RESUMO
Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.
Assuntos
Neuropatias Amiloides Familiares , Polineuropatias , Humanos , Feminino , Idoso , Pré-Albumina/genética , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/genética , Mutação , Polineuropatias/etiologia , Polineuropatias/genéticaRESUMO
BACKGROUND: Before the advent of tyrosine kinase inhibitors (TKIs), patients with Philadelphia-positive Acute Lymphoblastic Leukemia (Ph+ALL) had a poor prognosis. The association of TKIs to intensive chemotherapy (CT) improved outcome. AIM: To evaluate results of an intensive CT protocol including TKI in a public hospital in Santiago, Chile. MATERIAL AND METHODS: All patients with Ph+ALL diagnosed between January 2010 and February 2019, and who met inclusion criteria for intensive CT, received the Ph+ALL national protocol in association with imatinib and were included in this analysis. RESULTS: Thirty-five patients aged 15 to 59 years received treatment. Complete response (CR) was obtained in 97%. Measurable residual disease (MRD) was negative in 61% (19/31 evaluable cases) during follow-up, and 55% (16/29) were MRD (-) before three months. Relapse was observed in 13 cases. Three patients underwent allogeneic hematopoietic stem cell transplant (HSCT), two in CR1. The overall survival (OS) and event-free survival (EFS) at three years were 52 and 34%, respectively. In patients who achieved MRD negativity before three months, no statistically significant differences in OS (64 and 42% respectively, p = 0.15) or EFS (35 and 32% respectively, p = 0.37) were observed. CONCLUSIONS: The prognosis of Ph+ALL improved with the association of imatinib to intensive CT. MRD-negative status before three months in this series was not significantly associated with better outcomes. Our series suggests that the Ph+ALL national protocol associated to TKI is a therapeutic alternative with high CR and aceptable MRD (-) rates.
Assuntos
Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adolescente , Adulto , Humanos , Mesilato de Imatinib/uso terapêutico , Pessoa de Meia-Idade , Neoplasia Residual/diagnóstico , Neoplasia Residual/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Adulto JovemRESUMO
Intrathecal chemotherapy may be complicated with the development of myelopathies or toxic radiculopathies. This myeloradicular involvement, of toxic character, is unpredictable, since these patients have repeatedly received Intrathecal chemotherapy with the same drugs without apparent injury. The toxic effect should be mainly attributed to Cytarabine and not to methotrexate, since the central nervous system lacks Cytidine deaminase, the enzyme that degrades Cytarabine. We report two patients, an 18-year-old woman and a 16 years old male, who received systemic and intrathecal chemotherapy (methotrexate, cytarabine) for the treatment of an acute lymphoblastic leukemia and developed, in relation to this procedure, a spinal subacute combined degeneration. They had a proprioceptive and motor alteration of the lower extremities and neuroimaging showed selective rear and side spinal cord hyper intensity produced by central axonopathy. Two weeks later the woman developed a quadriplegia and the young man a flaccid paraplegia due to added root involvement.
Assuntos
Antimetabólitos Antineoplásicos/efeitos adversos , Citarabina/efeitos adversos , Metotrexato/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Degeneração Combinada Subaguda/induzido quimicamente , Adolescente , Antimetabólitos Antineoplásicos/administração & dosagem , Citarabina/administração & dosagem , Evolução Fatal , Feminino , Humanos , Injeções Espinhais , Imageamento por Ressonância Magnética , Metotrexato/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Degeneração Combinada Subaguda/diagnóstico por imagemRESUMO
BACKGROUND: The first line treatment for patients < 40 years old with aplastic anemia (AA) is allogeneic HLA-identical sibling donor transplantation (SCT). Immunosuppressive therapy (IST) with a combination of Thymoglobuline (ATG) and cyclosporine is used for older patients or those without a donor. Five year overall survival (OS) for both therapies is > 70%. AIM: To report the experience with SCT and ATG for AA in a public hospital. PATIENTS AND METHODS: AA was diagnosed in 42 patients between 1998 and 2016, according to Camitta criteria. Thirty eight (90%) received treatment, 7 (18%) under 40 years old received SCT, and 31 (82%) IST. The rest were not treated. OS was calculated from date of diagnosis until last control, death or loss from follow up. RESULTS: Complete or partial hematologic response, was obtained in 71% and 58% of cases with SCT and IS, respectively. Five year OS was 71% and 55% with SCT and IST, respectively. No difference in response was observed between horse and rabbit ATG. CONCLUSIONS: SCT from an HLA-identical sibling donor had a high response rate and survival. IST instead, had a lower response and survival, due to an initial high mortality rate.
