Experiencia clínica de uso de Brentuximab-vedotin como terapia de linfoma de Hodgkin recaído o refractario en Hospital Guillermo Grant Benavente, Concepción, Chile / Clinical experience of using brentuximab-vedotin as therapy in relapsed/refractory Hodgkin's lymphoma at Hospital Guillermo Grant Benavente, Concepción, Chile

INTRODUCCIÓN: El linfoma de Hodgkin es una neoplasia de células B de buen pronóstico, pero con mala respuesta a quimioterapia en casos refractarios o recaídas. Brentuximab vedotin es un anticuerpo monoclonal antiCD30 aprobado para su uso en estos casos. El presente trabajo tiene como objetivo describir la experiencia clínica de los pacientes tratados con brentuximab vedotin bajo la modalidad de acceso expandido. MATERIAL Y MÉTODOS: Estudio retrospectivo sobre información clínica de pacientes diagnosticados de linfoma de Hodgkin refractario o en recaída y tratados con brentuximab-vedotin en el Hospital Regional de Concepción en el período 2015-2021. RESULTADOS: Se identificaron 7 pacientes, 5/7 de sexo masculino, con una mediana de edad de 35 años (21-50). Cinco casos fueron celularidad mixta y 2 esclerosis nodular. Cuatro estaban en etapa II, 1/7 en etapa III y 3/7 en etapa IV. La mediana de líneas de tratamiento previas fue 4 (3-5) y en 2 casos la recaída fue postrasplante. En 6/7 casos se empleó como inducción y en un caso se empleó como mantención postransplante autólogo de médula ósea. La administración fue ambulatoria por vía periférica con una mediana de dosis 150 mg y 10 ciclos. En un caso se necesitó ajuste de dosis por toxicidad. Tres de 6 pacientes lograron remisión completa y fueron a trasplante autólogo de médula ósea. CONCLUSIONES: brentuximab vedotin es un medicamento ambulatorio de baja toxicidad que puede optimizar el tratamiento de pacientes con linfoma de Hodgkin recaído-refractario.

INTRODUCTION: Hodgkin's lymphoma is a B-cell neoplasm with a good prognosis but a poor response to chemotherapy in refractory or relapsed cases. Brentuximab-vedotin is an anti-CD30 monoclonal antibody approved for use in these cases. This study aims to describe the clinical experience of patients treated with brentuximab-vedotin through expanded access modality. MATERIALS AND METHODS: A retrospective study on clinical information of patients diagnosed with refractory or relapsed Hodgkin's lymphoma treated with brentuximab-vedotin at the Regional Hospital of Concepción in the period 2015-2021. RESULTS: 7 patients were identified, 5/7 male, with a median age of 35 years (21-50). Five cases were mixed cellularity, and two were nodular sclerosis. Four were in stage II, 1/7 in stage III, and 3/7 in stage IV. The median number of previous treatment lines was 4 (3-5), and the relapse was post-transplantation in two cases. In 6/7 cases, brentuximab-vedotin was used as induction, and in one case, it was used as post-autologous bone marrow transplant maintenance. The administration was outpatient via a peripheral route with a median dose of 150 mg and ten cycles. In one case, dose adjustment was required due to toxicity. Three out of 6 patients achieved complete remission and underwent autologous stem cell transplantation. CONCLUSION: brentuximab-vedotin is an outpatient medication with low toxicity that can optimize the treatment of patients with relapsed-refractory Hodgkin's lymphoma.

