The Latin American experience in POEMS syndrome. A study of the GELAMM (Grupo de Estudios Latinoamericanos 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 (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; 89% had monoclonal gammopathy, 33% had sclerotic bone lesions. Only ten 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.

Therapeutic Choices in Patients with Ph-Positive Chronic Myelogenous Leukemia In Mexico in the Era of Tyrosine Kinase Inhibitors: Stem Cell Transplantation or Tyrosine Kinase Inhibitors? Fifteen Years Later.

Background: Chronic myelogenous leukemia is a neoplastic proliferation of the granulocytic series. In Mexico, chronic myelogenous leukemia accounts for approximately 10% of all leukemias. Tyrosine-kinase inhibitors are considered front-line therapy in high-income countries, whereas allogeneic hematopoietic stem cell transplantation is a recognized therapeutic approach, mainly in low- and middle-income countries. Objective: To analyze the overall survival of persons with chronic myelogenous leukemia who have received tyrosine-kinase inhibitors or allogeneic hematopoietic stem cell transplantation in a medical center, since 1994, and briefly discuss the current indications of these treatments in the tyrosine-kinase inhibitors era. Methods: We retrospectively analyzed all patients with a diagnosis of chronic myelogenous leukemia treated in a medical center between 1994 and 2023; subsets of individuals who received an allogeneic hematopoietic stem cell transplantation or tyrosine-kinase inhibitors therapy as first-line treatment were analyzed. Results: 60 persons with chronic myelogenous leukemia were treated with allogeneic hematopoietic stem cell transplantation or tyrosine-kinase inhibitors: 35 received an allogeneic hematopoietic stem cell transplantation, whereas 25 were given tyrosine-kinase inhibitors. All patients who underwent an allogeneic hematopoietic stem cell transplantation engrafted successfully, and the procedure was completed on an outpatient basis in most cases (29/35). The median survival in allogeneic hematopoietic stem cell transplantation was 78.3 months (CI 95%: 0-205.6) and in persons given tyrosine-kinase inhibitors the median was not reached. Conclusion: Tyrosine-kinase inhibitors were significantly superior to allogeneic hematopoietic stem cell transplantation in prolonging the overall survival of persons with chronic myelogenous leukemia in our single institution experience.

Epidemiology of childhood acute leukemias in marginalized populations of the central-south region of Mexico: results from a population-based registry.

Introduction: Acute leukemias (AL) are the main types of cancer in children worldwide. In Mexico, they represent one of the main causes of death in children under 20 years of age. Most of the studies on the incidence of AL in Mexico have been developed in the urban context of Greater Mexico City and no previous studies have been conducted in the central-south of the country through a population-based study. The aim of the present work was to identify the general and specific incidence rates of pediatric AL in three states of the south-central region of Mexico considered as some of the marginalized populations of Mexico (Puebla, Tlaxcala, and Oaxaca). Methods: A population-based study was conducted. Children aged less than 20 years, resident in these states, and newly diagnosed with AL in public/private hospitals during the period 2021-2022 were identified. Crude incidence rates (cIR), standardized incidence rates (ASIRw), and incidence rates by state subregions (ASIRsr) were calculated. Rates were calculated using the direct and indirect method and reported per million children under 20 years of age. In addition, specific rates were calculated by age group, sex, leukemia subtype, and immunophenotype. Results: A total of 388 cases with AL were registered. In the three states, the ASIRw for AL was 51.5 cases per million (0-14 years); in Puebla, it was 53.2, Tlaxcala 54.7, and Oaxaca de 47.7. In the age group between 0-19 years, the ASIRw were 44.3, 46.4, 48.2, and 49.6, in Puebla, Tlaxcala, and Oaxaca, respectively. B-cell acute lymphoblastic leukemia was the most common subtype across the three states. Conclusion: The incidence of childhood AL in the central-south region of Mexico is within the range of rates reported in other populations of Latin American origin. Two incidence peaks were identified for lymphoblastic and myeloid leukemias. In addition, differences in the incidence of the disease were observed among state subregions which could be attributed to social factors linked to the ethnic origin of the inhabitants. Nonetheless, this hypothesis requires further investigation.

Prevalence and Consequences of a Delayed Diagnosis in Multiple Myeloma: A Single Institution Experience.