Assuntos
Anemia Aplástica/mortalidade , Anemia Aplástica/cirurgia , Soro Antilinfocitário/administração & dosagem , Ciclosporina/administração & dosagem , Imunossupressores/administração & dosagem , Transplante de Células-Tronco , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada , Hospitais Públicos , Humanos , Estimativa de Kaplan-Meier , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Fatores de Tempo , Adulto JovemRESUMO
BACKGROUND: Fatigue is one of the most common and distressing symptoms experienced by cancer patients. AIM: To validate the Brief Fatigue Inventory in people treated for hematological neoplasms. MATERIAL AND METHODS: In a cross-sectional study, the Brief Fatigue Inventory was answered by 122 patients aged 40 ± 14 years (50% women) treated for hematological neoplasms at an intensive hematological unit of a public hospital between July 2010 and July 2013. Socio-demographic and clinical parameters were obtained from their clinical records. RESULTS: Fatigue was present in nearly all patients (99.2%) in minor (50%), moderate (36.9%) or severe (12.3%) levels. The average fatigue score was 4.5 ± 1.9). The Brief Fatigue Inventory had a good internal consistency (Cronbachs alpha = 0.973) and proved to be one-dimensional (84.3% of the explained variance). Women reported that fatigue interfered more with enjoy-ment of life than men (p = 0.036). CONCLUSIONS: The Brief Fatigue Inventory is a reliable instrument that can be used in clinical practice. It allows a quick assessment of the level of fatigue. People treated for hematologic cancer have a high prevalence of fatigue.
Assuntos
Fadiga/diagnóstico , Fadiga/etiologia , Neoplasias Hematológicas/complicações , Adolescente , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Medição de Risco , Adulto JovemRESUMO
BACKGROUND: Intensified treatment of Philadelphia chromosome negative acute lymphoblastic leukemia (Ph(-)ALL) in adolescents by pediatric teams, with fve years disease free survival (DFS) rate of 65%, encouraged the use of intensified protocols in patients between 15 and 30 years, improving the DFS from 45% to 60-80%. The protocol LLA 15-30 for patients between 15 and 30 years with Ph(-)ALL, based on the Children's Oncology Group (COG) protocol AALL0232 resulting in a five years DFS of 78%, was started in 2007 by the PANDA national program. AIM: To report the results of the prospective cohort study evaluating the results of this protocol four years after its implementation. PATIENTS AND METHODS: Between January 2007 and December 2010, 68 Ph(-) ALL patients, aged between 15-30 years (75% males) were incorporated. Survival was evaluated using Kaplan-Meier and log-rank tests. RESULTS: Fifty percent of patients were of high risk. A complete response was achieved in 91%, early death occurred in 6% and induction failure in 3%. Median follow-up was 23 months. Overall survival, disease free survival and relapse rates at 35 months were 61.8, 67.5% and 31% respectively. CONCLUSIONS: LLA 15-30 protocol significantly improved three-year overall survival from 31 to 62%. The 20% difference observed with AALL0232 protocol is explained by the high rate of relapse. Improving provider and patient compliance with protocols may eliminate this gap.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Adolescente , Adulto , Asparaginase/administração & dosagem , Estudos de Coortes , Dexametasona/administração & dosagem , Feminino , Humanos , Masculino , Metotrexato/administração & dosagem , Prognóstico , Estudos Prospectivos , Resultado do Tratamento , Vincristina/administração & dosagem , Adulto JovemRESUMO
BACKGROUND: The current recommendations for treatment of patients with newly diagnosed acute promyelocytic leukemia (APL) include all-trans-retinoic acid (ATRA) and anthracycline based chemotherapy. AIM: To evaluate the results of the Chilean protocol following the LPA99 regimen of the Spanish PETHEMA group, except for the replacement of Idarubicin by Daunorubicin. PATIENTS AND METHODS: Induction consisted of Daunorubicin 45 mg/m² on days 2, 4, 6 and 8 plus ATRA 45 mg/m² daily until complete remission. Patients in complete remission (CR) received three monthly chemotherapy courses: Daunorubicin 45 mg/m²/d/4days i.v. and ATRA 45 mg/m²/d/15 days p.o. (course no. 1); Mitoxantrone 10 mg/m²/d/5 days i.v. and ATRA 45 mg/m²/d/15 days p.o. (course no. 2); Daunorubicin 60 mg/m²/d/ day 1 i.v. in the low risk group, and 1 and 2 in the intermediate-high risk groups and ATRA 45 mg/m²/d/15 days p.o. (course no. 3). Maintenance therapy consisted of mercaptopurine 90 mg/m²/d p.o., methotrexate 15 mg/m²/wk p.o. and, ATRA intermittently, 45 mg/m²/d p.o. for 15 days every three months. RESULTS: Between January 2000 and December 2005, 56 patients with newly diagnosed APL from 10 centers were enrolled. A total of 46 patients achieved CR (85%), 8 (15%) died of early complications, seven patients relapsed, with a 16% relapse risk at three years. The 5-year Kaplan-Meier estimates of overall survival and relapse-free survival were 64% and 84% respectively. CONCLUSIONS: These data indicate that this protocol has a good antileukemic effect but further reduction of early death and relapse, especially in the high risk group is needed.