Características clínicas de neoplasia de células dendríticas plasmocitoides blásticas en Chile: reporte de 10 casos

Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una hemopatía maligna poco frecuente y de mal pronóstico, con reportes de casos aislados en la realidad nacional. Produce compromiso cutáneo y de médula ósea y frecuentemente es confundida con otras patologías al diagnóstico. El presente trabajo tiene como objetivo describir las características clínicas de 10 pacientes diagnosticados en centros asistenciales chilenos. Material y Métodos: Se obtuvo en forma retrospectiva información clínica e inmunofenotípica de pacientes diagnosticados de NCDPB en los centros participantes en el periodo 2013-2021. Resultados: Se identificaron 10 pacientes, el 80% de sexo masculino, con una mediana de edad de 66 años (15-81). Los diagnósticos iniciales de derivación más frecuentes fueron linfoma T (4/10) y leucemia aguda mieloblástica (3/10). La mayoría presentó afección cutánea (7/10) y compromiso de médula (7/10) y en menor frecuencia adenopatías, esplenomegalia y hepatomegalia. En el hemograma se observó anemia y leucopenia, con blastos en frotis en 5/10. Se indicó CHOP en 8/10 casos con remisión en 5/8 y en un caso HyperCVAD seguido de trasplante alogénico de médula ósea. La mediana de sobrevida fue de 10 meses (IC 95% 4,2-15,8 meses) con 9/10 fallecidos. Se documentó recaída en sistema nervioso central en 2 casos. Conclusiones: La NCDPB es una patología poco frecuente que se presenta en la realidad nacional de forma similar a lo descrito en la literatura. Es susceptible de responder a quimioterapia inicial asociada a terapia intratecal.

Background: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor with a dismal prognosis, with isolated case reports in Chile. The BPDCN can present skin and bone marrow compromise, and its diagnosis is frequently confused with other pathologies. This study aimed to evaluate the clinical and immunophenotypical features of BPDCN in the Chilean population. Methods: We performed a retrospective study from 2013 to 2021 in clinical records of 2 public Chilean referral hospitals, including ten patients, 80% male, with a median age of 66 years (15-81). Results: The most frequent initial referral diagnoses were T-cell lymphoma (4/10) and acute myeloblastic leukemia (3/10). Seven patients presented skin and bone marrow involvement; we found a lower frequency of adenopathies (5/10), splenomegaly (2/10), and hepatomegaly (2/10). The complete blood count revealed anemia and leukopenia, with blasts in 5/10. Nine patients received induction therapy. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) was indicated in 8/10 cases with remission in 5/8, and 1 patient received HyerCVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone, methotrexate, cytarabine) and an allogeneic bone marrow transplant. The median survival was 10 months (95% CI 4.2-15.8 months) with 9/10 deaths. Relapse in the central nervous system was documented in 2 cases. Conclusions: Our study found that BPDCN, a rare pathology in the Chilean population, shows a similar clinical presentation compared to previous studies. It is susceptible to respond to initial systemic and intrathecal chemotherapy.

Flow cytometry-detected changes in megaloblastic anemia secondary to cobalamin deficiency.

Introduction: Megaloblastic anemias secondary to Vitamin B12 deficiency are a group of pathologies produced by defective nuclear DNA synthesis. Objective: To describe the maturation alterations found in hematopoietic precursors of the bone marrow in a series of patients with megaloblastic anemia. Methods: Were included patients attended at the Regional Hospital of Concepción with bone marrow samples sent for the study of cytopenia by flow cytometry whose final diagnosis was megaloblastic anemia. The immunophenotype was performed with CD45, CD34, CD117, HLA-DR, markers of neutrophil (CD13, CD11b, CD10, CD16) and/or erythroblast (CD105, CD71, CD36) maturation. Results: From the flow cytometry laboratory database, 8 patients with megaloblastic anemia were identified, and myelodysplastic syndromes (n=9) and normal or reactive bone marrow (n=10) were used as controls. 44% were men, with a median age of 58 years. Megaloblastic anemia was associated with a higher proportion of size and complexity of erythroid and myeloid progenitors compared to lymphocytes compared to controls. The total percentage of erythroblasts and the proportion of CD34+ myeloid cells associated with erythroid lineage was higher in megaloblastic anemia, associated with a maturation arrest in the CD105+ precursor stage (69% vs 19% and 23%, p<0.001). The heterogeneity of CD36 and CD71 in megaloblastic anemia was similar to myelodysplastic syndromes. Conclusions: Megaloblastic anemia produces a heterogeneous involvement of hematopoiesis, characterized by a greater size and cellular complexity of precursors of the neutrophil and erythroid series and a maturation arrest of the erythroblasts.

