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INTRODUCTION AND OBJECTIVES: Thrombocytopenia frequently occurs after transcatheter aortic valve implantation (TAVI) but its impact is poorly understood. We aimed to analyze the incidence, clinical impact, and predictors of acquired thrombocytopenia after TAVI. METHODS: This retrospective multicenter registry included 3913 patients undergoing TAVI with a baseline platelet count of ≥ 100 *109/L. Acquired thrombocytopenia was defined as a decrease in baseline platelet count of ≥ 50% (early nadir ≤ 3 days and late nadir ≥ 4 days) post-TAVI. The primary endpoint was 30-day all-cause mortality and secondary endpoints were procedural safety and 2-year all-cause mortality. RESULTS: The incidence of acquired thrombocytopenia was 14.8% (early nadir: 61.5%, late nadir: 38.5%). Thirty-day mortality occurred in 112 (3.0%) patients and was significantly higher in those with thrombocytopenia (8.5% vs 2.0%, adjusted OR, 2.3; 95%CI, 1.3-4.2). Procedural safety was lower and 2-year mortality was higher in patients with thrombocytopenia vs those without (47.9 vs 33.0%; P < .001, and 30.2% vs 16.8%; HR, 2.2, 95%IC, 1.3-2.7) and especially in those with late nadir thrombocytopenia (54.2% vs 45.5%; P = .056, and 38.6% vs 23.8%, HR, 2.1; 95%CI, 1.5-2.9). Independent predictors of thrombocytopenia comprised baseline and procedural factors such as body surface area, absence of diabetes, poorer renal function, peripheral vascular disease, nontransfemoral access, vascular complications, type of transcatheter heart valve, and earlier TAVI procedures. CONCLUSIONS: Acquired thrombocytopenia was common (15%) after TAVI and was associated with increased short- and mid-term mortality and decreased procedural safety. Moreover, late thrombocytopenia compared with early thrombocytopenia was associated with significantly worse clinical outcomes. Further investigations are needed to elucidate the etiologic mechanisms behind these findings.

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Introducción: El dengue es la enfermedad arboviral más común en los seres humanos. Un diagnóstico temprano y preciso del dengue puede respaldar el manejo clínico, la vigilancia y el control de la enfermedad y es fundamental, por ello en el diagnóstico del dengue es importante contar con pautas clínicas y epidemiológicas que permitan la identificación oportuna y una conducta terapéutica adecuada. Objetivos: Evaluar la validez de herramientas diagnósticas en pacientes pediátricos hospitalizados con diagnóstico presuntivo de dengue en un Hospital de Referencia de Paraguay durante los años de 2012 a 2020. Materiales y métodos: Estudio analítico de tipo observacional, retrospectivo correspondientes a pacientes pediátricos (0 a 18 años) internados en el Hospital de Referencia de Paraguay el periodo enero 2012 a julio 2020 con diagnostico presuntivo de dengue al ingreso. Se realizó́ un análisis bivariado relacionando las frecuencias de 20 grupos de criterios diagnósticos combinados y 3 criterios diagnósticos aislados (OMS 2009, nexo epidemiológico y antigenemia NS1 para dengue) con el gold standard de diagnóstico que fue la conversión serológica. Resultados: Participaron del estudio 342 sujetos. EL 44% tenía edad escolar y 70% tenía 5 años o más. El 52,76% (191) fueron masculinos. Se encontraron desnutrición y sobrepeso en el 13% y 2%, respectivamente. La combinación de proteína C reactiva con plaquetopenia se encontró́ en 0.45% de los pacientes sin dengue y en el 6% de los pacientes con diagnóstico final de dengue (p=0.004). Conclusión: Este resultado aporta la alternativa de uso de una combinación sencilla de exámenes de laboratorio que puede replicarse en salas de urgencias como en salas de internación en un primer contacto con pacientes febriles con sospecha de fiebre dengue.

Introduction: Dengue is the most common arboviral disease in humans. An early and accurate diagnosis of dengue can support the clinical management, surveillance and control of the disease and is essential, therefore in the diagnosis of dengue it is important to have clinical and epidemiological guidelines that allow timely identification and appropriate therapeutic conduct. Objectives: To evaluate the validity of diagnostic tools in pediatric patients hospitalized with a presumptive diagnosis of dengue in a Reference Hospital in Paraguay during the years 2012 to 2020. Materials and methods: Analytical study of case and control type, observational, longitudinal, retrospective corresponding to pediatric patients (0 to 18 years) admitted to the Reference Hospital of Paraguay from January 2012 to July 2020 with a presumptive diagnosis of dengue at income. A bivariate analysis was performed relating the frequencies of 20 groups of combined diagnostic criteria and 3 isolated diagnostic criteria (WHO 2009, epidemiological link and NS1 antigenemia for dengue) with the gold standard of diagnosis, which was serological conversion. Results: 342 subjects participated in the study. 44% were school age and 70% were 5 years old or older. 52.76% (191) were male. Malnutrition and overweight were found in 13% and 2%, respectively. The combination of C-reactive protein with thrombocytopenia was found in 0.45% of patients without dengue and in 6% of patients with a final diagnosis of dengue (p=0.004). Conclusion: This result provides the alternative of using a simple combination of laboratory tests that can be replicated in emergency rooms and inpatient wards in a first contact with febrile patients with suspected dengue fever.