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Daunorrubicina/administração & dosagem , Leucemia Promielocítica Aguda/tratamento farmacológico , Tretinoína/administração & dosagem , Adolescente , Adulto , Idoso , Chile , Feminino , Humanos , Quimioterapia de Indução , Estimativa de Kaplan-Meier , Leucemia Promielocítica Aguda/diagnóstico , Leucemia Promielocítica Aguda/mortalidade , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is an effective therapy for hematological diseases such as lymphoma and multiple myeloma. In 2004, the Cancer Unit of the Ministry of Health incorporated the HSCT to the National Cancer Program in Adults. Until 2008 we purchased services to private institutions while implementing the national center for HSCT of adults in the Hospital del Salvador. AIM: To report the first ten HSCT conducted in this center. MATERIAL AND METHODS: All cases were approved by a national commission for adult HSCT. The entire process was carried out based on evidence-based protocols. RESULTS: Six patients with Hodgkin lymphoma, three with multiple myeloma and one with a diffuse large B cell lymphoma were transplanted. Age range was 19 to 48 years and five patients were male. An average of 2.2 aphereses per patient was required. The CD 34 stem cell collection was 5.06 x 10(6) x Kg. The conditioning regimes were BEAM (carmustine, etoposide, cytosine arabinoside, melphalan) and melphalan 200 according to the underlying disease. Seventy percent of the patients developed mild to moderate mucositis and 50% had febrile neutropenia, with good response to treatment. In two cases there was an association with influenza. The engraftment of neutrophils and platelets was achieved on day +10 and +11 respectively. At follow-up until day +100, there was no morbidity or mortality. CONCLUSIONS: These results confirm the quality standard that this intervention has achieved in our institution. The Chilean National Center for HSCT on Adults should be established as a public core care, teaching and research facility.
Assuntos
Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Doença de Hodgkin/terapia , Linfoma Difuso de Grandes Células B/terapia , Mieloma Múltiplo/terapia , Adulto , Chile , Feminino , Doença de Hodgkin/diagnóstico , Hospitalização , Hospitais Públicos , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Condicionamento Pré-Transplante/métodos , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: People living with human immunodeficiency virus (PLWH) still face high morbidity and mortality resulting from lymphoma. AIM: To describe a population of PLWH and lymphoma in a Chilean public hospital and compare the overall survival (OS) with a previously reported cohort from the same institution. METHODS: Retrospective single-center cohort study. All the patients diagnosed between 2010 and 2017 were included. Demographic and clinical variables were obtained from medical records. The overall survival (OS) was estimated in treated patients from diagnosis until death or October 2020. The OS was then compared with a cohort of patients diagnosed between 1992 and 2008. MAIN RESULTS: Eighty-four patients were included. The most common histological types were Burkitt´s lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), Hodgkin´s lymphoma (HL) and plasmablastic lymphoma (PBL) at 31%, 27%, 21% and 14%, respectively. The three-year OS for the whole cohort of BL, DLBCL, HL and PBL was 58.9%, 65.2%, 47.4%, 76.4% and 50%, respectively. Compared to the cohort of 1992 to 2008, a global increase in the OS was found after excluding HL and adjusting for age and clinical stage (HR 0.38, p = 0.002). However, when the main types were analyzed individually, the increase in the OS was statistically significant only in DLBCL (HR 0.29, p = 0.007). Most patients with DLBCL received CHOP chemotherapy, as in the previous cohort. CONCLUSION: The OS has improved in this population, despite no major changes in chemotherapy regimens, mainly due to the universal access to antiretroviral therapy.