Introducción: Anemias megaloblásticas secundarias a la deficiencia de vitamina B12 son patologías producidas por una síntesis defectuosa del ADN nuclear. Objetivo: Describir las alteraciones madurativas encontradas en precursores hematopoyéticos de la médula ósea de una serie de pacientes con anemia megaloblástica. Métodos: Se incluyeron pacientes atendidos en el Hospital Regional de Concepción con muestras de médula ósea enviadas para estudio de citopenias por citometría de flujo cuyo diagnóstico fue anemia megaloblástica. El inmunofenotipo se realizó con CD45, CD34, CD117, HLA-DR, marcadores de maduración de serie de neutrófilo (CD13, CD11b, CD10, CD16) y/o eritroblasto (CD105, CD71, CD36). Resultados: Se identificaron 8 pacientes con anemia megaloblástica y como controles se utilizaron síndromes mielodisplásicos (n=9) y médula ósea normal o reactiva (n=10). El 44% eran hombres, con una mediana de edad de 58 años. La anemia megaloblástica se asoció con una mayor proporción de tamaño y complejidad de progenitores eritroides y mieloides con respecto de los linfocitos en comparación a los controles. El porcentaje total de eritroblastos y la proporción de células mieloides CD34+ comprometidas con el linaje eritroide fue mayor en anemia megaloblástica, asociado a una parada madurativa en la etapa de precursor CD105+ (69% vs 19% y 23%, p <0.001). La heterogeneidad de CD36 y CD71 en anemia megaloblástica fue similar a los síndromes mielodisplásicos. Conclusiones: la anemia megaloblástica produce una afectación heterogénea de la hematopoyesis, caracterizada por un mayor tamaño y complejidad celulares de precursores de la serie neutrófilo y eritroide y una detención madurativa de los eritroblastos.

Mutations in the bZip region of the CEBPA gene: A novel prognostic factor in patients with acute myeloid leukemia.

BACKGROUND: Hematopoiesis, the process of blood cell formation involves on a complex network of transcription factors. Among them, the CCAAT-enhancer-binding protein alpha (CEBPA) plays a crucial role in maintaining the balance between myeloid proliferation and differentiation. Imbalances in this network can lead to disrupted differentiation and contribute to the development of malignant diseases. AIM: Understanding of disease development and explore potential therapeutic strategies for hematological disorders associated CEPBA gen. MATERIALS AND METHODS: The research involved a comprehensive analysis of CEBPA gene mutations in the context of acute myeloid leukemia (AML). This encompassed a thorough exploration of point mutations and double mutations in AML patients. RESULTS: In the context of acute myeloid leukemia (AML), mutations in the CEBPA gene, especially point mutations, are frequently observed. A significant number of AML patients present with double mutations in CEBPA, which have been linked to a more favorable prognosis in terms of overall survival and event-free survival. These patients also tend to exhibit enhanced responsiveness to treatment. DISCUSSION: Unraveling the intricate interplay of transcription factors, particularly CEBPA, holds significant implications for decoding the mechanisms governing hematopoiesis. This understanding offers a potential avenue for deciphering disease development and devising novel therapeutic strategies for hematological disorders. CONCLUSION: The findings underscore that CEBPA mutations correlate with enhanced overall survival and event-free survival, with relevance to those presenting within the bZip framework. This knowledge may contribute to advancing personalized treatments for hematological conditions.