BACKGROUND: Multiple myeloma (MM) is a disease with unspecific initial symptoms which may lead into a delay in the diagnosis, seemingly increasing the risk of complications and in turn reducing the overall survival (OS). OBJECTIVE: To analyze the consequences of a delayed diagnosis of MM in both the OS and the progression-free survival (PFS) of the patients in a single center in México. METHODS: The study included patients with MM who were diagnosed at Clínica Ruiz, Puebla, México, between 1983 and 2022. According to the time elapsed between the onset of symptoms to the establishment of the definite diagnosis of MM, 4 groups were constructed: 1) Less than 3 months, 2) 3-6 months, 3) 6-12 months, and 4) More than 12 months. RESULTS: About 136 patients had a complete clinical record and at least a 3-month follow up period. A delay in the diagnosis of MM (more than 3 months from the onset of symptoms) was recorded in 92/136 persons (68%). The median follow-up for the whole group was 24.7 months, median OS was 131.4 months, whereas median PFS was 85.4 months. There was a significant trend for being in earlier stages of the disease and being diagnosed within 3 months from the onset of symptoms (P = .049). Both OS and PFS were similar in the patients diagnosed before or after 3 months from the symptoms onset (P = .772). The 6-12 months group was the group with the better median both OS (197.4 months) and DFS (197.4) from the diagnosis. The median OS for the other groups were similar among them. CONCLUSION: A delay in the diagnosis of MM is very frequent in México (68% of cases); despite the fact that there was a significant trend for being in earlier stages of the disease and being diagnosed within 3 months from the onset of symptoms, we did not find a relationship between a delay on the diagnosis of the disease and a higher risk of complications and/or poor prognosis. Possible explanations to these findings are discussed.

Neutrophil to lymphocyte ratio and systemic immune-inflammatory index as markers of response to autologous hematopoietic stem cell transplantation in persons with multiple sclerosis.

INTRODUCTION: Biomarkers that help to evaluate the immune system and could be useful in multiple sclerosis (MS) are the neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and systemic immune-inflammatory index (SII). The objective of this work is to evaluate the significance of the SII index, PLR, and NLR before and after transplantation in individuals with MS who underwent autologous hematopoietic stem cell transplant (aHSCT) at a single institution. METHODS: Patients with MS who received an aHSCT between 2017 and 2022 were included in the study. NLR, PLR, and SII index were calculated prior to the transplant and 100 days after, and evaluation of the expanded disability status scale (EDSS) was done before the transplant and 12 months after. The cohort was divided into two groups: aHSCT responders (R) and nonresponders (NR). RESULTS: Fifty-eight individuals were examined: 37 patients in the responders group R group and 21 in NR group. There was no statistically significant difference in the SII, NLR, and PLR prior to the transplant, however at 100 days post-HSCT, NLR in the R group was 1.8 versus 3.1 in the NR group (p = 0.003), PLR was 194 versus 295, respectively (p = 0.024), meanwhile SII index was 489.5 versus 729.3 (p < 0.001). CONCLUSION: High NLR and SII index values after the aHSCT were associated with a worsening in the EDSS score. However, since this is the first ever study that compared NLR and SII index with the aHSCT response in persons with MS, further studies must be performed to corroborate this information.

More about post-transplant cyclophosphamide in haploidentical grafts: full or reduced doses?

Haploidentical hematopoietic can be conducted on an outpatient basis but the two main reasons to accept into the hospital a patient in this setting are complications of the hematological toxicity and/or the cytokine-release syndrome. With the aim of reducing the post-transplant cyclophosphamide-dependent toxicity without compromising its effectivity, attempts to reduce the dose of post-transplant cyclophosphamide have been made: Decreases from the conventional total dose of post-transplant cyclophosphamide (100 mg/Kg) have been explored worldwide, showing that decreasing the total dose to even 50 mg/Kg significantly decreases the toxicity of the procedure without compromising its efficacy, safety and results. We present here the salient data of the attempts to diminish the doses of post-transplant cyclophosphamide which have been done and published worldwide, information that suggests that the conventional doses of post-transplant cyclophosphamide can be significantly reduced thus decreasing the toxicity, without compromising the effectiveness of the procedure, mainly the development of graft versus host disease.