RESUMO
A total of 5642 hematopoietic cell transplants (HCT) in 5445 patients (2196-40% allogeneic and 3249-60% autologous) were reported by 127 teams in 14 Latin American countries that answered the 2018 LABMT/WBMT Global Transplant Activity survey. The transplant rate (defined as the number of first transplants per 10 million inhabitants per year) was 85 (51 autologous and 34 allogeneic) in 2018. The main indications for allogeneic HCT were acute leukemias (60%), while plasma cell disorders and lymphomas were the most common conditions warranting autologous HCT (50 and 36%, respectively). In the allogeneic HCT, HLA-identical siblings were the main type of donor (44%) followed by related mismatched/haploidentical donors (32%). Peripheral blood stem cells were used in 98% of the autologous and in 64% of the allogeneic transplants. From 2012 to 2018, there was a 64% increase of reported HCT (54% in autologous and 80% in allogeneic). In the allogeneic setting, the most pronounced increase in donor type was observed in haploidentical relatives (from 94 procedures in 2012 up to 710 in 2018), surpassing unrelated donors as of 2017. Significant trends detected in Latin America include rising numbers of the procedures reported, a faster increase in allogeneic HCT compared with autologous HCT and a significant increase in family mismatched/haploidentical donors. The LABMT/WBMT activity survey provides useful data to understand the HCT activity and trends in Latin America.
Assuntos
Transplante de Células-Tronco Hematopoéticas , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , América Latina , Transplante Autólogo , Transplante Homólogo , Doadores não RelacionadosRESUMO
ABSTRACT Introduction: People living with human immunodeficiency virus (PLWH) still face high morbidity and mortality resulting from lymphoma. Aim: To describe a population of PLWH and lymphoma in a Chilean public hospital and compare the overall survival (OS) with a previously reported cohort from the same institution. Methods: Retrospective single-center cohort study. All the patients diagnosed between 2010 and 2017 were included. Demographic and clinical variables were obtained from medical records. The overall survival (OS) was estimated in treated patients from diagnosis until death or October 2020. The OS was then compared with a cohort of patients diagnosed between 1992 and 2008. Main Results: Eighty-four patients were included. The most common histological types were Burkitt's lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), Hodgkin's lymphoma (HL) and plasmablastic lymphoma (PBL) at 31%, 27%, 21% and 14%, respectively. The three-year OS for the whole cohort of BL, DLBCL, HL and PBL was 58.9%, 65.2%, 47.4%, 76.4% and 50%, respectively. Compared to the cohort of 1992 to 2008, a global increase in the OS was found after excluding HL and adjusting for age and clinical stage (HR 0.38, p = 0.002). However, when the main types were analyzed individually, the increase in the OS was statistically significant only in DLBCL (HR 0.29, p = 0.007). Most patients with DLBCL received CHOP chemotherapy, as in the previous cohort. Conclusion: The OS has improved in this population, despite no major changes in chemotherapy regimens, mainly due to the universal access to antiretroviral therapy.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Linfoma Difuso de Grandes Células B , Linfoma de Burkitt , Síndrome da Imunodeficiência Adquirida , Linfoma Plasmablástico , Prognóstico , Sobrevida , Doença de Hodgkin , Estudos de CoortesRESUMO
Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.
