Jovem
6.
Br J Haematol ; 185(5): 865-873, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30864146

RESUMO

The role of Ann Arbor staging in determining treatment intensity after achieving a negative positron emission tomography (PET) has not been established in classical Hodgkin lymphoma (cHL). Patients with stage I-IV cHL, received three cycles of ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) and an interim PET scan (PET3). PET3-negative patients received no further therapy. PET3-positive patients received three additional cycles of ABVD plus involved-field radiation therapy or salvage chemotherapy, if refractory to ABVD, and were re-evaluated by PET scan (PET6). Study endpoints were 3-year progression-free survival (PFS) and overall survival (OS) rates. Two hundred and thirty-nine patients with early-stage and 138 with advanced-stage were evaluable. Overall, 260 patients (70%) were PET3-negative and had higher 3-year PFS (90% vs. 65%; P < 0·0001) and OS (98% vs. 92%; P = 0·007) rates than PET3-positive patients. All PET3-negative patients, regardless of disease stage at diagnosis, achieved similarly good PFS (90-91%; P = 0·76) and OS (97-99%). The only independent prognostic factor for PFS was PET3-negativity (Hazard ratio 3·8; 95% confidence interval 2·4-6·3; P < 0·0001). This study suggests that cHL patients who achieve a negative PET3 following ABVD have an excellent outcome, regardless of stage at diagnosis. An appropriately powered, phase III trial will be necessary to confirm these findings.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/diagnóstico por imagem , Doença de Hodgkin/tratamento farmacológico , Tomografia por Emissão de Pósitrons/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/farmacologia , Bleomicina/farmacologia , Bleomicina/uso terapêutico , Dacarbazina/farmacologia , Dacarbazina/uso terapêutico , Doxorrubicina/farmacologia , Doxorrubicina/uso terapêutico , Feminino , Doença de Hodgkin/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Análise de Sobrevida , Vimblastina/farmacologia , Vimblastina/uso terapêutico , Adulto Jovem
7.
Rev. chil. infectol ; 34(6): 535-538, dic. 2017. tab, graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-899755

RESUMO

Resumen Introducción: La infección del torrente sanguíneo es una complicación usual, que puede comprometer la vida de los pacientes que recibieron trasplante de precursores hematopoyéticos. Objetivo: Analizar las características de las infecciones del torrente sanguíneo en receptores de trasplante de precursores hematopoyéticos. Materiales y Métodos: Estudio observacional, retrospectivo. Se revisaron los registros de 451 pacientes (trasplantes autólogos y alogénicos), desde enero de 2009 a octubre de 2015. Resultados: Hubo 99 hemocultivos positivos en 73 pacientes con infección del torrente sanguíneo (16%). Mortalidad atribuible a causas infecciosas: 17%. De las 99 infecciones sanguíneas, 63% fueron provocados por bacilos gramnegativos (Escherichia coli 45%, Klebsiella spp 23%, Pseudomonas spp 11%, Acinetobacter spp 6% y otros bacilos gramnegativos 15%), 33% por cocáceas grampositivas, 3% por hongos y 1% por bacilos grampositivos. Se observó resistencia a ciprofloxacina (81%), piperacilina/tazobactam (48%), Enterobacteriaceae productoras de β-lactamasa de espectro extendido (BLEE) (40%), cefepime (39%) y ausencia de resistencia a amikacina. Discusión: Existe mayor frecuencia de infección por bacilos gramnegativos, con un importante porcentaje de aislados multi-resistentes, y consecuente, alta resistencia al tratamiento antimicrobiano empírico.