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RESUMEN Las alteraciones en los recuentos celulares sanguíneos representan los hallazgos clínicos más notorios y recurrentes en pacientes que padecen enfermedad hepática, tanto aguda como crónica. Estos cambios constituyen un marcador importante de la disfunción hepática y, a menudo, desempeñan un papel crucial en la evaluación y manejo de estos pacientes. En conjunto con el alargamiento de las pruebas de coagulación, la trombocitopenia es la irregularidad más prevalente en estos individuos. Esta condición, así como las leucopenias, se le atribuye en gran medida al hiperesplenismo, una alteración en la que el bazo retiene y destruye las células sanguíneas, incluidas las plaquetas. Sin embargo, cuando el conteo plaquetario desciende por debajo de 10 x 103/µl, es fundamental considerar otras causas, como factores autoinmunitarios que pueden estar contribuyendo con la trombocitopenia. La anemia, definida como una disminución en el número de glóbulos rojos o en los niveles de hemoglobina, es otra característica constante que acompaña a la enfermedad hepática. Aunque en la mayoría de los casos la anemia es macrocítica, en algunas situaciones puede ser secundaria a eventos hemolíticos, como lo observado en el síndrome de Zieve. Esta diversidad en las manifestaciones de la anemia en pacientes hepáticos subraya la complejidad de las interacciones entre el hígado y los componentes sanguíneos. A pesar de los avances en la comprensión de las causas subyacentes de estas citopenias, las opciones del tratamiento siguen siendo limitadas. Generalmente, las opciones terapéuticas se enfocan en la administración de transfusiones de hemocomponentes para compensar las deficiencias en los recuentos celulares o en el uso de análogos de trombopoyetina (TPO) para estimular temporalmente la producción de las plaquetas en la medula ósea. No obstante, estos tratamientos tienden a abordar los síntomas más que las causas fundamentales de las alteraciones hematológicas en la enfermedad hepática. La persistencia y el empeoramiento de estas alteraciones pueden servir como indicadores tempranos de la progresión de la disfunción hepática. La relación intrincada entre el hígado y la homeostasis hematológica continúa siendo objeto de investigación, la compresión más profunda de estos mecanismos podría abrir potencialmente la puerta hacia enfoques terapéuticos más específicos y efectivos para abordar las citopenias en el contexto de la enfermedad hepática.

ABSTRACT Alterations in blood cell counts are the most prominent and recurrent clinical findings among patients suffering from both acute and chronic liver disease. These changes are an important marker of liver failure and often play a key role in the evaluation and management of these patients. Together with the prolongation of coagulation tests, thrombocytopenia is the most common disorder among these individuals. This condition, as well as leukopenia, is largely attributable to hypersplenism, a disorder in which the spleen retains and destroys blood cells, including platelets. However, when the platelet count drops below 10x103/µl, it is essential to consider other causes, such as autoimmune factors that may be contributing to the development of thrombocytopenia. Anemia, defined as a decrease in red blood cell count or hemoglobin levels, is another common characteristic of liver disease. Although in most cases macrocytic anemia occurs, in some situations it can be secondary to hemolytic events, as observed in Zieve's syndrome. This wide range of manifestations of anemia among liver patients highlights the complex interaction between liver and blood components. Despite advances in understanding the underlying causes of these cytopenias, treatment options remain limited. Therapeutic options generally focus on the transfusion of blood products to compensate for deficiencies in cell counts or on the use of thrombopoietin (TPO) analogues to temporarily stimulate platelet production in the bone marrow. However, these treatments tend to address the symptoms rather than the root causes of hematologic disorders in liver disease. The persistence and worsening of these disorders may serve as early indicators of the progression of liver failure. The complicated relationship between liver and hematological homeostasis remains the subject of research. A deeper understanding of these mechanisms could potentially open the door toward more targeted and effective therapeutic approaches to address cytopenias in the context of liver disease.

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Background Patients with chronic liver disease (CLD) often develop thrombocytopenia (TCP) as a complication. Severe TCP (platelet count<50×109/L) can increase morbidity and complicate CLD management, increasing bleeding risk during invasive procedures. Objectives To describe the real-world scenario of CLD-associated severe TCP patients’ clinical characteristics. To evaluate the association between invasive procedures, prophylactic treatments, and bleeding events in this group of patients. To describe their need of medical resource use in Spain. Methods This is a retrospective, multicenter study including patients who had confirmed diagnosis of CLD and severe TCP in four hospitals within the Spanish National Healthcare Network from January 2014 to December 2018. We analyzed the free-text information from Electronic Health Records (EHRs) of patients using Natural Language Processing (NLP), machine learning techniques, and SNOMED-CT terminology. Demographics, comorbidities, analytical parameters and characteristics of CLD were extracted at baseline and need for invasive procedures, prophylactic treatments, bleeding events and medical resources used in the follow up period. Frequency tables were generated for categorical variables, whereas continuous variables were described in summary tables as mean (SD) and median (Q1–Q3). Results Out of 1,765,675 patients, 1787 had CLD and severe TCP; 65.2% were male with a mean age of 54.7 years old. Cirrhosis was detected in 46% (n=820) of patients and 9.1% (n=163) had hepatocellular carcinoma. Invasive procedures were needed in 85.6% of patients during the follow up period. Patients undergoing procedures compared to those patients without invasive procedures presented higher rates of bleeding events (33% vs 8%, p<0.0001) and higher number of bleedings. While prophylactic platelet transfusions were given to 25.6% of patients undergoing procedures, TPO receptor agonist use was only detected in 3.1% of them... (AU)

Antecedentes Los pacientes con enfermedad hepática crónica (EHC) a menudo desarrollan trombocitopenia (TCP) como agravante de su enfermedad. La TCP grave (definida por un recuento de plaquetas < 50 x 109/L) puede aumentar la morbilidad y complicar el manejo de la EPC, incrementando el riesgo de hemorragia durante los procedimientos invasivos. Objetivos Describir el escenario de mundo real de las características clínicas de los pacientes con TCP grave asociado a EHC. Evaluar la asociación entre procedimientos invasivos, tratamientos profilácticos y eventos hemorrágicos en este grupo de pacientes, así como describir el uso de recursos médicos en España. Métodos Se plantea un estudio multicéntrico retrospectivo que incluye pacientes con diagnóstico confirmado de EHC y TCP grave en cuatro hospitales de la Red Nacional de Salud de España desde enero de 2014 hasta diciembre de 2018. Analizamos la información de texto libre de la Historia Clínica Electrónica (HCE) de pacientes que utilizan procesamiento de lenguaje natural (PLN), técnicas de aprendizaje automático y terminología de SNOMED-CT. Los datos demográficos, las comorbilidades, los parámetros analíticos y las características de la EHC se extrajeron al inicio del estudio, así como la necesidad de procedimientos invasivos, tratamientos profilácticos, eventos hemorrágicos y recursos médicos utilizados en el periodo de seguimiento. Se generaron tablas de frecuencia para las variables categóricas, mientras que las variables continuas se describieron en tablas resumen como media (SD) y mediana (Q1-Q3). Resultados De 1.765.675 pacientes identificados, 1.787 tenían EHC y TCP grave, siendo el 65,2% varones con una edad media de 54,7 años. Se detectó cirrosis en el 46% (n = 820) de los pacientes y el 9,1% (n = 163) de ellos presentaron un diagnóstico de carcinoma hepatocelular... (AU)

