RESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. Material and Methods: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. Conclusions: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
RESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
Asunto(s)
Trastornos Mieloproliferativos , Policitemia Vera , Mielofibrosis Primaria , Trombocitemia Esencial , Anciano , Chile/epidemiología , Humanos , Janus Quinasa 2/genética , Mutación , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/epidemiología , Trastornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiología , Policitemia Vera/genética , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/epidemiología , Mielofibrosis Primaria/genética , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/genéticaRESUMEN
BACKGROUND: Philadelphia-negative myeloproliferative neoplasms (Ph-MPN) are chronic hematological disorders characterized by the overproduction of one or more mature myeloid blood cell lineages. Classical Ph-MPN are polycythemia vera (PV), essential thrombocytopenia (ET) and primary myelofibrosis (PMF). AIM: To assess the epidemiological, clinical and diagnostic characteristics of Ph-MPN in Chile. MATERIAL AND METHODS: Retrospective review of medical records of all patients referred as MPN from 2012 to 2017. Patients with (9;21) translocation were excluded. RESULTS: Data of 462 cases with a median age of 69 years from 10 public hospitals was reviewed. ET was the most frequently Ph-MNP found. The incidence of Ph-MPN was 1.5 x 100.000 cases. The JAK2 V617F mutation study was performed in 96% of patients and only 30% had a bone marrow biopsy. Thrombotic events were observed in 29% of patients. Bleeding events were observed in 7%. Five-year overall survival was 87%. CONCLUSIONS: ET is the most frequent Ph-MPN. The mean incidence was lower than reported in the literature, in part because of a sub diagnosis.
Asunto(s)
Trastornos Mieloproliferativos , Policitemia Vera , Mielofibrosis Primaria , Trombocitemia Esencial , Anciano , Chile/epidemiología , Humanos , Janus Quinasa 2/genética , Mutación , Trastornos Mieloproliferativos/diagnóstico , Trastornos Mieloproliferativos/epidemiología , Trastornos Mieloproliferativos/genética , Policitemia Vera/diagnóstico , Policitemia Vera/epidemiología , Policitemia Vera/genética , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/epidemiología , Mielofibrosis Primaria/genética , Trombocitemia Esencial/diagnóstico , Trombocitemia Esencial/genéticaRESUMEN
The aim of this study was to describe clinical and survival characteristics of transplant-eligible multiple myeloma (MM) patients in Latin America (LA), with a special focus on differences between public and private healthcare facilities. We included 1293 patients diagnosed between 2010 and 2018. A great disparity in outcomes and survival between both groups was observed. Late diagnosis and low access to adequate frontline therapy and ASCT in public institutions probably explain these differences. Patients treated with novel drug induction protocols, followed by autologous stem cell transplantation (ASCT) and maintenance, have similar overall survival compared to that published internationally.
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Trasplante de Células Madre Hematopoyéticas , Mieloma Múltiple , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Humanos , América Latina/epidemiología , Mieloma Múltiple/diagnóstico , Mieloma Múltiple/epidemiología , Mieloma Múltiple/terapia , Trasplante Autólogo , Resultado del TratamientoRESUMEN
Background The treatment of choice of newly diagnosed multiple myeloma (NDMM) is an induction with proteasome inhibitors followed autologous stem cell transplantation (HSCT). Since 2013, the treatment of these patients in the public system is based on CTD (cyclophosphamide, thalidomide, and dexamethasone). Aim To evaluate the response rates achieved with CTD, and the results of HSCT in patients with NDMM in the public setting. Material and Methods Data from patients considered as candidates for HSCT from different centers of the National Adult Antineoplastic Drug Program (PANDA, for its acronym in Spanish), diagnosed between 2013 and 2017, was analyzed. The response to treatment of first and second lines of treatment was evaluated, in addition to the results of HSCT. An optimal Response was defined as the sum of strict complete remission, complete remission and very good partial response (sCR, CR and VGPR). Results One hundred and seventy-seven patients were analyzed, 54% women, and 53% with IgG multiple myeloma. Information about the international staging system was retrieved in 127 patients (71%). Seventeen percent were ISS I, 22% in ISS II and 32% ISS III. CTD was used as first treatment in 106 patients (60%), and cyclophosphamide, bortezomib and dexamethasone (CyBorD) in 13 (7%). As first line, CTD had an overall response of 50.9%, and CyBorD of 76.9%. Thirty patients were treated with bortezomib as second line treatment. Forty patients (22%) underwent HSCT. The 5-year Overall Survival (OS) in transplanted patients and non-transplanted patients was 100 and 62% respectively (p < 0.01). Conclusions The response rate achieved by CTD in these patients is suboptimal. The response to CyBorD was better.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Trasplante de Células Madre Hematopoyéticas/métodos , Mieloma Múltiple/terapia , Factores de Tiempo , Trasplante Autólogo , Dexametasona/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica , Estudios Retrospectivos , Terapia Combinada , Supervivencia sin Enfermedad , Ciclofosfamida/administración & dosificación , Estimación de Kaplan-Meier , Bortezomib/administración & dosificación , Mieloma Múltiple/mortalidadRESUMEN
BACKGROUND: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). AIM: To report the features of patients with pPCL. MATERIAL AND METHODS: Review of databases of the Hematology Department and the Hematology laboratory. RESULTS: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. CONCLUSIONS: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
