The 5T's of health disparities in multiple myeloma in Latin America.

PURPOSE OF REVIEW: Health disparities or inequities, which are defined as differences in the quality of medical and healthcare between populations among racial, ethnic, and socioeconomic groups, have been validated in numerous studies as determinants of survival and quality of life in different diseases, including cancer.Compared to the improvement in overall survival in developed countries in relation to better diagnostic opportunity and novel therapeutic approaches, low and middle-income countries still have significant barriers in accessing these therapies.The potential impact of overcoming these barriers is immense and offers hope for better outcomes. RECENT FINDINGS: There is great heterogeneity in the diagnostic and therapeutic approach to multiple myeloma among different latitudes. Latin America has been characterized by important limitations in using the best technologies currently available in developed countries. SUMMARY: Overcoming health disparities in multiple myeloma in LMICs could help improve survival and quality of life outcomes. Likewise, it is necessary to increase the representation of the Latin population in clinical studies, primarily in our region.

Follicular Lymphoma in Chile in the Adult Public Cancer Program: The Impact of Chemoimmunotherapy.

BACKGROUND: Follicular lymphoma (FL) is the most common indolent non-Hodgkin lymphoma (NHL) in the United States and Europe. However, data on FL from Latin America are scant. AIMS: This study aims at better understand the clinical features, treatment patterns and outcomes of patients with FL in Chile. Of special interest was to evaluate POD24 as an adverse marker. METHODS AND RESULTS: We collected retrospective data from 722 patients 15 years or older diagnosed with FL and treated in 17 cancer centers in Chile between 2000 and 2019. Time to first treatment (TTFT), progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Cox proportional-hazard regression models were fitted to investigate prognostic factor. The median age at diagnosis was 62 with a female predominance (63%); 73% of patients had advance stage disease and 68% had bone marrow involvement; 63% had intermediate or high FLIPI scores. The 1-year TTFT rate was 96%, and 30% of patients received chemoimmunotherapy. Adding rituximab to chemotherapy was associated with a higher complete response (69% vs. 60%; p < 0.001) and superior median OS (16 vs. 8 years; p < 0.001). Patients who experience POD24 had an inferior median OS (2.4 vs. 15 years). CONCLUSION: Our study shows a female predominance in patients with FL in Chile and confirms superior response and survival outcomes with adding rituximab to chemotherapy. Our study also confirms a poor OS in patients who experience POD24.

The Latin-American Experience in POEMS Syndrome: A Study of the GELAMM (Grupo de Estudio Latinoamericano de Mieloma Múltiple).

INTRODUCTION: POEMS syndrome is a rare paraneoplastic syndrome caused by an underlying plasma cell disorder. The acronym refers to the following features: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal paraproteinemia, and skin changes. METHODS: The study was conducted at 24 hematological centers across 8 Latin-American countries. The study included a total of 46 patients {median age was 52 years (interquartile range [IQR]: 42-61.5), 30 males and 16 females} fulfilling the POEMS syndrome criteria diagnosed over a period of 12 years (January 1, 2011, through July 31, 2023). Epidemiological and clinical data were collected in an ad hoc database sent to the members of GELAMM, as well as the Kolmogorov-Smirnov test and Kaplan-Meier estimates. RESULTS: All patients had polyneuropathy and monoclonal gammopathy; 89% had bone marrow plasma cell infiltration, 33% had sclerotic bone lesions. Only 10 patients underwent vascular endothelial growth factor (VEGF) testing in plasma samples. The paraproteinemia was IgG λ in 32% and IgA λ in 30%. 59% patients presented with cutaneous changes, mainly hyperpigmentation, 54% had organomegaly, and 74% endocrinopathy. The median interval from symptom onset to diagnosis was 7.7 months (IQR: 4.0-12.6). 69% of patients received a single line of treatment. The median follow-up period was 25 months (IQR: 9.37-52.0) and the 2-year overall survival rate was 100%. All patients who underwent transplantation (43%) are alive, with a median follow-up of 45.62 months (IQR: 15.46-70). CONCLUSION: This study investigates POEMS syndrome in Latin America and presents an initial overview of the disease in the region. VEGF usage is recommended for accurate diagnosis, but only 7 hematology centers in the region used it. Survival rate in Latin America is comparable with those observed internationally.

Multiple myeloma in Latin America: A systematic review.