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La trombopenia inducida por fármacos (DITP) es una trombocitopenia adquirida debida a anticuerpos reactivos a plaquetas dependientes de fármacos que causan destrucción plaquetaria. Desde el inicio de la vacunación para SARC-CoV-2 han ido apareciendo casos de diferentes efectos adversos. Entre los más sonados se encuentra la trombosis trombopénica inmune. Presentamos dos pacientes con trombopenia aislada con antecedente de vacunación para COVID-19 en las semanas previas como evento desencadenante, tras haber hecho un estudio completo que descartó las otras posibles etiologías, así como una revisión bibliográfica sobre esta entidad.(AU)

Drug-induced thrombocytopenia (DITP) is an acquired thrombocytopenia due to drug-dependent platelet-reactive antibodies causing platelet destruction. Since the onset of vaccination for SARS-CoV-2, cases of different adverse effects have been appearing. Among the most notorious is immune thrombopenic thrombosis. We report two patients with isolated thrombocytopenia with a history of vaccination for COVID19 in the previous weeks as the triggering event; after having performed a full study that ruled out other possible aetiologies, as well as a bibliographic review on this entity.(AU)

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INTRODUCTION: Immune thrombocytopenia (ITP) is a potentially severe manifestation of systemic lupus erythematosus (SLE) reported in 7-40% of SLE patients. ITP has been associated with a higher risk of organ damage and mortality. OBJECTIVES: To describe which factors are associated with the presence of ITP in SLE patients. METHODS: Retrospective case-control study. Cases were defined as SLE patients who had ever developed ITP and were sex- and age-matched with two controls. A predictive model was constructed to identify SLE patients who were at risk of developing ITP. RESULTS: ITP prevalence in our SLE cohort was 8.35%. Cases had a higher frequency of hemolytic anemia, while controls had a higher prevalence of arthritis at SLE diagnosis. During SLE progression, cases tested positive for anticardiolipin, anti-ß2-glycoprotein 1, and lupus anticoagulant antibodies more frequently. Cases received mycophenolic acid and azathioprine more often than controls and had a higher SLICC/ACR score. The model demonstrated a sensitivity of 87.53%, a positive predictive value of 81.92%, a specificity of 80.50%, area under the curve of 83.92%, a F1 of 83% and an overall accuracy of 83.68%. The variables that best explain the model were hemolytic anemia, arthritis, oral ulcers, Raynaud's phenomenon, low C4, low CH50, anticardiolipin and anti-ß2GP1 antibodies. CONCLUSION: SLE patients who develop ITP have a distinct phenotype characterized by more hemolytic anemia and less arthritis at SLE onset, and higher prevalence of antiphospholipid syndrome antibodies during SLE progression. This phenotype is associated with heightened organ damage and the need for more intensive therapies and stricter follow-up. Our predictive model has demonstrated an impressive ability to identify SLE patients at risk of developing ITP.

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El síndrome de Wiskott-Aldrich es un error innato de la inmunidad de herencia ligada al cromosoma X, producido por variantes en el gen que codifica la proteína del síndrome de Wiskott-Aldrich (WASp). Reportamos el caso clínico de un paciente de 18 meses con diagnóstico de Wiskott-Aldrich que no presentaba donante antígeno leucocitario humano (HLA) idéntico y recibió un trasplante de células progenitoras hematopoyéticas (TCPH) con donante familiar haploidéntico. La profilaxis para enfermedad de injerto contra huésped incluyó ciclofosfamida (PT-Cy). El quimerismo del día +30 fue 100 % del donante y la evaluación postrasplante de la expresión de la proteína WAS fue normal. Actualmente, a 32 meses del trasplante, presenta reconstitución hematológica e inmunológica y quimerismo completo sin evidencia de enfermedad injerto contra huésped. El TCPH haploidéntico con PT-Cy se mostró factible y seguro en este caso de síndrome de WiskottAldrich en el que no se disponía de un donante HLA idéntico.

Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.

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Objective The lack of consensus and specific guidelines, and the introduction of new treatments in thrombocytopenia management in liver cirrhosis patients, required a series of recommendations by experts to improve knowledge on this disease. This study's aim was to improve the knowledge around thrombocytopenia in liver cirrhosis patients, in order to contribute to the generation of future evidence to improve the management of this disease. Patients and methods A modified version of the RAND/UCLA appropriateness method was used. The scientific committee, a multidisciplinary team of 7 experts in managing thrombocytopenia in liver cirrhosis patients, identified the expert panel, and participated in elaborating the questionnaire. Thirty experts from different Spanish institutions were invited to answer a 48-item questionnaire covering 6 areas on a nine-point Likert scale. Two rounds were voted. The consensus was obtained if >77.7% of panelists reached agreement or disagreement. Results A total of 48 statements were developed by the scientific committee and then voted by the experts, resulting in 28 defined as appropriate and completely necessary, relating to evidence generation (10), care circuit, (8), hemorrhagic risk assessment, decision-making and diagnostic tests (14), professionals’ role and multidisciplinary coordination (9) and patient education (7). Conclusions This is the first consensus in Spain on the management of thrombocytopenia in liver cirrhosis patients. Experts indicated several recommendations to be carried out in different areas that could help physicians make better decisions in their clinical practice (AU)