The consequences of delayed diagnosis and treatment in persons with multiple sclerosis given autologous hematopoietic stem cell transplantation.

OBJECTIVES: We have analyzed the association of delayed both diagnosis and treatment of persons with MS with the long-term results of patients given autologous hematopoietic stem cell transplantation (aHSCT). METHODS: Patients with MS referred to the HSCT-Mexico program were included in the study; in 103, detailed pre- and post-transplant evolution could be recorded. Two groups of patients were analyzed according to the time of evolution between the onset of symptoms and the definite diagnosis of MS: more than 8 months (delayed diagnosis, DD), or less than 8 months (non-delayed diagnosis, NDD). The progression of MS was assessed by changes in the expanded disability status scale (EDSS). RESULTS: The time elapsed between the onset of symptoms and the correct diagnosis was lower for the NDD group (1.55 vs. 35.87 months, p<0.05). Both groups of patients showed a similar EDSS score at diagnosis (1.5 vs. 1.5); however, the EDSS at the time of the transplant was higher in the DD group (4.5 vs. 3.0, p=0.3) and the response of the EDSS score to the transplant was significantly better for the NDD group, the last EDSS scores being 2.5 vs. 4.25 (p=0.03). Both groups of patients responded to aHSCT by diminishing the EDSS, but the response was significantly better in the NDD group. CONCLUSIONS: These data indicate that both the pre-transplant progression of the disease and the response to aHSCT were significantly worse in the DD group. An early diagnosis and an early aHSCT intervention are critical for a good prognosis, in terms of lowering and stabilizing the motor disability in MS patients given autografts.

Second Autologous Hematopoietic Stem Cell Transplantation in Multiple Sclerosis: A Single-Center Prospective Experience.

Immunosuppressive therapy is useful in persons with multiple sclerosis (MS), and autologous hematopoietic stem cell transplantation (aHSCT) is the most effective immunosuppressive treatment in this setting. Information on the usefulness of a second aHSCT in patients with MS is scarce. In a group of 1225 individuals with MS prospectively managed with aHSCT, we analyzed the salient features of 4 patients who received 2 consecutive transplants. After a moderate initial response to the first aHSCT, the patients were transplanted again after deterioration of their neurologic status; the second transplant was well tolerated and, in all instances, was completed on an outpatient basis and with no associated undesired toxicity. The autograft protocol is registered in ClinicalTrials.gov, identifier NCT02674217. After the second graft, the expanded disability status scale score stabilized in 2 patients; in 1, the post-transplant period was too short to assess the response, and in another, the development of associated Parkinson's disease precluded the assessment of the outcome. In conclusion, a second aHSCT in persons with MS is feasible, safe, and may lead to a positive response in some cases.

Non-alcoholic fatty liver disease and thrombocytopenia IV: its association with granulocytopenia

ABSTRACT Introduction: We have previously shown that some patients present thrombocytopenia (less than 100 × 109/L platelets) in non-alcoholic fatty liver disease (NAFLD). To further explore the nature of this association, we have now analyzed the association of thrombocytopenia with neutropenia (less than 0.5 × 109/L granulocytes) in NAFLD. Material and methods: Persons with NAFLD were prospectively accrued in the study after February 2018. The presence of NAFLD was defined by both serologic determinations (Fibromax ®) and liver transient elastography (TE/Fibroscan ®). Results: In 123 consecutive patients with NAFLD without cirrhosis, thrombocytopenia was identified in 20 (16%), whereas neutropenia was identified in 9 (7%). In the subset of 20 patients with NAFLD and thrombocytopenia, granulocytopenia was identified in 5 (25%), whereas in the subset of 9 patients with granulocytopenia, thrombocytopenia was identified in 5 (55%). We found a significant association between thrombocytopenia and both leukopenia and granulocytopenia (OR 8.25, 95% CI 1.9-34.2, p = 0.004). Conclusions: Both thrombocytopenia and neutropenia were identified in persons with NAFLD and, as there is a significant relationship between these two variables, we speculate that this finding may support the possibility of hypersplenism being involved in the cytopenias found in NAFLD without cirrhosis.

¿Quién juzga la actuación médica? / Who judges medical practice?