The incidence of multiple myeloma (MM) has surged globally, particularly in Latin American countries, and is attributable to an aging population and increased life expectancy. This systematic review analyzes the epidemiology, patient characteristics, and treatment outcomes for MM in selected Latin American countries: Brazil, Mexico, Colombia, Argentina, Chile, Peru, and Uruguay. PubMed and the Latin American and Caribbean Health Sciences Literature (LILACS), conference abstracts (between June 2019 and June 2022), and GLOBOCAN registry (January 2010 to June 2022) were electronically searched. Qualitative analysis employed the Joanna Briggs Institute's critical appraisal tool. Among the 586 screened articles, 26 met the inclusion criteria. The participants' median age ranged from 54 to 67 years. GLOBOCAN data revealed that for MM, Brazil and Uruguay had the highest and lowest incidence, 5-year prevalence, and mortality, respectively. Immunoglobulin G was the most common subtype detected. Stage III was frequently diagnosed. Though many approved drugs are available and bispecific antibodies hold promise as a future therapy, limited access, especially for CAR-T cell-based therapy remains a concern. The incidence of MM is increasing in Latin America. Resource constraints and costs hinder access to novel drugs and regimens. Understanding disease patterns and patient characteristics is vital to improve MM management in these countries.

Reference interval of free light chains ratio in patients with end-stage renal disease on chronic hemodialysis.

Not available.

Psychometric properties of the Chilean version of the quality of life questionnaire for multiple myeloma.

OBJECTIVES: To evaluate the internal consistency and construct validity of the QLQ-MY20 for assessing the quality of life in multiple myeloma survivors in Chile. METHODS: This was a cross-sectional study conducted between March 2020 and December 2022. It involved 118 individuals from two public hospitals. The QLQ-C30 and QLQ-MY20 questionnaires were used. Internal consistency was assessed using Cronbach's alpha(α), and construct validity was evaluated through hypothesis testing (Mann-Whitney and Spearman correlation). RESULTS: The average age of participants was 67.2 years (SD=9.2). Internal consistency for the complete scale was α=0.779, for the "disease symptoms" dimension α=0.671, for the "side effects of treatments" dimension α=0.538, and for the "future perspective" dimension α=0.670. Four of the five construct validity hypotheses were confirmed: women, individuals with worse performance status, those with pain, and those with worse fatigue showed more symptoms. CONCLUSIONS: The Chilean version of the QLQ-MY20 demonstrates adequate internal consistency and construct validity.

[Impact of bariatric surgery on maternal-fetal outcomes during pregnancy compared to non-operated obese women]. / Impacto de la cirugía bariátrica en los resultados materno-fetales de la gestación en comparación con mujeres con obesidad no operadas.

Introduction: Objective: the aim of our study was to assess the effect of bariatric surgery on obstetric outcomes. Material and methods: a retrospective cohort study was conducted, including 54 pregnancies post-bariatric surgery and 219 pregnancies in non-operated obese women, from December 2018 to January 2023. Various maternal and obstetric characteristics were evaluated, ranging from baseline data to antepartum, intrapartum, postpartum, and neonatal outcomes. Results: bariatric surgery showed a significant 69 % reduction in the risk of gestational diabetes (GDM) (p = 0.045) and a 63.1 % reduction in cesarean sections (p = 0.014), but also a notable increase in the risk of miscarriage (3.5 times more, p = 0.046) and intrauterine growth restriction (35 times more, p = 0.009). Bariatric surgery was associated with a significant prolongation of postpartum hospital stay (7.5 times more, p = 0.001) and a decrease in the average weight of the newborn (213.71 g, p = 0.006). Conclusion: pregnancy after bariatric surgery presents benefits, such as a reduced risk of gestational diabetes (GDM) and cesarean section, but also presents challenges, such as an increased risk of miscarriage and intrauterine growth restriction (IUGR). These results highlight the importance of specialized obstetric care to optimize maternal-fetal outcomes in pregnant women with a history of bariatric surgery.