Objetivo La falta de consenso y guías específicas, y la introducción de nuevos tratamientos para el manejo de la trombocitopenia en pacientes con cirrosis hepática, requerían recomendaciones expertas para mejorar el conocimiento sobre dicha patología. El objetivo de este estudio es mejorar el conocimiento sobre la trombocitopenia en pacientes con cirrosis hepática de cara a contribuir en la generación de futuras evidencias que mejoren el manejo de esta patología. Metodología Ae utilizó una versión modificada de la metodología Delphi RAND/UCLA. El comité científico, formado por 7 expertos en el manejo de la trombocitopenia en pacientes con cirrosis hepática, identificó un panel de expertos y participó en la elaboración del cuestionario de recomendaciones. Treinta expertos de diferentes hospitales españoles fueron invitados a responder al cuestionario. Los expertos respondieron a 48 ítems divididos en 6 áreas en una escala Likert de 9 puntos. La votación tuvo lugar en 2 rondas, en las que se obtuvo consenso siempre y cuando >77,7% de los panelistas alcanzasen acuerdo o desacuerdo. Resultados Cuarenta y ocho recomendaciones fueron elaboradas por el comité científico para su votación por parte del panel de expertos. Finalmente 28 recomendaciones fueron consideradas apropiadas y completamente necesarias: 10 de ellas relativas a la generación de evidencia; 8 al circuito de cuidados; 14 a la evaluación de riesgo hemorrágico, la toma de decisiones y los test diagnósticos; 9 al papel de los profesionales y la coordinación multidisciplinar, y 7 a la educación de los pacientes. Conclusiones Se trata del primer consenso español en el manejo de la trombocitopenia en pacientes con cirrosis hepática. Los expertos definieron un amplio número de recomendaciones que podrían contribuir a la toma de decisiones clínicas y a la mejora en el manejo de estos pacientes en la práctica clínica real (AU)

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Introduction: Dengue is one of the most important vector-borne viral diseases in subtropical and tropical regions. The World Health Organization (WHO) 2009 classified dengue into three groups: dengue without alarm signs, dengue with alarm signs, and severe dengue. According to the type of dengue, various managements have been proposed. The authors suggest that a predictive factor to avoid a poor prognosis in this disease is to keep platelet levels stable in the patient to prevent a fatal outcome. The aim of this paper is to analyze the efficacy of transfusion of platelet agents in the outcome of patients with severe dengue and thrombocytopenia. Material and methods: A bibliographic search was carried out in the Medline, OVID, and Scielo databases from January 1, 2008, to April 31, 2023, using the MeSH terms. Results: 7 articles were included in the systematic review. A wide range in age was found (18 to 79 years). A minimal number of studies detail the comorbidities of patients in their enrollment. Regarding platelet transfusion, the average number of platelet units used in the transfusion case was 5 (2 - 14). Transfusions of ABO identical and compatible pooled platelets transfusion were found to be more successful in increasing platelets. Almost all of the patients included in the studies had platelets <20,000 at enrollment. One study found a significant increase in platelets after transfusion, while the rest did not find it compelling. No mortality associated with platelet transfusion was recorded. Conclusions: Prophylactic platelet transfusion is not recommended as a routine measure in patients with severe dengue and thrombocytopenia. The uncertainty highlights need to reach a specific consensus establishing the appropriate indications for platelet transfusion and what type of patients with dengue virus would be beneficial. (AU)

Introducción: El dengue es una de las enfermedades virales transmitidas por vectores más importantes en las regiones tropicales y subtropicales. La Organización Mundial de la Salud (OMS) en 2009 clasificó el dengue en tres grupos: dengue sin signos de alarma, dengue con signos de alarma y dengue grave. Según el tipo de dengue se han propuesto diversos manejos. Los autores sugieren que un factor predictivo para evitar un mal pronóstico en esta enfermedad es mantener estables los niveles de plaquetas en el paciente para evitar un desenlace fatal. El objetivo de este trabajo es analizar la eficacia de la transfusión de plaquetas en la evolución de pacientes con dengue grave y trombocitopenia. Material y métodos: Se realizó una búsqueda bibliográfica en las bases de datos Medline, OVID y Scielo desde el 1 de enero de 2008 al 31 de abril de 2023, utilizando los términos MeSH. Resultados: Se incluyeron 7 artículos en la revisión sistemática. Se encontró un amplio rango de edad (18 a 79 años). Un número mínimo de estudios detallan las comorbilidades de los pacientes en su inscripción. En cuanto a la transfusión de plaquetas, el promedio de unidades de plaquetas utilizadas en el caso de transfusión fue de 5 (2 - 14). Se encontró que las transfusiones de plaquetas combinadas ABO idénticas y compatibles tuvieron más éxito en el aumento de plaquetas. Casi todos los pacientes incluidos en los estudios tenían plaquetas <20 000 en el momento del reclutamiento. Un estudio encontró un aumento significativo de plaquetas después de la transfusión, mientras que el resto no lo encontró convincente. No se registró mortalidad asociada con la transfusión de plaquetas. Conclusiones: La transfusión profiláctica de plaquetas no se recomienda como medida de rutina en pacientes con dengue grave y trombocitopenia. ... (AU)

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Osimertinib es un inhibidor de tirosina quinasa (ITK) de tercera generación aprobado para el cáncer de pulmón no microcítico localmente avanzado o metastásico con mutación del EGFR. La prevalencia de efectos adversos hematológicos graves asociados a este fármaco es infrecuente según ficha técnica. Se describe el caso de una mujer de 69 años diagnosticada de cáncer de pulmón no microcítico localmente avanzado en tratamiento con osimertinib en primera línea con aparición de trombocitopenia severa que requirió de ingresos hospitalarios, transfusiones de sangre y plaquetas y de tratamiento con eltrombopag sin conseguir resultados favorables para la paciente. (AU)