Resumen Las demandas judiciales por la percepción del paciente de una actuación médica inadecuada son una realidad creciente en la práctica médica, la cual entraña una preocupación extendida en el gremio médico. Las demandas judiciales frecuentemente conllevan circunstancias adicionales a la primaria preocupación de prevenir o sancionar actos de negligencia médica. CETREMI emite algunas recomendaciones a los profesionales jurídicos y médicos para mejorar esta situación y evitar daños en la relación médico-paciente.

Abstract Lawsuits due to patient perception of inappropriate medical actions are a growing reality in medical practice, which entails widespread concern in the medical community. Lawsuits often entail additional circumstances beyond the primary concern of preventing or sanctioning acts of medical negligence. CETREMI proposes various recommendations aimed at legal and medical professionals to improve this circumstance and avoid harming the doctor-patient relationship.

Nuevas modalidades de interacción entre la industria farmacéutica y los médicos / New interaction modalities between the pharmaceutical industry and physicians

Resumen Debido a las restricciones sanitarias secundarias a la pandemia de COVID-19, diversas interacciones entre la industria farmacéutica y los médicos cambiaron. Una de ellas ha sido el método promocional de medicamentos a través de reuniones académicas en torno a padecimientos de interés financiero. Una modalidad reciente ha sido la promoción unilateral de un fármaco determinado por parte de la industria farmacéutica por medio de eventos académicos con la invitación de aparentes "expertos". Estas reuniones frecuentemente están sesgadas no hacia la atención óptima de un padecimiento, sino a la promoción comercial de un medicamento específico que pudiera o no ser la mejor opción o sin la consideración de alternativas terapéuticas asociadas. El Comité de Ética y Transparencia en la Relación Médico-Industria, de la Academia Nacional de Medicina de México, analiza esta nueva circunstancia y propone algunas consideraciones a la comunidad médica.

Abstract Due to sanitary restrictions secondary to the COVID-19 pandemic, various interactions between the pharmaceutical industry and physicians have changed. One of them has been the method for promoting medicinal products through academic meetings around diseases of financial interest. A recent modality has been unilateral promotion by the pharmaceutical industry through academic events with the invitation of so-called "experts" for the promotion of a specific drug; these meetings are often biased not towards optimal care of a disease, but rather towards commercial promotion of a specific drug, which may or may not be the best option, without considering associated therapeutic alternatives. The Committee of Ethics and Transparency in the Physician-Industry Relationship, of the National Academy of Medicine, analyzes this new circumstance and proposes some considerations to the medical community.

Familias de médicos en la Academia Nacional de Medicina de México / Families of doctors at Academia Nacional de Medicina de México

Resumen Introducción: La decisión de involucrarse en el estudio y la práctica de la medicina no es fácil. Dentro del ambiente científico, alcanzar el éxito tanto profesional como personal requiere de una disciplina estricta en donde el esfuerzo se vuelve parte esencial de la vida diaria, además, el tener el apoyo familiar se vuelve un pilar para no perder la ilusión ante las distintas adversidades que se presentan en la formación médica. Objetivo: Identificar a las familias donde mínimo dos miembros pertenecen a la Academia Nacional de Medicina. Métodos: Se llevó a cabo un estudio transversal para analizar las familias de médicos mexicanos en las que por lo menos dos miembros, consanguíneos o políticos, han sido o son miembros de la Academia Nacional de Medicina de México por medio de la consulta del Directorio de la Academia Nacional de Medicina del año 2017 y el contacto de manera personal con los distintos académicos. Resultados: Se recolectó información de 45 familias pertenecientes a la Academia Nacional de Medicina de México. Conclusiones: A partir de este estudio es posible evidenciar la gran influencia que emiten algunos médicos en su entorno familiar, que hace que el estudio de la medicina sea atractivo como proyecto de vida.

Abstract Introduction: The decision to get involved in the study and practice of medicine is not easy. Within the scientific environment, achieving both professional and personal success requires a strict discipline, where effort becomes an essential part of daily life; in addition, having family support becomes crucial in order for not to lose hope when confronting the different adversities that arise during medical training. Objective: To identify members where at least two members belong to the Academia Nacional de Medicina de México" (ANMM). Methods: A cross-sectional study was carried out to identify families of Mexican doctors where at least two members, consanguineous or in-laws, have been or are ANMM members of the through a review of 2017 ANMM Directory personal contact with the different academics. Results: Information on 45 families belonging to the ANMM was collected. Conclusions: From this study, it is possible to show the great influence that some doctors have in their family environment, wich makes the study of medicine attractive as a life project.