Background: Bloodstream infection is a common complication, which can be life-threatening for hematopoietic stem cells transplant recipients. Objective: To analyze the characteristics of bloodstream infections in hematopoietic stem cell transplant recipients. Materials and Methods: Observational, retrospective study. We reviewed the records of 451 patients (autologous and allogeneic transplants) from January 2009 to October 2015. Results: 99 positive blood cultures in 73 patients with bloodstream infection (16%) were found. Mortality attributable to infectious causes was 17%. From the 99 bloodstream infection, 63% were caused by gram-negative bacilli (Escherichia coli 45%, Klebsiella spp 23%, Pseudomonas spp 11% Acinetobacter spp % and other bacilli 15%), 33% by gram-positive cocci, 3% by fungi and 1% by gram-positive bacilli. The gram-negative bacilli were ciprofloxacin resistant (81%), piperacillin/tazobactam resistant (48%), extended-spectrum beta-lactamase (ESBL) producing Enterobacteriaceae (40%), cefepime resistant (39%) and there was no resistance noted to amikacin. Discussion: There is a higher frequency of gram-negative bacilli infection, with a high percentage of multiresistant microorganisms and high resistance to empirical antibiotic treatment.

8.
Rev Chilena Infectol ; 34(6): 535-538, 2017 Dec.
Artigo em Espanhol | MEDLINE | ID: mdl-29488545

RESUMO

BACKGROUND: Bloodstream infection is a common complication, which can be life-threatening for hematopoietic stem cells transplant recipients. OBJECTIVE: To analyze the characteristics of bloodstream infections in hematopoietic stem cell transplant recipients. MATERIALS AND METHODS: Observational, retrospective study. We reviewed the records of 451 patients (autologous and allogeneic transplants) from January 2009 to October 2015. RESULTS: 99 positive blood cultures in 73 patients with bloodstream infection (16%) were found. Mortality attributable to infectious causes was 17%. From the 99 bloodstream infection, 63% were caused by gram-negative bacilli (Escherichia coli 45%, Klebsiella spp 23%, Pseudomonas spp 11% Acinetobacter spp % and other bacilli 15%), 33% by gram-positive cocci, 3% by fungi and 1% by gram-positive bacilli. The gram-negative bacilli were ciprofloxacin resistant (81%), piperacillin/tazobactam resistant (48%), extended-spectrum beta-lactamase (ESBL) producing Enterobacteriaceae (40%), cefepime resistant (39%) and there was no resistance noted to amikacin. DISCUSSION: There is a higher frequency of gram-negative bacilli infection, with a high percentage of multiresistant microorganisms and high resistance to empirical antibiotic treatment.


Assuntos
Bacteriemia/epidemiologia , Bacteriemia/microbiologia , Fungemia/epidemiologia , Fungemia/microbiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Adolescente , Adulto , Distribuição por Idade , Antibacterianos/uso terapêutico , Antifúngicos/uso terapêutico , Argentina/epidemiologia , Bacteriemia/tratamento farmacológico , Bactérias/efeitos dos fármacos , Bactérias/isolamento & purificação , Resistência Microbiana a Medicamentos , Feminino , Fungemia/tratamento farmacológico , Fungos/efeitos dos fármacos , Fungos/isolamento & purificação , Humanos , Masculino , Testes de Sensibilidade Microbiana , Pessoa de Meia-Idade , Estudos Retrospectivos , Distribuição por Sexo , Adulto Jovem
9.
Hematology ; 21(3): 162-9, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26147089

RESUMO

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.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Síndromes Mielodisplásicas/mortalidade , Síndromes Mielodisplásicas/terapia , Adolescente , Adulto , Idoso , Aloenxertos , Argentina/epidemiologia , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
10.
Medicina (B Aires) ; 75(4): 201-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26339873