Osimertinib is a third generation, tyrosine kinase inhibitor (TKI) approved for locally advanced or metastatic non-small cell lung cancer with EGFR mutation. The prevalence of serious haematological adverse events associated with osimertinib is uncommon according to the summary of product characteristics. The case of study describes a 69-year-old woman diagnosed with locally advanced non-small cell lung cancer treated with osimertinib, with onset of severe thrombocytopenia that required hospital admissions, blood and platelet transfusions, and treatment with eltrombopag, without achieving favourable results. (AU)

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BACKGROUND: Patients with chronic liver disease (CLD) often develop thrombocytopenia (TCP) as a complication. Severe TCP (platelet count<50×109/L) can increase morbidity and complicate CLD management, increasing bleeding risk during invasive procedures. OBJECTIVES: To describe the real-world scenario of CLD-associated severe TCP patients' clinical characteristics. To evaluate the association between invasive procedures, prophylactic treatments, and bleeding events in this group of patients. To describe their need of medical resource use in Spain. METHODS: This is a retrospective, multicenter study including patients who had confirmed diagnosis of CLD and severe TCP in four hospitals within the Spanish National Healthcare Network from January 2014 to December 2018. We analyzed the free-text information from Electronic Health Records (EHRs) of patients using Natural Language Processing (NLP), machine learning techniques, and SNOMED-CT terminology. Demographics, comorbidities, analytical parameters and characteristics of CLD were extracted at baseline and need for invasive procedures, prophylactic treatments, bleeding events and medical resources used in the follow up period. Frequency tables were generated for categorical variables, whereas continuous variables were described in summary tables as mean (SD) and median (Q1-Q3). RESULTS: Out of 1,765,675 patients, 1787 had CLD and severe TCP; 65.2% were male with a mean age of 54.7 years old. Cirrhosis was detected in 46% (n=820) of patients and 9.1% (n=163) had hepatocellular carcinoma. Invasive procedures were needed in 85.6% of patients during the follow up period. Patients undergoing procedures compared to those patients without invasive procedures presented higher rates of bleeding events (33% vs 8%, p<0.0001) and higher number of bleedings. While prophylactic platelet transfusions were given to 25.6% of patients undergoing procedures, TPO receptor agonist use was only detected in 3.1% of them. Most patients (60.9%) required at least one hospital admission during the follow up and 14.4% of admissions were due to bleeding events with a hospital length of stay of 6 (3, 9) days. CONCLUSIONS: NLP and machine learning are useful tools to describe real-world data in patients with CLD and severe TCP in Spain. Bleeding events are frequent in those patients who need invasive procedures, even receiving platelet transfusions as a prophylactic treatment, increasing the further use of medical resources. Because that, new prophylactic treatments that are not yet generalized, are needed.

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OBJECTIVE: The lack of consensus and specific guidelines, and the introduction of new treatments in thrombocytopenia management in liver cirrhosis patients, required a series of recommendations by experts to improve knowledge on this disease. This study's aim was to improve the knowledge around thrombocytopenia in liver cirrhosis patients, in order to contribute to the generation of future evidence to improve the management of this disease. PATIENTS AND METHODS: A modified version of the RAND/UCLA appropriateness method was used. The scientific committee, a multidisciplinary team of 7 experts in managing thrombocytopenia in liver cirrhosis patients, identified the expert panel, and participated in elaborating the questionnaire. Thirty experts from different Spanish institutions were invited to answer a 48-item questionnaire covering 6 areas on a nine-point Likert scale. Two rounds were voted. The consensus was obtained if >77.7% of panelists reached agreement or disagreement. RESULTS: A total of 48 statements were developed by the scientific committee and then voted by the experts, resulting in 28 defined as appropriate and completely necessary, relating to evidence generation (10), care circuit, (8), hemorrhagic risk assessment, decision-making and diagnostic tests (14), professionals' role and multidisciplinary coordination (9) and patient education (7). CONCLUSIONS: This is the first consensus in Spain on the management of thrombocytopenia in liver cirrhosis patients. Experts indicated several recommendations to be carried out in different areas that could help physicians make better decisions in their clinical practice.

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Introducción: Las quemaduras graves alteran de manera significativa los parámetros hematológicos y de la coagulación. Objetivo: Describir las alteraciones hematológicas en el paciente gran quemado. Métodos: Se realizó un estudio descriptivo, longitudinal y prospectivo, en pacientes ingresados en el servicio de Cirugía Plástica y Caumatología del Hospital Universitario Manuel Ascunce Domenech, de la provincia Camagüey, durante el período comprendido entre mayo de 2020 a abril de 2022. Se estudiaron 38 pacientes, se tuvieron en cuenta las siguientes variables: alteración hematológica, superficie corporal quemada, índice pronóstico, estado al egreso. Resultados: De los pacientes con 30 % o más de superficie corporal quemada (12 en total) el 91,66 % presentó anemia. La anemia se encontró con más frecuencia en los pacientes clasificados como muy graves, 11 pacientes que representan el 28,95 %, es de destacar que los pacientes con este índice de gravedad constituyeron la mayoría en esta serie, 19 para un 50 %. En 23 de los enfermos presentaron alteraciones leucocitarias. Se encontró leucocitosis en el 44,73 % de los pacientes y leucopenia en seis enfermo que representó el 15,79 %. En el 23,68 % de los enfermos se observó la presencia de trombocitopenia. Conclusiones: Las alteraciones hematológicas influyen en la buena evolución de los pacientes quemados, de no tratarse de forma oportuna pueden llevar a estos lesionados a la muerte.