Introducción: Objetivo: el objetivo de nuestro estudio es evaluar el efecto de la cirugía bariátrica en los resultados obstétricos. Material y métodos: se realizó un estudio de cohortes retrospectivo que incluyó 47 gestaciones postcirugía bariátrica y 219 gestaciones en mujeres con obesidad no operadas, reclutadas en el Hospital Álvaro Cunqueiro de Vigo (Galicia, noroeste de España), en el periodo comprendido entre diciembre de 2018 y enero de 2023. Se evaluaron diversas características, tanto maternas como obstétricas, abarcando desde datos basales hasta los resultados anteparto, intraparto, posparto y neonatales. Resultados: la cirugía bariátrica mostró una significativa reducción del riesgo de diabetes gestacional (DMG) en un 69 % (p = 0,045) y del riesgo de cesáreas en un 63,1 % (p = 0,014), pero también un aumento notable del riesgo de aborto (3,5 veces más, p = 0,046) y del retraso del crecimiento intrauterino (35 veces más, p = 0,009). La cirugía bariátrica se asoció a una prolongación significativa de la estancia hospitalaria posparto (7,5 veces más, p = 0,001) y a una disminución del peso promedio del recién nacido (213,71 g, p = 0,006). Conclusión: la gestación postcirugía bariátrica presenta beneficios, como una reducción del riesgo de diabetes gestacional (DMG) y de cesárea, pero presenta desafíos, como un mayor riesgo de aborto y un retraso del crecimiento intrauterino (CIR). Estos resultados resaltan la importancia de una atención obstétrica especializada para optimizar los resultados materno-fetales en las gestantes con antecedentes de cirugía bariátrica.

Outcomes of Modified Mayo Stage IIIa and IIIb Cardiac Light-Chain Amyloidosis: Real-World Experience in Clinical Characteristics and Treatment-67 Patients Multicenter Analysis.

Light-chain amyloidosis (AL) is a rare multisystem disorder characterized by the deposition of misfolded amyloid fibrils derived from monoclonal immunoglobulin light chains in various organs. One of the most common organs involved in AL is the heart, with 50-70% of patients clinically symptomatic at diagnosis. We conducted a multi-center, retrospective analysis of 67 patients diagnosed between July 2012 and August 2022 with the European 2012 modification of Mayo 2004 stage III cardiac AL. The most important factors identified in the univariate Cox analysis contributing to a longer OS included Eastern Cooperative Oncology Group performance status (ECOG PS) ≤ 1, New York Heart Association functional classification (NYHA FC) ≤ 2, the use of autologous stem cell transplantation (ASCT) after induction treatment, achieving a hematological response (≥very good partial response) and cardiac (≥partial response) response after first-line treatment. The most important prognostic factors with the most significant impact on OS improvement in patients with modified Mayo stage III cardiac AL identified by multivariate Cox analysis are ECOG PS ≤ 1, NYHA FC ≤ 2, and achieving hematological response ≥ VGPR and cardiac response ≥ PR after first-line treatment.

Psychometric properties of the Chilean version of the quality of life questionnaire for multiple myeloma / Propriedades psicométricas da versão chilena do questionário de qualidade de vida para mieloma múltiplo / Propiedades psicométricas de la versión chilena del cuestionario de calidad de vida para mieloma múltiple

ABSTRACT Objectives: To evaluate the internal consistency and construct validity of the QLQ-MY20 for assessing the quality of life in multiple myeloma survivors in Chile. Methods: This was a cross-sectional study conducted between March 2020 and December 2022. It involved 118 individuals from two public hospitals. The QLQ-C30 and QLQ-MY20 questionnaires were used. Internal consistency was assessed using Cronbach's alpha(α), and construct validity was evaluated through hypothesis testing (Mann-Whitney and Spearman correlation). Results: The average age of participants was 67.2 years (SD=9.2). Internal consistency for the complete scale was α=0.779, for the "disease symptoms" dimension α=0.671, for the "side effects of treatments" dimension α=0.538, and for the "future perspective" dimension α=0.670. Four of the five construct validity hypotheses were confirmed: women, individuals with worse performance status, those with pain, and those with worse fatigue showed more symptoms. Conclusions: The Chilean version of the QLQ-MY20 demonstrates adequate internal consistency and construct validity.