RESUMO

We have retrospectively reviewed 137 medical records of patients older than 50 years receiving an allogeneic hematopoietic stem cell transplantation (HSCT) between January 1997 and July 2013. Median follow up was 1.3 years. Sex, age, diagnosis, disease stage, comorbidities (according to HCT-CI score), type of donor, histocompatibility, conditioning regimen and graft-versus-host disease (GVHD) prophylaxis were evaluated. The incidence and severity of acute and chronic GVHD, overall survival (OS), disease free survival (DFS), non-relapse mortality (NRM) and relapse were investigated according those variables. Acute GVHD incidence was 41% (7.3% GIII-IV). Patients with acute myeloid leukemia had lesser aGVH GII-IV (14% vs. 35%, p<0.01) comparing to the entire population. Extensive cGVHD incidence was 9.4%. Global OS 1-3 years was 44-20%, DFS 33-20%, relapse 35-41% and NRM 36-43% respectively. The presence of comorbidities showed a significant increase in NRM (CT-CI 0 vs. 1 vs ≥2: 1-3 years 17-24% vs. 40-46% vs. 45-67%, p=0.001, MA HR 2.03, CI 95% 1.02-5.29), as well as cyclosporine vs. tacrolimus (1-3 years 47-53% vs. 25-36%, p=0.01). Tacrolimus patients had higher 1-3 years OS (49-25% vs. 31-13%, p=0.01) and DFS (41-26% vs. 20-11%, p<0.01). Age, type of donor and myeloablative conditioning showed no significant differences in any outcome. Allogeneic HSCT is a valid therapeutic option for older patients in Argentina. The main risk factor for a significantly increased NRM and a trend to inferior OS was the number of comorbidities. Age was not a factor for a worse result. The other factor having a significant effect in better outcome was tacrolimus administration.


Assuntos
Doença Enxerto-Hospedeiro/mortalidade , Transplante de Células-Tronco Hematopoéticas/mortalidade , Fatores Etários , Idoso , Ciclosporina/uso terapêutico , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Tacrolimo/uso terapêutico , Fatores de Tempo
11.
Medicina (B.Aires) ; 75(4): 201-206, Aug. 2015. graf, tab
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-841495

RESUMO

We have retrospectively reviewed 137 medical records of patients older than 50 years receiving an allogeneic hematopoietic stem cell transplantation (HSCT) between January 1997 and July 2013. Median follow up was 1.3 years. Sex, age, diagnosis, disease stage, comorbidities (according to HCT-CI score), type of donor, histocompatibility, conditioning regimen and graft-versus-host disease (GVHD) prophylaxis were evaluated. The incidence and severity of acute and chronic GVHD, overall survival (OS), disease free survival (DFS), non-relapse mortality (NRM) and relapse were investigated according those variables. Acute GVHD incidence was 41% (7.3% GIII-IV). Patients with acute myeloid leukemia had lesser aGVH GII-IV (14% vs. 35%, p < 0.01) comparing to the entire population. Extensive cGVHD incidence was 9.4%. Global OS 1-3 years was 44-20%, DFS 33-20%, relapse 35-41% and NRM 36-43% respectively. The presence of comorbidities showed a significant increase in NRM (CT-CI 0 vs. 1 vs ≥ 2: 1-3 years 17-24% vs. 40-46% vs. 45-67%, p = 0.001, MA HR 2.03, CI 95% 1.02-5.29), as well as cyclosporine vs. tacrolimus (1-3 years 47-53% vs. 25-36%, p = 0.01). Tacrolimus patients had higher 1-3 years OS (49-25% vs. 31-13%, p = 0.01) and DFS (41-26% vs. 20-11%, p < 0.01). Age, type of donor and myeloablative conditioning showed no significant differences in any outcome. Allogeneic HSCT is a valid therapeutic option for older patients in Argentina. The main risk factor for a significantly increased NRM and a trend to inferior OS was the number of comorbidities. Age was not a factor for a worse result. The other factor having a significant effect in better outcome was tacrolimus administration.