Introduction: The serious burns alter of significant way the hematological and coagulation parameters. Objective: To describe the hematological alterations in the large burned patient. Methods: A descriptive, longitudinal and prospective study was carried out for the sake of describing the main hematological alterations in the large burned patient, once 2020 were deposited in the Manuel Ascunce Domenech's Universitary Hospital service of Plastic Surgery and Caumatology, of the City Camagüey, during the period understood between May, 2020 to April, 2022. They studied 38 patients in those who they had in account variables: hematological alteration, corporal burned-out surface, index forecast, state to the discharge. Results: Of the patients with 30 % or more of corporal burned-out surface (12 in total) the 91.66 % presented anemia. Anemia was found with more frequency in the patient classified as very serious condition, 11 patients that represent the 28.95 %, it is from standing out that the patients with this severity rate constituted the majority in this series, 19 for a 50 %. In 23 of the sick persons of this series they presented leukocytal alterations. Leucocytosis was found in 44.73 % of the patients, and leukopenia in six patients which represented 15.79 %. In 23.68 % of the patients the presence of thrombocytopenia was observed. Conclusions: Hematological alterations influence the good progress of burned patients, and if not treated in a timely manner can lead to death.

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Introducción: La pseudotrombocitopenia dependiente del ácido etilendiaminotetraacético (PTCP-EDTA) es un fenómeno de laboratorio con una prevalencia estimada del 0,1-2% entre los pacientes hospitalizados y entre el 15-17% en los pacientes ambulatorios evaluados por trombocitopenia aislada. Se produce por cambios conformacionales a nivel de las glicoproteínas de la superficie plaquetaria que inducen la agregación de plaquetas tras la exposición al EDTA. Esta agregación da lugar a una falsa disminución del conteo de unidades totales de plaquetas al utilizar analizadores automatizados.  Presentación del caso: Presentamos el caso de un paciente de 5 años que presentaba niveles bajos de plaquetas sin signos de hemorragia activa. El paciente fue ingresado en el hospital mientras se estudiaba la causa de su bajo conteo plaquetario. Para el diagnóstico de PTCP-EDTA, se realizó un frotis de sangre periférica y se compararon los niveles plaquetarios utilizando el tubo con anticoagulante de citrato. Conclusión: La PTCP-EDTA suele ser un diagnóstico que se pasa por alto y que puede dar lugar a procedimientos innecesarios y gastos adicionales para los pacientes que presentan este fenómeno in vitro. (provisto por Infomedic International)

Introduction: Ethylenediaminetetraacetic acid-dependent pseudothrombocytopenia (PTCP-EDTA) is a laboratory phenomenon with an estimated prevalence of 0.1-2% among hospitalized patients and between 15-17% in outpatients evaluated for isolated thrombocytopenia. It is caused by conformational changes at the level of platelet surface glycoproteins that induce platelet aggregation following EDTA exposure. This aggregation results in a false decrease in total platelet unit count when using automated analyzers. Case presentation: We present the case of a 5-year-old patient who presented with low platelet levels without signs of active bleeding. The patient was admitted to the hospital while the cause of his low platelet count was being studied. For the diagnosis of PTCP-EDTA, a peripheral blood smear was performed and platelet levels were compared using the tube with citrate anticoagulant. Conclusion: PTCP-EDTA is often an overlooked diagnosis that may result in unnecessary procedures and additional expense for patients presenting with this phenomenon in vitro. (provided by Infomedic International)

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Introducción: La trombocitopenia inducida por fármacos es un efecto adverso cuya incidencia es desconocida, pero que puede ser potencialmente severo. Pacientes y métodos: Se presentan los casos de dos pacientes con trombocitopenia asociada a ceftarolina y/o daptomicina utilizados en asociación en el tratamiento de endocarditis infecciosa por Staphylococcus aureus meticilin-resistente (SARM). Resultados: En los dos casos descritos se observó un descenso en el recuento de plaquetas durante el tratamiento combinado, continuando el efecto pese a la reducción de dosis y asociándose a ceftarolina por la secuencia temporal fármaco/efecto.Ambos casos fueron notificados al Servicio de Farmacovigilancia. La evaluación de causalidad de ceftarolina mediante el algoritmo de Karch Lasagna modificado por Naranjo et al. resultó como posible en primer caso y probable en el segundo.Conclusiones: Ante los dos casos descritos y otros recogidos en la revisión bibliográfica sobre el riesgo de trombocitopenia asociada a ceftarolina, se plantea la necesidad de realizar controles hematológicos, especialmente en pacientes con tratamientos prolongados y/o con dosis elevadas. Son necesarios estudios postautorización para evaluar la incidencia de efectos adversos poco frecuentes. (AU)

Introduction: Drug-induced thrombocytopenia is an adverse effect whose incidence is unknown, but which can be potentially severe. Patients and methods: The cases of two patients with thrombocytopenia associated with ceftaroline and/or daptomycin used in association in the treatment of infective endocarditis due to methicillin-resistant Staphylococcus aureus (MRSA) are presented. Results: In the two cases described, a decrease in the platelet count is shown during the combined treatment, continuing the effect despite the dose reduction and being associated with ceftaroline due to the drug/effect temporal sequence. Both cases were notified to the Pharmacovigilance Service. The causality assessment of ceftaroline using the Karch Lasagna algorithm modified by Naranjo et al. was possible in the first case and probable in the second. Conclusions: Given the two cases described and others collected in the literature review on the risk of thrombocytopenia associated with ceftaroline, it is necessary to carry out haematological controls, especially in patients with prolonged treatments and/or with high doses. Post-authorization studies are necessary to assess the incidence of rare adverse effects. (AU)

