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Abstract Acute myeloid leukemia (AML) comprises a heterogeneous group of hematopoietic cell neoplasms of myeloid lineage that arise from the clonal expansion of their precursors in the bone marrow, interfering with cell differentiation, leading to a syndrome of bone marrow failure. AML is a consequence of genetic and epigenetic changes (point mutations, gene rearrangements, deletions, amplifications, and arrangements in epigenetic changes that influence gene expression) in hematopoietic precursor cells, which create a clone of abnormal cells that are capable of proliferating but cannot differentiate into mature hematopoietic cells or undergo programmed cell death. The diagnosis requires more than 20% myeloid blasts in the bone marrow and certain cytogenic abnormalities. Treatment will depend on age, comorbidities, and cytogenetic risk among the most frequent.
ABSTRACT
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Abstract Hemophilia is a hemorrhagic disorder with a sex-linked inherited pattern, characterized by an inability to amplify coagulation due to a deficiency in coagulation factor VIII (hemophilia A or classic) or factor IX (hemophilia B). Sequencing of the genes involved in hemophilia has provided a description and record of the main mutations, as well as a correlation with the various degrees of severity. Hemorrhagic manifestations are related to levels of circulating factor, mainly affecting the musculoskeletal system and specifically the large joints (knees, ankles and elbows). This document is a review and consensus of the main genetic aspects of hemophilia, from the inheritance pattern to the concept of women carriers, physiopathology and classification of the disorder, the basic and confirmation studies when hemophilia is suspected, the various treatment regimens based on infusion of the deficient coagulation factor as well as innovative factor-free therapies and recommendations for the management of complications associated with treatment (development of inhibitors and/or transfusion transmitted infections) or secondary to articular hemorrhagic events (hemophilic arthropathy). Finally, relevant reviews of clinical and treatment aspects of hemorrhagic pathology charachterized by acquired deficiency of FVIII secondary to neutralized antibodies named acquired hemophilia.
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Resumen El linfoma de Hodgkin (LH) se debe a la transformación clonal de células originadas en los linfocitos B, lo que genera las células binucleadas patognomónicas de Reed-Sternberg. El LH es una enfermedad de células B con una distribución bimodal, con mayor incidencia en la adolescencia y la tercera década de la vida y un segundo pico en personas mayores de 55 años. Las células del LH clásico habitualmente sufren una reprogramación de la expresión génica, ya que pierden la expresión de la mayoría de los genes típicos de las células B y han adquirido la expresión de múltiples genes que son típicos de otros tipos de células del sistema inmunitario. El algoritmo de tratamiento dependerá si se trata de LH clásico o de predominio linfocítico, si es un estadio temprano con marcadores de pronóstico desfavorables o no, el esquema inicial de manejo y si existe enfermedad voluminosa, entre las variables más relevantes.
Abstract Hodgkin's lymphoma is due to the clonal transformation of cells originating from B lymphocytes, generating the pathognomonic binucleate Reed-Sternberg cells. Hodgkin's lymphoma is a B cell disease with a bimodal distribution, with higher incidence in adolescence and the third decade of life, showing a second peak in people over 55 years of age. Classic Hodgkin lymphoma cells routinely undergo gene expression reprogramming, as they lose the expression of most of the typical B-cell genes and acquire the expression of multiple genes that are typical of other types of cells in the immune system. The treatment algorithm will depend on whether it is classic or predominantly lymphocytic HL, if it is early stage with unfavorable prognostic markers or not, the initial management regimen, and whether there is bulky disease, among the most relevant variables.
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Resumen La pandemia por enfermedad por coronavirus 2019 (COVID-19), causada por el coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2), ha afectado ya a 180 países. Los pacientes con cáncer/inmunosupresión a mayor edad tienen más riesgo de presentar formas graves de la enfermedad. Los pacientes con leucemia aguda son un reto para el manejo durante la pandemia. Las recomendaciones para el manejo de estos pacientes están basadas en opinión de expertos. Se trata de una población en la que hay que realizar de forma sistemática pruebas de reacción en cadena de la polimerasa para SARS-CoV-2 y diferir en la medida de lo posible la quimioterapia citotóxica en los pacientes que resulten positivos. Por otro lado, algunos de los fármacos frecuentemente utilizados como los corticosteroides, el rituximab o la asparaginasa, pueden potencialmente complicar el curso del COVID-19, por lo que se deberá de considerar diferirlos o ajustarlos en poblaciones de mayor riesgo. De la misma forma, tomando en cuenta las particularidades de cada centro, en ciertos casos se podrá considerar dar preferencia a los esquemas de tratamiento ambulatorios que nos permitan además disminuir el requerimiento transfusional. Finalmente, muchos de los pacientes con leucemia aguda son candidatos para recibir trasplante alogénico de células progenitoras hematopoyéticas (aloTCPH). Debe tomarse en cuenta la limitación de los espacios en terapia intensiva, así como el grado de inmunosupresión derivado del trasplante. La recomendación es no diferir los aloTCPH en los pacientes con una mayor riesgo de recaída de la enfermedad. Más adelante conoceremos las consecuencias de las modificaciones en el tratamiento derivadas de la pandemia sobre la leucemia.