Flow cytometry-detected changes in megaloblastic anemia secondary to cobalamin deficiency

Introduction: Megaloblastic anemias secondary to Vitamin B12 deficiency are a group of pathologies produced by defective nuclear DNA synthesis. Objective: To describe the maturation alterations found in hematopoietic precursors of the bone marrow in a series of patients with megaloblastic anemia. Methods: Were included patients attended at the Regional Hospital of Concepción with bone marrow samples sent for the study of cytopenia by flow cytometry whose final diagnosis was megaloblastic anemia. The immunophenotype was performed with CD45, CD34, CD117, HLA-DR, markers of neutrophil (CD13, CD11b, CD10, CD16) and/or erythroblast (CD105, CD71, CD36) maturation. Results: From the flow cytometry laboratory database, 8 patients with megaloblastic anemia were identified, and myelodysplastic syndromes (n=9) and normal or reactive bone marrow (n=10) were used as controls. 44% were men, with a median age of 58 years. Megaloblastic anemia was associated with a higher proportion of size and complexity of erythroid and myeloid progenitors compared to lymphocytes compared to controls. The total percentage of erythroblasts and the proportion of CD34+ myeloid cells associated with erythroid lineage was higher in megaloblastic anemia, associated with a maturation arrest in the CD105+ precursor stage (69% vs 19% and 23%, p<0.001). The heterogeneity of CD36 and CD71 in megaloblastic anemia was similar to myelodysplastic syndromes. Conclusions: Megaloblastic anemia produces a heterogeneous involvement of hematopoiesis, characterized by a greater size and cellular complexity of precursors of the neutrophil and erythroid series and a maturation arrest of the erythroblasts.

Introducción: Anemias megaloblásticas secundarias a la deficiencia de vitamina B12 son patologías producidas por una síntesis defectuosa del ADN nuclear. Objetivo: Describir las alteraciones madurativas encontradas en precursores hematopoyéticos de la médula ósea de una serie de pacientes con anemia megaloblástica. Métodos: Se incluyeron pacientes atendidos en el Hospital Regional de Concepción con muestras de médula ósea enviadas para estudio de citopenias por citometría de flujo cuyo diagnóstico fue anemia megaloblástica. El inmunofenotipo se realizó con CD45, CD34, CD117, HLA-DR, marcadores de maduración de serie de neutrófilo (CD13, CD11b, CD10, CD16) y/o eritroblasto (CD105, CD71, CD36). Resultados: Se identificaron 8 pacientes con anemia megaloblástica y como controles se utilizaron síndromes mielodisplásicos (n=9) y médula ósea normal o reactiva (n=10). El 44% eran hombres, con una mediana de edad de 58 años. La anemia megaloblástica se asoció con una mayor proporción de tamaño y complejidad de progenitores eritroides y mieloides con respecto de los linfocitos en comparación a los controles. El porcentaje total de eritroblastos y la proporción de células mieloides CD34+ comprometidas con el linaje eritroide fue mayor en anemia megaloblástica, asociado a una parada madurativa en la etapa de precursor CD105+ (69% vs 19% y 23%, p <0.001). La heterogeneidad de CD36 y CD71 en anemia megaloblástica fue similar a los síndromes mielodisplásicos. Conclusiones: la anemia megaloblástica produce una afectación heterogénea de la hematopoyesis, caracterizada por un mayor tamaño y complejidad celulares de precursores de la serie neutrófilo y eritroide y una detención madurativa de los eritroblastos.

La citometría de flujo aumenta la proporción de muestras valorables en líquido cefalorraquídeo con recuento celular normal en hemopatías malignas / Flow cytometry increases the proportion of valuable samples in cerebrospinal fluid with normal cell count in malignant blood diseases