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RESUMEN Introducción : El síndrome inflamatorio multisistémico en pediatría (SIM-C) es una infrecuente entidad asociada a COVID-19 con un amplio espectro de presentación: desde un cuadro similar a la enfermedad de Kawasaki a una afectación multisistémica con shock. Se han descripto asociaciones entre valores de laboratorio y mala evolución, pero no existen puntos de corte que predigan la misma. Objetivo : El objetivo de este estudio fue describir y analizar las características de los pacientes con SIM-C y las relaciones de estas con los hallazgos de laboratorio. Material y métodos : Se realizó un estudio analítico y retrospectivo de niños internados con diagnóstico de SIM-C entre mayo 2020 y junio 2021 en el HNRG. Se estudiaron 32 pacientes, 17 femeninas (53,13%) y 15 masculinos (46,87%), edad promedio de 7,67 años (rango 0,5-14,91). Diez de los pacientes (31,25%) presentaron shock. Se obtuvieron datos clínicos, ecocardiográficos y valores de troponina I ultrasensible, NT-proBNP, plaquetas y linfocitos al momento del diagnóstico; y se analizaron comparativamente entre quienes presentaron shock durante la evolución (Grupo 1) y quienes no (Grupo 2). Resultados : La diferencia en un valor inicial de NT-proBNP elevado fue estadísticamente significativa entre ambos grupos (p=0,008), en tanto que la troponina y el recuento de linfocitos y plaquetas, no. De los 13 pacientes que requirieron inotrópicos, el 58% presentó linfopenia inicialmente (p=0,006 vs aquellos que no los necesitaron). Conclusiones : Si bien la mortalidad debido al SIM-C es baja, la afectación cardiovascular y el compromiso hemodinámico en los paci entes que presentaron este síndrome puede ser frecuente. Poder contar con una herramienta de laboratorio ampliamente difundida para la categorización de pacientes podría ayudar a mitigar riesgos y obtener una derivación temprana a centros especializados.

ABSTRACT Background : Multisystem inflammatory syndrome in children (MIS-C) is an uncommon condition associated with COVID-19 with a wide spectrum of presentations, ranging from Kawasaki-like disease to multisystem involvement with shock. The as sociation between the laboratory characteristics and unfavorable outcome has been described, but the cut-off points associated with higher risk have not yet been defined. Objective : The aim of this study was to describe and analyze the characteristics of patients with MIS-C and their associations with the laboratory findings. Methods : We conducted an analytical and retrospective study of pediatric patients hospitalized between May 2020 and June 2021 with diagnosis of MIS-C in Hospital General de Niños Dr. Ricardo Gutiérrez (HNRG). The cohort was made up of 23 patients, 17 female (53.13%) and 15 male (46.87%); mean age was 7.67 years (range 0.5-14.91). Ten patients (31.25%) presented shock. Clinical and echocardiographic data and values of high-sensitive troponin I, N-terminal pro-B-type natriuretic peptide (NT-proBNP), platelets and lymphocytes at the time of diagnosis were obtained and compared between those with shock during evolution (group 1) and those without shock (group 2). Results : There was a significant difference in baseline elevated NT-proBNP values between both groups (p = 0.008), but not in troponin levels and lymphocyte and platelet counts. Of the 13 patients who required inotropic agents, 58% had baseline lymphopenia (p = 0.006 vs those who did not require inotropic drugs). Conclusions : Although mortality due to MIS-C is low, cardiac involvement and hemodynamic impairment may be common. The availability of a commonly used laboratory tool for patient categorization could help to mitigate risks and obtain early referral to specialized centers.

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Introducción: Las microangiopatías trombóticas (MAT) son entidades infrecuentes que suelen causar afectación renal, hematológica, neurológica y cardiovascular, con síntomas inespecíficos pero graves. Con la finalidad de mejorar el conocimiento de sus características clínicas, el proceso diagnóstico y el tratamiento en la fase aguda, se ha creado el registro de MAT en las unidades de cuidados intensivos pediátricos (UCIP) de España (Registro MATUCIP). Pacientes y métodos: Estudio observacional, multicéntrico, realizado en 20 UCIP españolas desde enero de 2017 hasta diciembre de 2021 que incluyó niños mayores de 1mes con diagnóstico de MAT y seguimiento hasta el alta de la UCIP. Resultados: Se incluyeron 97 pacientes (51,5% mujeres), con una mediana de edad de 2,6años (RIQ: 1,6-5,7). La clínica inicial fue de tipo gastrointestinal (74,2%), respiratoria (14,4%), cuadro febril (5,2%), neurológica (3,1%) y otras (3,1%). Al ingreso, el 75,3% presentaban anemia hemolítica microangiopática, el 95,9% trombocitopenia y el 94,8% daño renal agudo. Fueron diagnosticados de síndrome hemolítico urémico (SHU) asociado a Escherichia coli productora de toxina Shiga el 57,7%, SHU por Streptococcus pneumoniae el 14,4%, SHU atípico el 15,6%, MAT secundaria el 10,3% y púrpura trombótica trombocitopénica el 2,1%. Desarrollaron hipertensión arterial el 89,7%, manifestaciones digestivas el 49,5%, respiratorias el 22,7%, neurológicas el 25,8% y cardiacas el 12,4%. El 60,8% requirieron depuración extrarrenal y el 2,1%, plasmaféresis. Recibieron eculizumab 20 pacientes. La mediana de estancia en la UCIP fue de 8,5días (RIQ: 5-16,5). Dos niños fallecieron. (AU)

Introduction: Thrombotic microangiopathies (TMA) are rare diseases usually presenting with renal, haematological, neurologic and cardiovascular involvement and nonspecific but severe symptoms. A registry of TMA cases managed in Spanish paediatric intensive care units (the MATUCIP registry) was established with the aim of gaining knowledge on their clinical characteristics, diagnosis and acute-phase treatment. Patients and methods: We conducted a prospective multicentre observational study in 20 paediatric intensive care units (PICUs) in Spain from January 2017 to December 2021 in children aged more than 1month with TMAs, who were followed up through the discharge from the PICU. Results: The sample included 97 patients (51.5% female) with a median age of 2.6years (interquartile range [IQR]: 1.6-5.7). The initial manifestations were gastrointestinal (74.2%), respiratory (14.4%), fever (5.2%), neurologic (3.1%) and other (3.1%). At admission, 75.3% of patients had microangiopathic haemolytic anaemia, 95.9% thrombocytopenia and 94.8% acute kidney injury. Of the total sample, 57.7% of patients received a diagnosis of Shiga toxin-associated haemolytic uraemic syndrome (HUS), 14.4% of Streptococcus pneumoniae-associated HUS, 15.6% of atypical HUS, 10.3% of secondary TMA and 2.1% of thrombotic thrombocytopenic purpura. Eighty-seven patients (89.7%) developed arterial hypertension, and 49.5% gastrointestinal, 22.7% respiratory, 25.8% neurologic and 12.4% cardiac manifestations. Also, 60.8% required renal replacement therapy and 2.1% plasma exchange. Twenty patients received eculizumab. The median PICU stay was 8.5days (IQR: 5-16.5). Two children died. (AU)