Se efectuó un análisis retrospectivo de 137 historias clínicas de pacientes mayores de 50 años que recibieron un trasplante alogénico de precursores hematopoyéticos (TAPH). Se evaluaron las siguientes características: sexo, edad, enfermedad, estadio, comorbilidades (según el HCT-CI), donante, acondicionamiento e inmunosupresión. Se analizó la incidencia de enfermedad injerto vs. huésped aguda (aEICH) y crónica (cEICH), supervivencia global (SG), supervivencia libre de enfermedad (SLE), recaída y mortalidad libre de enfermedad (MLE). Los trasplantes fueron realizados entre 1997-2013, mediana de seguimiento 1.3 años. La incidencia de aEICH fue de 41% (7.3% GIII-IV). Los pacientes con leucemia mieloide aguda presentaron menor incidencia de EICHa GII-IV (14% vs. 34%, p < 0.01). La incidencia de EICHc extenso fue de 9.4%. La SG a 1-3 años fue 44-20%, SLE 33-20%, recaída 35-41% y la MLE 36-43%. Los pacientes con comorbilidades tuvieron un aumento significativo de la MLE (HCT-CI 0 vs. 1 vs. ≥2: 1-3 años 17-24% vs. 40-46% vs. 45-67%, p = 0.001, AMV HR 2.03, IC 95% 1.02-5.29), al igual que el uso de ciclosporina vs. tacrolimus (1-3 años 47-53% vs. 25-36%, p = 0.01). Los pacientes que recibieron tacrolimus tuvieron una mayor SG (1-3 años 49-25% vs. 31-13%, p = 0.01) y SLE (1-3 años 41-26% vs. 20-11%, p < 0.01). La edad, tipo de donante y acondicionamiento no resultaron significativos para ningún evento. El TAPH es una herramienta terapéutica válida en pacientes mayores. Los factores pronósticos que inciden mayormente en el trasplante son las comorbilidades y no la edad. El otro factor que demostró un efecto significativo fue el uso de tacrolimus.

12.
Open Forum Infect Dis ; 2(2): ofv060, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26180822

RESUMO

We report a case of Chagas disease reactivation in a patient with stage IIb follicular lymphoma in the cecum. He was admitted to the hospital with neutropenia and fever. He had a history of right hemicolectomy 6 months earlier and had received the sixth cycle of chemotherapy with cyclophosphamide/doxorubicin/vincristine/prednisone/rituximab. Blood and urine cultures were negative, but the fever persisted. Reactivation of Chagas disease was confirmed by means of quantitative real-time polymerase chain reaction (qRT-PCR). Parasitic load was 577 950 parasite equivalents/mL. The patient began treatment with benznidazole 5 mg/k per day every 12 hours. After 1 month, the qRT-PCR control was undetectable. The patient completed 60 days of treatment and is currently asymptomatic. Trypanosoma cruzi qRT-PCR may become a useful diagnostic method for reactivation of Chagas disease.

13.
Pediatr Blood Cancer ; 62(1): 153-7, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25264233

RESUMO

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.


Assuntos
Doença Enxerto-Hospedeiro/epidemiologia , Transplante de Células-Tronco Hematopoéticas , Síndromes Mielodisplásicas/terapia , Recidiva Local de Neoplasia/epidemiologia , Adolescente , Adulto , Argentina/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Doença Enxerto-Hospedeiro/mortalidade , Humanos , Lactente , Masculino , Síndromes Mielodisplásicas/mortalidade , Recidiva Local de Neoplasia/mortalidade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Condicionamento Pré-Transplante , Transplante Homólogo , Adulto Jovem
16.
Hematology ; 14(6): 323-30, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19941738

RESUMO

OBJECTIVE: To determine the prevalence of JAK2 V617F mutation and its clinical correlation in patients with chronic myeloproliferative disorders (CMD): polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). MATERIALS AND METHODS: Detection of JAK2 V617F mutation by allele specific-PCR. RESULTS: One hundred and three patients with CMD were included in the study. JAK2 V617F distribution was PV 40/45 (89%), ET 30/43 (69%), and IMF 7/15 (47%). In PV and ET patients only, 18 had thrombosis at diagnosis and 12 during follow-up (these were microvascular: 11, venous: 7 and arterial: 12); of these 28/70 (40%) were JAK2pos versus 2/18 (11%) JAK2neg; P=0.02. In a median of 4 years, two patients with PV JAK2pos evolved to myelofibrosis and one patient with PV presented in leukemic transformation (JAK2pos before and after transformation); six patients died: four patients with IMF and two patients with PV. CONCLUSIONS: We found an association between JAK2 V617F and thrombotic events in patients with PV and ET.