Assuntos
Humanos , Feminino , Idoso , Polineuropatias/etiologia , Polineuropatias/genética , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico , Neuropatias Amiloides Familiares/genética , Pré-Albumina/genética , MutaçãoRESUMO
Background: Before the advent of tyrosine kinase inhibitors (TKIs), patients with Philadelphia-positive Acute Lymphoblastic Leukemia (Ph+ALL) had a poor prognosis. The association of TKIs to intensive chemotherapy (CT) improved outcome. Aim: To evaluate results of an intensive CT protocol including TKI in a public hospital in Santiago, Chile. Material and Methods: All patients with Ph+ALL diagnosed between January 2010 and February 2019, and who met inclusion criteria for intensive CT, received the Ph+ALL national protocol in association with imatinib and were included in this analysis. Results: Thirty-five patients aged 15 to 59 years received treatment. Complete response (CR) was obtained in 97%. Measurable residual disease (MRD) was negative in 61% (19/31 evaluable cases) during follow-up, and 55% (16/29) were MRD (-) before three months. Relapse was observed in 13 cases. Three patients underwent allogeneic hematopoietic stem cell transplant (HSCT), two in CR1. The overall survival (OS) and event-free survival (EFS) at three years were 52 and 34%, respectively. In patients who achieved MRD negativity before three months, no statistically significant differences in OS (64 and 42% respectively, p = 0.15) or EFS (35 and 32% respectively, p = 0.37) were observed. Conclusions: The prognosis of Ph+ALL improved with the association of imatinib to intensive CT. MRD-negative status before three months in this series was not significantly associated with better outcomes. Our series suggests that the Ph+ALL national protocol associated to TKI is a therapeutic alternative with high CR and aceptable MRD (-) rates.
Assuntos
Humanos , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras B/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Neoplasia Residual/diagnóstico , Neoplasia Residual/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Mesilato de Imatinib/uso terapêuticoRESUMO
The severity and duration of post chemotherapy neutropenia were recognized during the 1960s as main predisposing factors for infections in cancer patients. At the beginning of the 70's a standard management approach for all febrile neutropenia (FN) episodes was proposed, based on hospitalization and intravenous empirical broad spectrum antibiotic therapy. Widespread use of this approach resulted in a significant reduction in mortality attributable to bacterial infections. During the last 10 to 15 years, reappraisal of this standard approach has been done by several research groups who question the benefit of treating all FN patients similarly without taking in to consideration differences in severity of the FN episodes. This reappraisal has led during the 1990s to the development of the concept of high and low risk FN episodes that has been the base for the adoption of selective therapies based on the risk categorization of the individual patient. The Chilean Infectious Diseases Society called upon two government National Programs responsible for the appropriate distribution of chemotherapeutic drugs to all pediatric and adults cancer patients within the public health system, and upon the Chilean Hematology Society for the development of a Consensus on Diagnosis, Treatment and Prevention of Infections during FN Episodes in Cancer patients. The need for this Consensus is based on two main aspects: the new approaches proposed during the past year for management of these episodes, and the increasing population of cancer patients receiving improved chemotherapeutic agents that has increased there survival possibilities as well as there possibility to suffer a FN episode. The topics discussed in this document are based on an updated systematic and analytic review of the medical literature including epidemiology, laboratory diagnostics, risk categorization, treatment and prophylaxis. National data was included when available in order to provide the healthcare personnel that take care of these patients with best evidence based recommendations adjusted to the Chilean reality.
Assuntos
Antineoplásicos/efeitos adversos , Febre , Neoplasias/tratamento farmacológico , Neutropenia , Infecções Oportunistas , Infecções Bacterianas/diagnóstico , Infecções Bacterianas/etiologia , Infecções Bacterianas/prevenção & controle , Febre/diagnóstico , Febre/etiologia , Febre/prevenção & controle , Humanos , Neoplasias/complicações , Neutropenia/diagnóstico , Neutropenia/etiologia , Neutropenia/prevenção & controle , Infecções Oportunistas/diagnóstico , Infecções Oportunistas/etiologia , Infecções Oportunistas/prevenção & controle , Medição de RiscoRESUMO
Intrathecal chemotherapy may be complicated with the development of myelopathies or toxic radiculopathies. This myeloradicular involvement, of toxic character, is unpredictable, since these patients have repeatedly received Intrathecal chemotherapy with the same drugs without apparent injury. The toxic effect should be mainly attributed to Cytarabine and not to methotrexate, since the central nervous system lacks Cytidine deaminase, the enzyme that degrades Cytarabine. We report two patients, an 18-year-old woman and a 16 years old male, who received systemic and intrathecal chemotherapy (methotrexate, cytarabine) for the treatment of an acute lymphoblastic leukemia and developed, in relation to this procedure, a spinal subacute combined degeneration. They had a proprioceptive and motor alteration of the lower extremities and neuroimaging showed selective rear and side spinal cord hyper intensity produced by central axonopathy. Two weeks later the woman developed a quadriplegia and the young man a flaccid paraplegia due to added root involvement.