RESUMO Objetivos: Avaliar consistência interna e validade de construto do QLQ-MY20 para avaliação da qualidade de vida em sobreviventes de mieloma múltiplo no Chile. Métodos: Estudo transversal, realizado entre março de 2020 e dezembro de 2022. Participaram 118 pessoas de dois hospitais públicos. Foram utilizados questionários QLQ-C30 e QLQ-MY20. A consistência interna foi avaliada com alfa de Cronbach(α) e a validade de construto através de testes de hipóteses (Mann Whitney e correlação de Spearman). Resultados: A idade média dos participantes era de 67,2 (DP=9,2) anos. Consistência interna para escala completa (α=0,779), dimensão "sintomas da doença" (α=0,671), dimensão "efeitos colaterais dos tratamentos" (α=0,538) e dimensão "perspectiva de futuro" (α=0,670). Quatro das cinco hipóteses de validade de construto foram confirmadas: as mulheres apresentaram mais sintomas, assim como pessoas com pior estado de desempenho, com dor e com maior fadiga. Conclusões: A versão chilena do QLQ-MY20 apresenta consistência interna adequada e validade de construto.

RESUMEN Objetivos: Evaluar consistencia interna y validez de constructo del QLQ-MY20 para valoración de calidad de vida en sobrevivientes de mieloma múltiple en Chile. Métodos: Estudio transversal, realizado entre marzo 2020 y diciembre 2022. Participaron 118 personas de dos hospitales públicos. Se utilizaron los cuestionarios QLQ-C30 y QLQ-MY20. Fueron evaluadas la consistencia interna con alfa de Cronbach (α) y validez de constructo mediante pruebas de hipótesis (Mann Whitney y correlación de Spearman). Resultados: El promedio de edad de los participantes era 67,2 (DE=9,2) años. Consistencia interna para escala completa (α=0,779), dimensión "síntomas de la enfermedad" (α=0,671), dimensión "efectos secundarios de los tratamientos" (α=0,538) y dimensión "perspectiva de futuro" (α=0,670). Se comprobaron cuatro de las cinco hipótesis de la validez de constructo: presentaron más síntomas las mujeres, personas con peor performance estatus, con dolor y con peor fatiga. Conclusiones: La versión chilena del QLQ-MY20 presenta adecuada consistencia interna y validez de constructo.

Patients Age 40 Years and Younger With Multiple Myeloma Have the Same Prognosis as Older Patients: An Analysis of Real-World Patients' Evidence From Latin America.

PURPOSE: Multiple myeloma (MM) is a highly heterogeneous, incurable disease most frequently diagnosed in the elderly. Therefore, data on clinical characteristics and outcomes in the very young population are scarce. PATIENTS AND METHODS: We analyzed clinical characteristics, response to treatment, and survival in 103 patients with newly diagnosed MM age 40 years or younger compared with 256 patients age 41-50 years and 957 patients age 51 years or older. RESULTS: There were no statistical differences in sex, isotype, International Scoring System, renal involvement, hypercalcemia, anemia, dialysis, bony lesions, extramedullary disease, and lactate dehydrogenase (LDH). The most used regimen in young patients was cyclophosphamide, bortezomib, dexamethasone, followed by cyclophosphamide, thalidomide, dexamethasone and bortezomib, thalidomide, dexamethasone. Of the patients age 40 years or younger, only 53% received autologous stem-cell transplant (ASCT) and 71.1% received maintenance. There were no differences in overall survival (OS) in the three patient cohorts. In the multivariate analysis, only high LDH, high cytogenetic risk, and ASCT were statistically associated with survival. CONCLUSION: In conclusion, younger patients with MM in Latin America have similar clinical characteristics, responses, and OS compared with the elderly.

An elevated HScore is associated with poor clinical outcomes in COVID-19.

BACKGROUND: Patients with Coronavirus Disease 2019 (COVID-19) frequently experience a hyperinflammatory syndrome leading to unfavorable outcomes. This condition resembles Secondary Hemophagocytic Lymphohistiocytosis (sHLH) described in neoplastic, rheumatic and other infectious diseases. A scoring system (HScore) that evaluates underlying immunosuppression, temperature, organomegaly, cytopenias, ferritin, triglycerides, fibrinogen and AST was validated for sHLH, and recently proposed to evaluate hyperinflammation in COVID-19. AIM: To assess the presence of sHLH among patients with COVID-19 admitted for hospitalization and to evaluate Hscore as a prognostic tool for poor outcomes. MATERIAL AND METHODS: One hundred forty-three patients aged 21-100 years (64% males) admitted because of COVID-19 were enrolled in a prospective study. HScore was calculated within 72 hours admission. The incidence of sHLH during hospitalization was evaluated. Additionally, the relationship between a HScore ≥ 130 points and either the requirement of mechanical ventilation or 60-days mortality was explored. RESULTS: The median HScore was 96 (33-169). A SHLH was diagnosed in one patient (incidence 0.7%), whose HScore was 169. After adjusting for age, sex, comorbidities and obesity, HScore ≥ 130 was independently associated with the composite clinical outcome (Hazard rartio 2.13, p = 0.022). CONCLUSIONS: sHLH is not frequent among COVID-19 patients. HScore can be useful to predict the risk for poor outcomes.