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Leucemia de Células Plasmáticas/epidemiología , Leucemia de Células Plasmáticas/genética , Adulto , Recuento de Células Sanguíneas , Calcio/sangre , Chile/epidemiología , Creatinina/sangre , Análisis Citogenético , Femenino , Citometría de Flujo/métodos , Humanos , Hibridación Fluorescente in Situ , Leucemia de Células Plasmáticas/patología , Leucemia de Células Plasmáticas/terapia , Masculino , Persona de Mediana Edad , Paraproteinemias/epidemiología , Paraproteinemias/genética , Paraproteinemias/patología , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
Background The treatment of choice of newly diagnosed multiple myeloma (NDMM) is an induction with proteasome inhibitors followed autologous stem cell transplantation (HSCT). Since 2013, the treatment of these patients in the public system is based on CTD (cyclophosphamide, thalidomide, and dexamethasone). Aim To evaluate the response rates achieved with CTD, and the results of HSCT in patients with NDMM in the public setting. Material and Methods Data from patients considered as candidates for HSCT from different centers of the National Adult Antineoplastic Drug Program (PANDA, for its acronym in Spanish), diagnosed between 2013 and 2017, was analyzed. The response to treatment of first and second lines of treatment was evaluated, in addition to the results of HSCT. An optimal Response was defined as the sum of strict complete remission, complete remission and very good partial response (sCR, CR and VGPR). Results One hundred and seventy-seven patients were analyzed, 54% women, and 53% with IgG multiple myeloma. Information about the international staging system was retrieved in 127 patients (71%). Seventeen percent were ISS I, 22% in ISS II and 32% ISS III. CTD was used as first treatment in 106 patients (60%), and cyclophosphamide, bortezomib and dexamethasone (CyBorD) in 13 (7%). As first line, CTD had an overall response of 50.9%, and CyBorD of 76.9%. Thirty patients were treated with bortezomib as second line treatment. Forty patients (22%) underwent HSCT. The 5-year Overall Survival (OS) in transplanted patients and non-transplanted patients was 100 and 62% respectively (p < 0.01). Conclusions The response rate achieved by CTD in these patients is suboptimal. The response to CyBorD was better.
Asunto(s)
Trasplante de Células Madre Hematopoyéticas/métodos , Mieloma Múltiple/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica , Bortezomib/administración & dosificación , Terapia Combinada , Ciclofosfamida/administración & dosificación , Dexametasona/administración & dosificación , Supervivencia sin Enfermedad , Femenino , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Mieloma Múltiple/mortalidad , Estudios Retrospectivos , Factores de Tiempo , Trasplante AutólogoRESUMEN
Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Leucemia de Células Plasmáticas/genética , Leucemia de Células Plasmáticas/epidemiología , Paraproteinemias/genética , Paraproteinemias/patología , Paraproteinemias/epidemiología , Recuento de Células Sanguíneas , Leucemia de Células Plasmáticas/patología , Leucemia de Células Plasmáticas/terapia , Análisis de Supervivencia , Chile/epidemiología , Calcio/sangre , Estudios Retrospectivos , Resultado del Tratamiento , Hibridación Fluorescente in Situ , Creatinina/sangre , Análisis Citogenético , Citometría de Flujo/métodosAsunto(s)
Inflamación/parasitología , Neurocisticercosis/parasitología , Taenia solium/metabolismo , Corticoesteroides/metabolismo , Corticoesteroides/uso terapéutico , Animales , Antihelmínticos/uso terapéutico , Biomarcadores/líquido cefalorraquídeo , Encéfalo , Quimioterapia Combinada , Enfermedades Transmitidas por los Alimentos/parasitología , Humanos , Inflamación/líquido cefalorraquídeo , Mediadores de Inflamación/metabolismo , Neurocisticercosis/líquido cefalorraquídeo , Neurocisticercosis/tratamiento farmacológico , Praziquantel/uso terapéutico , Medicina de Precisión/métodos , Receptores de Glucocorticoides/metabolismo , Receptores de Glucocorticoides/uso terapéutico , Porcinos/parasitología , Taenia solium/efectos de los fármacos , Resultado del TratamientoRESUMEN
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.
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Anemia Aplásica/mortalidad , Anemia Aplásica/cirugía , Suero Antilinfocítico/administración & dosificación , Ciclosporina/administración & dosificación , Inmunosupresores/administración & dosificación , Trasplante de Células Madre , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Terapia Combinada , Hospitales Públicos , Humanos , Estimación de Kaplan-Meier , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Factores de Tiempo , Adulto JovenRESUMEN
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.
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Humanos , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Adulto Joven , Ciclosporina/administración & dosificación , Trasplante de Células Madre , Inmunosupresores/administración & dosificación , Anemia Aplásica/cirugía , Anemia Aplásica/mortalidad , Suero Antilinfocítico/administración & dosificación , Factores de Tiempo , Índice de Severidad de la Enfermedad , Terapia Combinada , Estimación de Kaplan-Meier , Hospitales PúblicosRESUMEN
Se presenta el caso de un varón de 37 años, esquizofrénico en tratamiento con Clozapina. Es hospitalizado por neurotropenia severa y brusca, evolucionando rápidamente con compromiso de conciencia, agitación psicomotora, temperatura alta e hipotensión refractaria a aporte de volumen. Manejado en la Unidad de Cuidados Intensivos, se asiste a complicaciones renales y respiratorias, destacando la gran hipertonía muscular generalizada. Finalmente, el paciente fallece por paro cardio-respiratorio en asistolía. Se analiza el cuadro de hipertermia y se le relaciona con el uso de neurolépticos, describiéndose la toxicidad por clozapina, que produce neutropenia. Se plantea el diagnóstico diferencial entre el síndrome neuroléptico maligno, la hipertermia maligna y el síndrome serotoninérgico. Por último, se describe el manejo médico de la hipertermia.