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INTRODUCTION: Thrombotic microangiopathies (TMA) are rare diseases usually presenting with renal, haematological, neurologic and cardiovascular involvement and nonspecific but severe symptoms. A registry of TMA cases managed in Spanish paediatric intensive care units (the MATUCIP Registry) was established with the aim of gaining knowledge on their clinical characteristics, diagnosis and acute-phase treatment. METHODS: We conducted a prospective multicentre observational study in 20 paediatric intensive care units (PICUs) in Spain from January 2017 to December 2021 in children aged more than 1 month with TMAs, who were followed up through the discharge from the PICU. RESULTS: The sample included 97 patients (51.5% female) with a median age of 2.6 years (interquartile range [IQR], 1.6-5.7). The initial manifestations were gastrointestinal (74.2%), respiratory (14.4%), fever (5.2%), neurologic (3.1%) and other (3.1%). At admission, 75.3% of patients had microangiopathic haemolytic anaemia, 95.9% thrombocytopenia and 94.8% acute kidney injury. Of the total sample, 57.7% of patients received a diagnosis of Shiga toxin-associated haemolytic uraemic syndrome (HUS), 14.4% of Streptococcus pneumoniae-associated HUS, 15.6% of atypical HUS, 10.3% of secondary TMA and 2.1% of thrombotic thrombocytopenic purpura. Eighty-seven patients (89.7%) developed arterial hypertension, and 49.5% gastrointestinal, 22.7% respiratory, 25.8% neurologic and 12.4% cardiac manifestations. Also, 60.8% required renal replacement therapy and 2.1% plasma exchange. Twenty patients received eculizumab. The median PICU stay was 8.5 days (IQR, 5-16.5). Two children died. CONCLUSIONS: The MATUCIP registry demonstrates the clinical variability of TMA cases requiring admission to the PICU. Knowledge of the presentation and outcomes of TMAs can facilitate early aetiological diagnosis. This registry can help improve our understanding of the clinical spectrum of these diseases, for which there is a dearth of published data.

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El cavernoma cerebral es una malformación vascular de diagnóstico infrecuente. Se define como una malformación a nivel de la vasculatura microcerebral que, dependiendo a la ubicación y si existe la posibilidad de ruptura, conlleva a una emergencia que puede terminar en la muerte del paciente. En esta oportunidad se reporta el caso de un paciente con cavernoma cerebral asociado al síndrome de Evans. Se decide manejo quirúrgico de la lesión por aumento de intensidad de cefalea e intolerancia oral. Dada la coexistencia del Síndrome de Evans y la alta tasa de morbimortalidad es que se decide manejo quirúrgico mediante radiocirugía estereotáxica con gamma knife. El uso de dosis de margen bajo para tratamiento con gamma knife para uso en cavernomas cerebrales produce un manejo controlado para sintomatología de convulsiones y mejor expectativa de calidad de vida.

Cerebral cavernoma is an infrequently diagnosed vascular malformation. It is defined as a malformation at the level of the microcerebral vasculature that, depending on the location and if there is a possibility of rupture, leads to an emergency that can end in the death of the patient. On this occasion, we report a case of a patient with cerebral cavernoma associated with Evans syndrome. Surgical management of the lesion was decided due to increased intensity of headache and oral intolerance. Given the coexistence of Evans Syndrome and the high rate of morbidity and mortality, surgical management was decided by stereotaxic radiosurgery with a gamma knife. The use of low-margin doses for treatment with gamma knife for use in brain cavernomas produces controlled management for seizure symptoms and better quality of life expectancy.

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Introducción: El ductus arterioso persistente (DAP) es una cardiopatía congénita de especial interés. La oclusión intervencionista es el tratamiento de elección, no obstante, el dispositivo médico "oclusor para DAP", usado en este procedimiento, puede dar lugar a la aparición de eventos adversos de rara frecuencia como hemorragia, trombocitopenia, entre otros. Reporte de caso: Se presenta un caso de una paciente pediátrica con trombocitopenia severa posterior a la intervención. Durante su hospitalización, la paciente mantuvo niveles bajos de plaquetas hasta que logra su recuperación y sale de alta sin una nueva manifestación hematológica. El caso fue reportado como sospecha de incidente adverso asociada al oclusor para DAP. Conclusión: Al realizarse el análisis causa-raíz, se concluye que el tamaño del oclusor para DAP podría ser la principal causa de la trombocitopenia. El presente caso es una muestra que los dispositivos médicos pueden causar eventos adversos severos, por lo que, es necesaria la implementación de tecnovigilancia, principalmente a los dispositivos médicos de alto riesgo.

Introduction: Patent ductus arteriosus (PDA) is a congenital heart condition of special interest. Interventional occlusion is the treatment of choice; however, a PDA occlude, used in this procedure, is a medical device that rarely could lead to adverse events such as hemorrhage, thrombocytopenia, etc. Case report: We present a case of a pediatric patient with severe thrombocytopenia after surgery. During her hospitalization, platelet levels remained lower and, finally, she was fully recovered without any hematological manifestation. The case was reported as a suspected adverse incident related to PDA occlude. Conclusion: After performing a cause-root analysis, we concluded that the size of the PDA occlude may be the main cause of thrombocytopenia. This case demonstrates that medical devices could lead to adverse events. Thus, it is important for clinicians to implement medical device surveillance, mainly for high-risk medical devices.