INTRODUCCIÓN: El compromiso del líquido cefalorraquídeo (LCR) en hemopatías malignas es un marcador de mal pronóstico y es habitualmente estudiado por citometría de flujo o citología. Ocasionalmente, las muestras de LCR oligocelulares (≤ 5 céls/dL) pueden ser consideradas como no aptas para diagnóstico por la baja cantidad de eventos. Objetivo: Evaluar la proporción de muestras reportadas como valorables para diagnóstico obtenidas por citometría y citología en muestras de LCR oligocelular. Material y Métodos: Se seleccionaron 169 muestras de LCR oligocelular correspondientes a 115 pacientes con hemopatías malignas. Las muestras fueron obtenidas mediante punción lumbar en tubos acondicionados con EDTA y preservante celular (Transfix®). El inmunofenotipo se realizó con panel de 8 colores, 55 (32%) de las cuales se hizo con panel para pequeñas muestras (SST). En todos los casos se incluyó CD14 para identificación de monocitos y CD3 para linfocitos T. La adquisición se realizó en citómetro FACSCantoII® y el análisis en software Infinicyt®. Resultados: La proporción de muestras valorables fue mayor en citometría en comparación con la citología (98% vs 61%, p < 0,000). En la mayoría se identificaron linfocitos T (98%) y/o monocitos (90%). En las muestras con SST, la cantidad de eventos obtenida fue menor en muestras con < de 1 mL (140 vs 556, p < 0,001) y se logró identificar una mediana de 3 poblaciones celulares. Conclusión: La citometría proporciona una mayor cantidad de muestras valorables en los LCR paucicelulares en relación con la citología en muestras de LCR enviadas para estudio de compromiso de LCR por hemopatías malignas.

BACKGROUND: The alteration of cerebrospinal fluid (CSF) in hematologic neoplasms is a poor prognostic marker. The characteristics of CSF are usually analyzed by flow cytometry or cytology. However, paucicellular CSF samples (≤5 cells/dL) can sometimes be considered unsuitable for analysis due to the low number of events. Objective: To evaluate the proportion of samples reported as suitable for analysis obtained by cytometry (FCM) and cytology in paucicellular CSF samples. Material and Methods: 169 samples ofpaucicellular CSF corresponding to 115 patients with hematologic neoplasms were selected. The samples were obtained by lumbar puncture in tubes conditioned with EDTA and Transfix®. We characterized the immunophenotype ofCSF samples with an 8-color panel, and 55 samples (32%) were in a small sample tube (SST). In all cases, monocytes were identified by CD14 labeling and T lymphocytes by CD3 labeling. The acquisition was carried out in a FACSCantoII® cytometer, and the analysis was performed using Infinicyt® software. Results: The proportion of samples suitable for analysis was higher in FCM compared to cytology (98% vs 61%, p < 0.000). We identified the presence of T lymphocytes and/or monocytes in most samples (98% and 90%, respectively). In the SST samples, the number of events recorded in low-volume samples (< 1 mL) was lower than in samples with higher volume (140 vs 556, p < 0.001), with a median of identification of 3 cell populations. Conclusion: FCM allows the analysis of a higher proportion ofpaucicellular CSF samples than cytology in hematologic neoplasms study.

Primary plasma cell leukemia in Latin America: demographic, clinical, and prognostic characteristics. A study of GELAMM group.

Primary plasma cell leukemia (pPCL) is an infrequent and aggressive plasma cell disorder. The prognosis is still very poor, and the optimal treatment remains to be established. A retrospective, multicentric, international observational study was performed. Patients from 9 countries of Latin America (LATAM) with a diagnosis of pPCL between 2012 and 2020 were included. 72 patients were included. Treatment was based on thalidomide in 15%, proteasome inhibitors (PI)-based triplets in 38% and chemotherapy plus IMIDs and/or PI in 29%. The mortality rate at 3 months was 30%. The median overall survival (OS) was 18 months. In the multivariate analysis, frontline PI-based triplets, chemotherapy plus IMIDs and/or PI therapy, and maintenance were independent factors of better OS. In conclusion, the OS of pPCL is still poor in LATAM, with high early mortality. PI triplets, chemotherapy plus IMIDs, and/or PI and maintenance therapy were associated with improved survival.