Hematopoietic stem cell transplantation (HSCT) in the COVID-19 pandemic era / Trasplante de células progenitoras hematopoyéticas (TCPH)en la era del COVID-19

Abstract The pandemic of coronavirus infectious disease-2019 (COVID-19) caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is a threat to global health. This has become an obstacle for the practice of hematology, mostly in low-middle income countries. For those hematologist and oncologists who perform hematopoietic stem cell transplantation HSCT the usual challenges of this kind of medical procedures have increased because of the COVID-19 pandemic. The importance of find the best route for performing HSCT avoiding excessive COVID-19 risk is of great importance. By doing so, we have been able to conduct 170 hematopoieic stem cell transplants during the COVID-19 pandemia.

Resumen La pandemia de COVID-19 ha causado daños graves a la práctica de la medicina y se ha convertido en un obstáculo para la práctica de algunas especialidades. Los trasplantes de células hematopoyéticas han sufrido las consecuencias de la pandemia y muchos centros de trasplante hematopoyético en todo el mundo suspendieron sus labores. Gracias a la implementación de medidas de escrutinio y aislamiento de los pacientes sometidos a trasplante hematopoyético, hemos podido trasplantar a 170 pacientes en la época del COVID-19.

Importancia del cuadro básico de medicamentos en la prescripción médica / Importance of the list of essential medicines in medical prescription

Resumen La implementación en instituciones de salud de un cuadro básico permite adquirir y administrar una larga lista de medicamentos que presenta a los médicos las alternativas de tratamiento, así como la descripción académica colegiada de indicaciones, dosis, efectos secundarios, interacciones y análisis de costo-beneficio, con lo que se facilita la prescripción médica y la administración de insumos para la salud. El Comité de Ética y Transparencia en la Relación Médico-Industria emite diversas recomendaciones para la optimización de los beneficios generados por los cuadros básico de medicamentos.

Abstract The implementation of an essential medicines list in health institutions allows acquiring and administering a long list of drugs that offers treatment alternatives to physicians, as well as a collegiate academic description of indications, doses, side effects, interactions and cost-benefit analyses, thus facilitating medical prescription and administration of health products. The Committee of Ethics and Transparency in the Physician-Industry Relationship issues several recommendations for optimizing the benefits generated by essential medicines lists.

Automedicación y autoprescripción / Self-medication and self-prescription

Resumen La automedicación y la autoprescripción son acciones de los pacientes; la primera como elemento del autocuidado que involucra medicamentos de venta libre y la segunda como una violación a la ley de salud, pues comprende medicamentos que solo pueden expenderse con receta. Todos los inconvenientes que se han atribuido a la automedicación en realidad lo son de la autoprescripción.

Abstract Self-medication and self-prescription are actions undertaken by patients; the former, as an element of self-care that involves over-the-counter drugs, and the latter, as a violation of the Statute of Health, since it includes drugs that can only be dispensed with a medical prescription. All the drawbacks that have been attributed to self-medication are actually associated with self-prescription.

La receta médica: ¿reflejo condicionado o reflexión consciente? / Medical prescription: conditioned reflex or conscious reflection?

Resumen Extender una receta o indicar un tratamiento suele ser la última parte de la consulta médica. Este proceso crucial puede desvirtuarse debido a múltiples factores como capacidad prescriptiva limitada, exceso de trabajo y falta de reflexión o tiempo. La información insuficiente acerca del paciente o del tratamiento afecta el proceso prescriptivo y propicia errores que pueden ser graves para la salud del enfermo. La Academia Nacional de Medicina, en consonancia con la Organización Mundial de la Salud, hace énfasis en hacer del proceso prescriptivo un ejercicio de reflexión.

Abstract Writing a prescription or indicating a treatment is usually the last part of medical consultation. This crucial process can be undermined by multiple factors such as limited prescriptive ability, overwork, and lack of reflection or time. Insufficient information about the patient or the treatment affects the prescriptive process and leads to errors that can be serious for patient health. The National Academy of Medicine, in line with the World Health Organization, emphasizes the relevance of making the prescriptive process a reflective exercise.