Assuntos
Humanos , Feminino , Adolescente , Metotrexato/efeitos adversos , Citarabina/efeitos adversos , Degeneração Combinada Subaguda/induzido quimicamente , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Antimetabólitos Antineoplásicos/efeitos adversos , Injeções Espinhais , Imageamento por Ressonância Magnética , Metotrexato/administração & dosagem , Evolução Fatal , Citarabina/administração & dosagem , Degeneração Combinada Subaguda/diagnóstico por imagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Antimetabólitos Antineoplásicos/administração & dosagemRESUMO
Background: The first line treatment for patients < 40 years old with aplastic anemia (AA) is allogeneic HLA-identical sibling donor transplantation (SCT). Immunosuppressive therapy (IST) with a combination of Thymoglobuline (ATG) and cyclosporine is used for older patients or those without a donor. Five year overall survival (OS) for both therapies is > 70%. Aim: To report the experience with SCT and ATG for AA in a public hospital. Patients and Methods: AA was diagnosed in 42 patients between 1998 and 2016, according to Camitta criteria. Thirty eight (90%) received treatment, 7 (18%) under 40 years old received SCT, and 31 (82%) IST. The rest were not treated. OS was calculated from date of diagnosis until last control, death or loss from follow up. Results: Complete or partial hematologic response, was obtained in 71% and 58% of cases with SCT and IS, respectively. Five year OS was 71% and 55% with SCT and IST, respectively. No difference in response was observed between horse and rabbit ATG. Conclusions: SCT from an HLA-identical sibling donor had a high response rate and survival. IST instead, had a lower response and survival, due to an initial high mortality rate.
Assuntos
Humanos , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Ciclosporina/administração & dosagem , Transplante de Células-Tronco , Imunossupressores/administração & dosagem , Anemia Aplástica/cirurgia , Anemia Aplástica/mortalidade , Soro Antilinfocitário/administração & dosagem , Fatores de Tempo , Índice de Gravidade de Doença , Terapia Combinada , Estimativa de Kaplan-Meier , Hospitais PúblicosRESUMO
Background: Fatigue is one of the most common and distressing symptoms experienced by cancer patients. Aim: To validate the Brief Fatigue Inventory in people treated for hematological neoplasms. Material and Methods: In a cross-sectional study, the Brief Fatigue Inventory was answered by 122 patients aged 40 ± 14 years (50% women) treated for hematological neoplasms at an intensive hematological unit of a public hospital between July 2010 and July 2013. Socio-demographic and clinical parameters were obtained from their clinical records. Results: Fatigue was present in nearly all patients (99.2%) in minor (50%), moderate (36.9%) or severe (12.3%) levels. The average fatigue score was 4.5 ± 1.9). The Brief Fatigue Inventory had a good internal consistency (Cronbachs alpha = 0.973) and proved to be one-dimensional (84.3% of the explained variance). Women reported that fatigue interfered more with enjoyment of life than men (p = 0.036). Conclusions: The Brief Fatigue Inventory is a reliable instrument that can be used in clinical practice. It allows a quick assessment of the level of fatigue. People treated for hematologic cancer have a high prevalence of fatigue.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Neoplasias Hematológicas/complicações , Fadiga/diagnóstico , Fadiga/etiologia , Estudos Transversais , Medição de RiscoRESUMO
Background: Intensified treatment of Philadelphia chromosome negative acute lymphoblastic leukemia (Ph(-)ALL) in adolescents by pediatric teams, with fve years disease free survival (DFS) rate of 65%, encouraged the use of intensified protocols in patients between 15 and 30 years, improving the DFS from 45% to 60-80%. The protocol LLA 15-30 for patients between 15 and 30 years with Ph(-)ALL, based on the Children’s Oncology Group (COG) protocol AALL0232 resulting in a five years DFS of 78%, was started in 2007 by the PANDA national program. Aim: To report the results of the prospective cohort study evaluating the results of this protocol four years after its implementation. Patients and Methods: Between January 2007 and December 2010, 68 Ph(-) ALL patients, aged between 15-30 years (75% males) were incorporated. Survival was evaluated using Kaplan-Meier and log-rank tests. Results: Fifty percent of patients were of high risk. A complete response was achieved in 91%, early death occurred in 6% and induction failure in 3%. Median follow-up was 23 months. Overall survival, disease free survival and relapse rates at 35 months were 61.8, 67.5% and 31% respectively. Conclusions: LLA 15-30 protocol significantly improved three-year overall survival from 31 to 62%. The 20% difference observed with AALL0232 protocol is explained by the high rate of relapse. Improving provider and patient compliance with protocols may eliminate this gap.