[Clinical experience of using brentuximab-vedotin as therapy in relapsed/refractory Hodgkin's lymphoma at Hospital Guillermo Grant Benavente, Concepción, Chile]. / Experiencia clínica de uso de Brentuximab-vedotin como terapia de linfoma de Hodgkin recaído o refractario en Hospital Guillermo Grant Benavente, Concepción, Chile.

INTRODUCTION: Hodgkin's lymphoma is a B-cell neoplasm with a good prognosis but a poor response to chemotherapy in refractory or relapsed cases. Brentuximab-vedotin is an anti-CD30 monoclonal antibody approved for use in these cases. This study aims to describe the clinical experience of patients treated with brentuximab-vedotin through expanded access modality. MATERIALS AND METHODS: A retrospective study on clinical information of patients diagnosed with refractory or relapsed Hodgkin's lymphoma treated with brentuximab-vedotin at the Regional Hospital of Concepción in the period 2015-2021. RESULTS: 7 patients were identified, 5/7 male, with a median age of 35 years (21-50). Five cases were mixed cellularity, and two were nodular sclerosis. Four were in stage II, 1/7 in stage III, and 3/7 in stage IV. The median number of previous treatment lines was 4 (3-5), and the relapse was post-transplantation in two cases. In 6/7 cases, brentuximab-vedotin was used as induction, and in one case, it was used as post-autologous bone marrow transplant maintenance. The administration was outpatient via a peripheral route with a median dose of 150 mg and ten cycles. In one case, dose adjustment was required due to toxicity. Three out of 6 patients achieved complete remission and underwent autologous stem cell transplantation. CONCLUSION: brentuximab-vedotin is an outpatient medication with low toxicity that can optimize the treatment of patients with relapsed-refractory Hodgkin's lymphoma.

[Clinical features of blastic plasmacytoid dendritic neoplasm in Chile: report of 10 cases]. / Características clínicas de neoplasia de células dendríticas plasmocitoides blásticas en Chile: reporte de 10 casos.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor with a dismal prognosis, with isolated case reports in Chile. The BPDCN can present skin and bone marrow compromise, and its diagnosis is frequently confused with other pathologies. This study aimed to evaluate the clinical and immunophenotypical features of BPDCN in the Chilean population. METHODS: We performed a retrospective study from 2013 to 2021 in clinical records of 2 public Chilean referral hospitals, including ten patients, 80% male, with a median age of 66 years (15-81). RESULTS: The most frequent initial referral diagnoses were T-cell lymphoma (4/10) and acute myeloblastic leukemia (3/10). Seven patients presented skin and bone marrow involvement; we found a lower frequency of adenopathies (5/10), splenomegaly (2/10), and hepatomegaly (2/10). The complete blood count revealed anemia and leukopenia, with blasts in 5/10. Nine patients received induction therapy. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) was indicated in 8/10 cases with remission in 5/8, and 1 patient received HyerCVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone, methotrexate, cytarabine) and an allogeneic bone marrow transplant. The median survival was 10 months (95% CI 4.2-15.8 months) with 9/10 deaths. Relapse in the central nervous system was documented in 2 cases. CONCLUSIONS: Our study found that BPDCN, a rare pathology in the Chilean population, shows a similar clinical presentation compared to previous studies. It is susceptible to respond to initial systemic and intrathecal chemotherapy.

[Flow cytometry increases the proportion of valuable samples in cerebrospinal fluid with normal cell count in malignant blood diseases]. / La citometría de flujo aumenta la proporción de muestras valorables en líquido cefalorraquídeo con recuento celular normal en hemopatías malignas.