Outcome of Second Primary Malignancies Developing in Multiple Myeloma Patients.

BACKGROUND: There is an increased risk of second primary malignancies (SMPs) in patients with multiple myeloma (MM). This multinational 'real-world' retrospective study analyzed the characteristics and outcomes of MM patients that developed SPMs. RESULTS: 165 patients were analyzed: 62.4% males; 8.5% with a prior cancer; 113 with solid SPMs, mainly ≥stage 2; and 52 with hematological SPM (hemato-SPM), mainly MDS/AML. Patients with hemato-SPM were younger (p = 0.05) and more frequently had a prior AutoHCT (p = 0.012). The time to SPM was shorter in the older (>65 years) and more heavily pretreated patients. One hundred patients were actively treated at the time of SPM detection. Treatment was discontinued in 52, substituted with another anti-MM therapy in 15, and continued in 33 patients. Treatment discontinuation was predominant in the patients diagnosed with hemato-SPM (76%). The median OS following SPM detection was 8.5 months, and the main cause of death was SPM. A poor ECOG status predicted a shorter OS (PS 3 vs. 0, HR = 5.74, 2.32-14.21, p < 0.001), whereas a normal hemoglobin level (HR = 0.43, 0.19-0.95, p = 0.037) predicted longer OS. CONCLUSIONS: With the continuing improvement in OS, a higher proportion of MM patients might develop SPM. The OS following SPM diagnosis is poor; hence, frequent surveillance and early detection are imperative to improve outcomes.

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.

PRO-CTCAE reveals under-recognition of dermatologic symptom burden in hospitalized cancer patients.

PURPOSE: Dermatologic adverse events (dAEs) occur frequently in hospitalized patients and can significantly reduce quality of life. Physicians grade dAEs using the Common Terminology Criteria of Adverse Events (CTCAE). However, they often underestimate symptom frequency and severity. The patient-reported outcomes (PRO) version of the CTCAE (PRO-CTCAE) was developed to assess symptoms from the patient's perspective. In this study, we assessed the patient-reported burden of dAEs via the PRO-CTCAE questionnaire and compared results with dAE assessment by treating oncologists and dermatologists. METHODS: Patients admitted to Memorial Sloan Kettering Cancer Center from 6/1/2018 to 4/30/2019 and received a dermatology consultation were eligible. Once enrolled, participants completed a PRO-CTCAE questionnaire on 14 dermatologic symptoms. CTCAE grades assigned by oncology and dermatology were obtained from clinical notes, and kappa statistics were calculated to evaluate the level of agreement between physician and patient evaluations. RESULTS: A total of 100 patients (mean age 59.4, 55% male) were prospectively enrolled. The most common patient-reported dAEs were rash (72%), swelling (67%), pruritus (64%), bruising (53%), and hives (37%). Oncologists and dermatologists underreported dAEs except for rash (median kappa values 0.3 [0.02-0.84] and 0.32 [0.02-0.87], respectively). Oncologists and dermatologists were concordant with each other's documented assessment of dAEs (median kappa value 0.985 [0.55-1]). CONCLUSION: Oncology patient-reported dAEs in a tertiary academic oncologic referral center were under-recognized by providers. PRO-CTCAE may be a useful tool to optimize inpatient dermatologic care for cancer patients by detecting and allowing management of patient-reported dAEs.

An elevated HScore is associated with poor clinical outcomes in COVID-19 / Un HScore elevado se asocia a mal pronóstico en pacientes con COVID-19