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Adulto Jovem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Asparaginase/administração & dosagem , Estudos de Coortes , Dexametasona/administração & dosagem , Metotrexato/administração & dosagem , Prognóstico , Estudos Prospectivos , Resultado do Tratamento , Vincristina/administração & dosagemRESUMO
Background: The current recommendations for treatment of patients with newly diagnosed acute promyelocytic leukemia (APL) include all-trans-retinoic acid (ATRA) and anthracycline based chemotherapy. Aim: To evaluate the results of the Chilean protocol following the LPA99 regimen of the Spanish PETHEMA group, except for the replacement of Idarubicin by Daunorubicin. Patients and Methods: Induction consisted of Daunorubicin 45 mg/m² on days 2, 4, 6 and 8 plus ATRA 45 mg/m² daily until complete remission. Patients in complete remission (CR) received three monthly chemotherapy courses: Daunorubicin 45 mg/m²/d/4days i.v. and ATRA 45 mg/m²/d/15 days p.o. (course no. 1); Mitoxantrone 10 mg/m²/d/5 days i.v. and ATRA 45 mg/m²/d/15 days p.o. (course no. 2); Daunorubicin 60 mg/m²/d/ day 1 i.v. in the low risk group, and 1 and 2 in the intermediate-high risk groups and ATRA 45 mg/m²/d/15 days p.o. (course no. 3). Maintenance therapy consisted of mercaptopurine 90 mg/m²/d p.o., methotrexate 15 mg/m²/wk p.o. and, ATRA intermittently, 45 mg/m²/d p.o. for 15 days every three months. Results: Between January 2000 and December 2005, 56 patients with newly diagnosed APL from 10 centers were enrolled. A total of 46 patients achieved CR (85%), 8 (15%) died of early complications, seven patients relapsed, with a 16% relapse risk at three years. The 5-year Kaplan-Meier estimates of overall survival and relapse-free survival were 64% and 84% respectively. Conclusions: These data indicate that this protocol has a good antileukemic effect but further reduction of early death and relapse, especially in the high risk group is needed.
Assuntos
Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Daunorrubicina/administração & dosagem , Leucemia Promielocítica Aguda/tratamento farmacológico , Tretinoína/administração & dosagem , Chile , Quimioterapia de Indução , Estimativa de Kaplan-Meier , Leucemia Promielocítica Aguda/diagnóstico , Leucemia Promielocítica Aguda/mortalidadeRESUMO
El objetivo de esta investigación fue adaptar y validar para Chile el Cuestionario Multidimensional de Adaptación a la Enfermedad para Pacientes Onco-hematológicos (CMAE-OH) en la Unidad de Hematología Intensiva de un hospital general de Santiago de Chile. Este instrumento diseñado en España, es semiestructurado, consta de ítems cualitativos y cuantitativos, que proporcionan información para intervención preventiva, evalúan bienestar psicológico y adaptación a la enfermedad en los pacientes hemato-oncológicos. La metodología de trabajo fue la validación de contenido realizada por un panel de expertos. La muestra total fue de 42 pacientes hospitalizados, con 62 por ciento de hombres y 38 por ciento de mujeres, entre 15 y 56 años de edad. Se comparan los índices de una muestra de Santiago de Chile con una muestra de Madrid a través de análisis factoriales e índice de confiabilidad. Tanto en Chile como en España la variable información sobre la enfermedad, que mide el nivel de conocimiento del diagnóstico, tratamiento y pronóstico, es la variable que presenta más alta correlación con la adaptación a la enfermedad...
The purpose of this research was to adapt and validate the Cuestionario Multidimensional de Adaptación a la Enfermedad para Pacientes Onco hematológicos (CMAE-OH) in the Intensive Hematology Unit of a general hospital in Santiago de Chile. This instrument was designed in Spain as a semi-structured format with qualitative and quantitative items. These give information for preventive interventions, evaluate subjective well-being, and measure adaptation to hemato-oncologic diseases. The methodology consisted in content validation using an expert panel. The total simple were 42 inpatients, 62 percent males and 38 percent females, with ages between 15 and 56 years. The Santiago de Chile results are compared with a Madrid sample through a factor analysis. In Chile and in Spain the variable information of the disease, which measures the level of knowledge of the diagnosis, treatment and prognosis, is the variable that has the highest correlation with adaptation to the disease...