BACKGROUND: The alteration of cerebrospinal fluid (CSF) in hematologic neoplasms is a poor prognostic marker. The characteristics of CSF are usually analyzed by flow cytometry or cytology. However, paucicellular CSF samples (≤5 cells/dL) can sometimes be considered unsuitable for analysis due to the low number of events. OBJECTIVE: To evaluate the proportion of samples reported as suitable for analysis obtained by cytometry (FCM) and cytology in paucicellular CSF samples. MATERIAL AND METHODS: 169 samples ofpaucicellular CSF corresponding to 115 patients with hematologic neoplasms were selected. The samples were obtained by lumbar puncture in tubes conditioned with EDTA and Transfix®. We characterized the immunophenotype ofCSF samples with an 8-color panel, and 55 samples (32%) were in a small sample tube (SST). In all cases, monocytes were identified by CD14 labeling and T lymphocytes by CD3 labeling. The acquisition was carried out in a FACSCantoII® cytometer, and the analysis was performed using Infinicyt® software. RESULTS: The proportion of samples suitable for analysis was higher in FCM compared to cytology (98% vs 61%, p < 0.000). We identified the presence of T lymphocytes and/or monocytes in most samples (98% and 90%, respectively). In the SST samples, the number of events recorded in low-volume samples (< 1 mL) was lower than in samples with higher volume (140 vs 556, p < 0.001), with a median of identification of 3 cell populations. CONCLUSION: FCM allows the analysis of a higher proportion ofpaucicellular CSF samples than cytology in hematologic neoplasms study.

Características y desenlace clínico de 30 pacientes con hemopatías malignas y COVID-19 en un hospital público regional / Features of COVID-19 infection among patients with hematological malignancies

BACKGROUND: COVID-19 infection can be especially severe in certain risk populations such as patients with hematologic malignancies. Aim: To describe the characteristics and clinical outcomes of a population of patients with hematologic malignancies and COVID-19. MATERIAL AND METHODS: Review of medical records of patients with COVID-19 and hematologic malignancies, treated in Hematology Service of a regional hospital in Chile, between April 1 and October 30, 2020. Demographic characteristics, chronic comorbidities and clinical characteristics related to the underlying disease and COVID-19 infection were recorded. Results: Thirty adults aged 17 to 73 years (67% men) with COVID-19 confirmed by RT-PCR, were evaluated. Forty percent had comorbidities, mainly hypertension (30%), obesity (27%) and diabetes (10%). Two thirds of cases came from a nosocomial outbreak and 77% were symptomatic. Half of the cases had mild disease and 20% required mechanical ventilation. Five patients (17%) died from COVID 19. Female sex, the presence of comorbidities and obesity were more common among deceased patients. Only 1 of 5 deceased patients were in complete remission. No differences were found in the mean survival according to requirement for intubation or the presence of complete remission. CONCLUSIONS: This population with hematologic malignancies and COVID-19 had special characteristics leading to a greater fatality rate which, in this series, does not increase with the use of mechanical ventilation.

[Features of COVID-19 infection among patients with hematological malignancies]. / Características y desenlace clínico de 30 pacientes con hemopatías malignas y COVID-19 en un hospital público regional.

BACKGROUND: COVID-19 infection can be especially severe in certain risk populations such as patients with hematologic malignancies. AIM: To describe the characteristics and clinical outcomes of a population of patients with hematologic malignancies and COVID-19. MATERIAL AND METHODS: Review of medical records of patients with COVID-19 and hematologic malignancies, treated in Hematology Service of a regional hospital in Chile, between April 1 and October 30, 2020. Demographic characteristics, chronic comorbidities and clinical characteristics related to the underlying disease and COVID-19 infection were recorded. RESULTS: Thirty adults aged 17 to 73 years (67% men) with COVID-19 confirmed by RT-PCR, were evaluated. Forty percent had comorbidities, mainly hypertension (30%), obesity (27%) and diabetes (10%). Two thirds of cases came from a nosocomial outbreak and 77% were symptomatic. Half of the cases had mild disease and 20% required mechanical ventilation. Five patients (17%) died from COVID 19. Female sex, the presence of comorbidities and obesity were more common among deceased patients. Only 1 of 5 deceased patients were in complete remission. No differences were found in the mean survival according to requirement for intubation or the presence of complete remission. CONCLUSIONS: This population with hematologic malignancies and COVID-19 had special characteristics leading to a greater fatality rate which, in this series, does not increase with the use of mechanical ventilation.