Abstract The coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has already affected 180 countries. Older patients and patients with cancer or immunosuppression are at greater risk of severe forms of the disease. Patients with acute leukemia are challenging to manage during the pandemic. Recommendations for the management of these patients are based on expert opinion. This is a population in which polymerase chain reaction tests for SARS-CoV-2 must be performed routinely and cytotoxic chemotherapy should be deferred as far as possible in positive patients. On the other hand, some of the frequently used drugs such as corticosteroids, rituximab or asparaginase, can potentially complicate the course of COVID-19, so consideration should be given to deferring or adjusting them in higher-risk populations. In the same way, considering the particularities of each center, in certain cases it may be reasonable to give preference to outpatient regimens that also allow us to decrease the transfusion requirement. Finally, many of the patients with acute leukemia are candidates to receive allogeneic hematopoietic stem cell transplantation (alloHSCT). The limitation of the spaces in intensive care units must be considered, as well as the degree of immunosuppression derived from the transplant. The recommendation is not to defer alloHSCT in patients with an increased risk of relapse. Later, we will learn about the consequences on of the modifications in treatment on leukemia derived from the pandemic.
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Climate and subsurface sea temperature in Bahía Culebra, Costa Rica. Bahía Culebra, Golfo de Papagayo, Costa Rica is a seasonal upwelling area. To determine the relationship of climate and the subsurface temperature variability at Bahía Culebra, we analyzed nine records of sea subsurface temperature from the Bay, continuously recorded from 1998 to 2010. The analysis characterized the annual cycle and explored the influence of different climate variability sources on the subsurface sea temperature and air temperature recorded in Bahía Culebra. Data from an automatic meteorological station in the bay were studied, obtaining the annual and daily cycle for air surface temperature and wind speed. Sea surface temperature (SST) trend from 1854 to 2011 was calculated from reanalysis for the region that coverts 9-11°N, 85-87°W. Because of the positive SST trend identified in this region, results showed that annual and daily cycles in Bahía Culebra should be studied under a warming scenario since 1854, that is coherent with the global warming results and its climate variability is influenced by El Niño-Southern Oscillation (ENSO) in the Equatorial Pacific and by atmospheric forcing triggered by climate variability with Atlantic Ocean origin, because warm (cold) events in Bahía Culebra tend to occur in concordance with positive & negative (negative & positive) anomalies in Niño 3.4 (NAO) index.
Bahía Culebra, Golfo de Papagayo, Costa Rica es una región de afloramiento estacional. Para determinar la relación entre el clima y la variabilidad de la temperatura sub-superficial, se analizaron los registros de la temperatura sub-superficial del mar de nueve estaciones localizadas en la Bahía. El análisis permitió caracterizar su ciclo anual y explorar su relación con fuentes de variabilidad climática que influencian el clima regional para el periodo 1998-2010. Los resultados se contextualizaron usando además los datos de una estación meteorológica automática que funcionó en la bahía junto con el registro de la temperatura superficial del mar para una rejilla que cubre la región de 9-11°N, 85-87°W, para el periodo 1854-2011. Debido a la tendencia positiva encontrada en la región para la temperatura superficial del mar, se concluye que los resultados mostrados asociados a los ciclos anuales y diarios en Bahía Culebra deben ser interpretados bajo un escenario de cambio climático, asociado a un calentamiento ocurrido desde 1854, además coherente con lo observado globalmente, y que su variabilidad climática está influenciada no sólo por aquella ligada a la de El Niño-Oscilación del Sur, en el Pacífico Ecuatorial, sino también por influencias de tipo atmosférico relacionadas con la variabilidad en el Océano Atlántico, debido a que los eventos cálidos (fríos) en Bahía Culebra tienden a ocurrir en concordancia con anomalías positivas y negativas (negativas y positivas) de los índices Niño 3.4 y OAN, respectivamente.