Assuntos
Alelos , Janus Quinase 2/genética , Mutação de Sentido Incorreto , Transtornos Mieloproliferativos/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Substituição de Aminoácidos , Doença Crônica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/complicações , Reação em Cadeia da Polimerase , Estudos Prospectivos , Trombose/etiologia , Trombose/genética
17.
Hematología (B. Aires) ; 13(2): 49-52, mayo-ago. 2009. tab, graf
Artigo em Inglês | LILACS | ID: lil-547257

RESUMO

Background: Patients with Refractory/Relapsed (RIR) acute leukemia (AL) have a poor prognosis. Objective: We aimed to evaluate the chemotherapy regimen fludarabine, cytarabine, granulocyte colony-stimulating factor, and idarubicin (FLAG-IDA) in patients with RIR AL. Patients: We studied 33 patients with R/R AL. Distribution of the AL subtype was: myeloblastic n=17 (52%), lymphoblastic n=14 (42%),) and biphenotypic n=2 (6%). Results: Complete remission (CR) was achieved in 15 cases (45.5%) and seven patients dead resulting in a mortality of 21.1%. In patients with hematological recovery the median time to neutrophils recovery (> 0.5 x 10º/1) was 24 days (range 10-38); platelet levels of more than 20 x 1Oº/l and 50 x 10º/1 were reached in a median time of 24 (range 17-44) and 27 days (range 18-51), respectively. After CR, five patients underwent allogeneic transplan- tation and 10 patients received a second course of FLAG – IDA. Ten out of 15 patients who achieved CR with FLAG-IDA relapsed at a median of 7.7 months (95% CI 1.8 to 13.6 months). Overall survival (OS) after FLAG-IDA in the surviving cohort had a median of 4 months. We found a significantly better OS in patients who received allogeneic transplantation post-FLAG-IDA than those who did not (median 11.4 months vs. 2.7 monthsj HR 0.29; 95% CI 0.1 to 0.6; p=0.017). Conclusions: In our series, FLAG-IDA demonstrated to be an effective salvage chemotherapy regimen, however, the benefit in survival of this rescue treatment was restrained to patients who unde.rwent al1ogeneic transplantation


Assuntos
Leucemia , Transplante
18.
Hematología (B. Aires) ; 13(2): 49-52, mayo-ago. 2009. tab, graf
Artigo em Inglês | BINACIS | ID: bin-124594

RESUMO

Background: Patients with Refractory/Relapsed (RIR) acute leukemia (AL) have a poor prognosis. Objective: We aimed to evaluate the chemotherapy regimen fludarabine, cytarabine, granulocyte colony-stimulating factor, and idarubicin (FLAG-IDA) in patients with RIR AL. Patients: We studied 33 patients with R/R AL. Distribution of the AL subtype was: myeloblastic n=17 (52%), lymphoblastic n=14 (42%),) and biphenotypic n=2 (6%). Results: Complete remission (CR) was achieved in 15 cases (45.5%) and seven patients dead resulting in a mortality of 21.1%. In patients with hematological recovery the median time to neutrophils recovery (> 0.5 x 10º/1) was 24 days (range 10-38); platelet levels of more than 20 x 1Oº/l and 50 x 10º/1 were reached in a median time of 24 (range 17-44) and 27 days (range 18-51), respectively. After CR, five patients underwent allogeneic transplan- tation and 10 patients


Assuntos
Leucemia , Transplante
19.
Haematologica ; 92(5): 704-5, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17488700

RESUMO

Granulocyte activation parameters have been described in patients with myeloproliferative disorders (MPD). We have evaluated the accuracy of leukocyte alkaline phosphatase (LAP) score to predict JAK2 V617F mutation. LAP score was obtained using a cytochemical reaction in granulocytes of patients' peripheral blood with MPD.


Assuntos
Fosfatase Alcalina/sangue , Janus Quinase 2/genética , Leucócitos/enzimologia , Mutação de Sentido Incorreto , Transtornos Mieloproliferativos/sangue , Mutação Puntual , Adulto , Idoso , Substituição de Aminoácidos , Área Sob a Curva , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Mieloproliferativos/enzimologia , Ativação de Neutrófilo , Valor Preditivo dos Testes , Método Simples-Cego
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