Different outcomes for transplant-eligible newly diagnosed multiple myeloma patients in Latin America according to the public versus private management: a GELAMM study.

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.

Mieloma múltiple en Chile: Respuesta a tratamiento en pacientes con mieloma múltiple elegibles para trasplante autólogo de progenitores hematopoyéticos / Response rates to first-line treatment in eligible patients to autologous stem transplantation in Chile

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.

[Response rates to first-line treatment in eligible patients to autologous stem transplantation in Chile]. / Mieloma múltiple en Chile: Respuesta a tratamiento en pacientes con mieloma múltiple elegibles para trasplante autólogo de progenitores hematopoyéticos.

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.

Efectividad de Rituximab en el tratamiento de citopenias autoinmunes refractarias en adultos / Effectiveness of Rituximab in the treatment of cytopenias refractory autoimmune in adults

The term autoimmune cytopenias is referred to a heterogeneous group of diseases characterized by a reduced peripheral blood cell counts in one or more cellular series, because an immunological disorder. The first line therapy is steroids, followed by splenectomy or immunesupressant therapy in non-responders. Rituximab is an anti CD20 monoclonal antibody used as a third line in refractory patients or as an alternative to splenectomy. We present a retrospective study of nine patients with autoimmune cytopenias treated in a public hospital setting with rituximab. Five patients with the diagnosis of inmune thrombocytopenic purpura received it, all of them achieved hematological response (4 complete and one partial). The median time to the best response was 6 weeks, staying in this category after 6 months of follow up. Four patients with autoimmune hemolytic anemia received rituximab, three of them achieving partial response and one was lost from follow up. No severe adverse effects related to rituximab were registered.

[Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: Report of one case]. / Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central: Caso clínico.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 year-old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.

Neoplasia de células dendríticas plasmocitoides blásticas con respuesta inicial a quimioterapia y recaída en sistema nervioso central: caso clínico / Blastic plasmacytoid dendritic cell neoplasm with complete clinical remission with chemotherapy and central nervous system relapse: report of one case

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses. We report a 24 year-old male with BPDCN, initially diagnosed and treated as non-Hodgkin CD4+ T-cell lymphoma, with initial complete remission who evolved with early central nervous system relapse. A second attempt of chemotherapy failed and the patient died two months later.

Minimal residual disease evaluation by flow cytometry is a complementary tool to cytogenetics for treatment decisions in acute myeloid leukaemia.

The clinical utility of minimal residual disease (MRD) analysis in acute myeloid leukaemia (AML) is not yet defined. We analysed the prognostic impact of MRD level at complete remision after induction therapy using multiparameter flow cytometry in 306 non-APL AML patients. First, we validated the prognostic value of MRD-thresholds we have previously proposed (≥ 0.1%; ≥ 0.01-0.1%; and <0.01), with a 5-year RFS of 38%, 50% and 71%, respectively (p=0.002). Cytogenetics is the most relevant prognosis factor in AML, however intermediate risk cytogenetics represent a grey zone that require other biomarkers for risk stratification, and we show that MRD evaluation discriminate three prognostic subgroups (p=0.03). Also, MRD assessments yielded relevant information on favourable and adverse cytogenetics, since patients with favourable cytogenetics and high MRD levels have poor prognosis and patients with adverse cytogenetics but undetectable MRD overcomes the adverse prognosis. Interestingly, in patients with intermediate or high MRD levels, intensification with transplant improved the outcome as compared with chemotherapy, while the type of intensification therapy did not influenced the outcome of patients with low MRD levels. Multivariate analysis revealed age, MRD and cytogenetics as independent variables. Moreover, a scoring system, easy in clinical practice, was generated based on MRD level and cytogenetics.