BACKGROUND: Patients with Coronavirus Disease 2019 (COVID-19) frequently experience a hyperinflammatory syndrome leading to unfavorable outcomes. This condition resembles Secondary Hemophagocytic Lymphohistiocytosis (sHLH) described in neoplastic, rheumatic and other infectious diseases. A scoring system (HScore) that evaluates underlying immunosuppression, temperature, organomegaly, cytopenias, ferritin, triglycerides, fibrinogen and AST was validated for sHLH, and recently proposed to evaluate hyperinflammation in COVID-19. AIM: To assess the presence of sHLH among patients with COVID-19 admitted for hospitalization and to evaluate Hscore as a prognostic tool for poor outcomes. MATERIAL AND METHODS: One hundred forty-three patients aged 21-100 years (64% males) admitted because of COVID-19 were enrolled in a prospective study. HScore was calculated within 72 hours admission. The incidence of sHLH during hospitalization was evaluated. Additionally, the relationship between a HScore ≥ 130 points and either the requirement of mechanical ventilation or 60-days mortality was explored. RESULTS: The median HScore was 96 (33-169). A SHLH was diagnosed in one patient (incidence 0.7%), whose HScore was 169. After adjusting for age, sex, comorbidities and obesity, HScore ≥ 130 was independently associated with the composite clinical outcome (Hazard rartio 2.13, p = 0.022). CONCLUSIONS: sHLH is not frequent among COVID-19 patients. HScore can be useful to predict the risk for poor outcomes.

ANTECEDENTES: Los pacientes con Enfermedad por Coronavirus 2019 (COVID-19), experimentan frecuentemente un síndrome hiperinflamatorio que lleva a resultados desfavorables. Esta situación se asemeja al Síndrome Hemofagocítico Secundario (sHLH) descrito en enfermedades neoplásicas, reumatológicas y por otros agentes infecciosos. Un sistema simple de puntaje (HScore) que evalúa inmunosupresión, temperatura organomegalia, citopenias, ferritina, triglicéridos, fibrinógeno y AST ha sido validado para el diagnóstico de sHLH y ha sido propuesto recientemente para evaluar la hiperinflamación en COVID-19. OBJETIVO: Medir la frecuencia de sHLH entre pacientes con COVID-19 hospitalizados, y evaluar a HScore como una herramienta pronóstica. MATERIAL Y MÉTODOS: Ciento cuarenta y tres pacientes de 21 a 100 años (64% hombres) fueron ingresados en este estudio de cohorte prospectivo, unicéntrico. Se calculó HScore dentro de las primeras 72 horas desde el ingreso, y se midió la incidencia de sHLH durante la hospitalización. Adicionalmente, se evaluó la relación entre HScore ≥ 130 puntos y un desenlace compuesto de ventilación mecánica o muerte a los 60 días. RESULTADOS: La mediana de HScore fue 96 (33-169) puntos. Un paciente fue diagnosticado con sHLH (incidencia 0,7%). Luego de ajustar por edad, sexo, comorbilidades y obesidad, un HScore ≥ 130 se asoció de manera independiente con el desenlace compuesto. CONCLUSIONES: El sHLH no es frecuente en los pacientes con COVID-19. El uso de HScore puede ser útil para predecir el riesgo de desenlaces clínicos desfavorables.

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 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.

[Characterization of patients with Philadelphia negative myeloproliferative neoplasms and concomitant monoclonal gammopathies: Just coincidence?] / Caracterización de pacientes con Neoplasias mieloproliferativas Philadelphia negativas y Gammapatias monoclonales concomitantes ¿Sólo coincidencia?

Philadelphia negative myeloproliferative neoplasms [MPN Ph (-)] and monoclonal gammopathies (MG) stem from different hematopoietic progenitor lines. The association between both has a frequency between 3 to 14%, and it has been associated with a higher risk of thrombosis. This study aimed to describe the clinical characteristics of patients with both entities at our center. METHODS: Retrospective observational study of case series. The MPN Ph (-) database of our center between 2015 and 2020 was consulted. The clinical records were reviewed, obtaining demographic, clinical, and management determinants. Descriptive statistical analysis was performed. RESULTS: Among 144 patients, 6 patients diagnosed with MG and MPN were found, all of them female. The median age was 71 years at diagnosis of MPN Ph (-) and 70 years at diagnosis of MG. Two were diagnosed concomitantly with both pathologies, in 2 the MG preceded the MPN, and in 2 the MPN was previously diagnosed. No patient has progressed to acute leukemia or myelofibrosis. Regarding the treatments received, all received Hydroxicarbamide, some with aspirin and one with anticoagulation. Of the MGs, one patient with solitary bone plasmacytoma progressed to multiple myeloma, requiring treatment after 2 years. Two had a thrombotic event, both arterials. CONCLUSION: The observed MG frequency of 4% was similar to what was expected for the age of the patients. Although it is noteworthy that 2 had thrombotic events, further studies are needed to evaluate this association.