Recommendations for the future management of thrombocytopenia in patients with liver cirrhosis: A modified RAND/UCLA appropriateness method.

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.

Chronic liver disease-associated severe thrombocytopenia in Spain: Results from a retrospective study using machine learning and natural language processing.

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.

Impact of preoperative platelet count on perioperative outcome after laparoscopic splenectomy for idiopathic thrombocytopenic purpura. / Impacto del recuento plaquetario preoperatorio en la evolución perioperatoria tras la esplenectomía laparoscópica por púrpura trombocitopénica idiopática.

INTRODUCTION: Laparoscopic splenectomy (LS) is the preferred treatment of idiopathic thrombocytopenic purpura (ITP) when medical treatment fails. The objective was to evaluate the feasibility and safety of LS according to the preoperative platelet count. METHODS: This study is a retrospective analysis of a series of 199 patients who underwent LS for ITP from 1993 to 2015. The patients were divided into 3 groups according to platelet count: group i (<10×10(9)/L), group ii (10-50×10(9)/L) and group iii (> 50×10(9)/L). RESULTS: Operative time was significantly lower in Group III compared to Group I and II (100±53 and 105±61min, P<.025)). Intraoperative blood loss was statistically higher in group i (263±551ml) with respect to the other 2: group ii (128±352ml) and group iii (24±62ml) (P<.003). Hospital stay was 6.4±5.8 days in group i, significantly higher compared to groups ii and iii (3.8±2.3 and 3.2±1.8 days, respectively (P<.003)). CONCLUSION: Conducting a LS in ITP patients with low platelet counts is effective and safe.

Thrombocytopenia after transcatheter aortic valve implantation.

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.

Immune thrombocytopenia in systemic lupus erythematosus: Prevalence, risk factors, and a novel predictive model for risk assessment.

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.

Thrombotic microangiopathies in critically ill children: The MATUCIP registry in Spain.

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.

Distribution of peripheral blood cells after splenectomy in immune thrombocytopenia patients.

BACKGROUND: There are some difficulties regarding the evaluation of the post-splenectomy state. OBJECTIVE: The objective of the study is to compare the post-splenectomy blood changes of immune thrombocytopenia (ITP) patients with those of trauma patients, 1 month and ≥ 6 months after surgery. METHODS: Medical records of patients, who had undergone total splenectomy for ITP and trauma at a tertiary center between January 2009 and December 2019, were retrospectively reviewed. RESULTS: The current study included 52 patients, who had undergone splenectomy for ITP (57.7%), and trauma (42.3%). Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit, and platelet levels. Neutrophils were responsible for the preoperative leukocytosis in ITP patients, and neutrophilia was ameliorated by splenectomy and also withdrawal of the steroid therapy in some patients. Decreased neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio supported the finding that splenectomy ameliorated inflammation in ITP patients. Splenectomy resulted in a change in percentages of leukocytes in favor of basophils in ITP patients. CONCLUSIONS: Splenectomy, irrespective of the indications, resulted in an increase in hemoglobin concentration, hematocrit and platelet levels, lymphocyte, monocyte, and eosinophil counts. Splenectomy ameliorated inflammation in ITP patients and resulted in a change in percentages of leukocytes in favor of basophils.

ANTECEDENTES: Existen algunas dificultades con respecto a la evaluación del estado post-esplenectomía. OBJETIVO: Comparar los cambios sanguíneos post-esplenectomía de pacientes con PTI con los de pacientes traumatizados, 1 mes y ≥ 6 meses después de la cirugía. MÉTODOS: Se revisaron retrospectivamente las historias clínicas de los pacientes que habían sido sometidos a esplenectomía total por PTI y trauma en un centro terciario entre enero de 2009 y diciembre de 2019. RESULTADOS: El presente estudio incluyó a 52 pacientes, que habían sido sometidos a esplenectomía por PTI (57.7%) y traumatismo (42.3%). La esplenectomía, independientemente de las indicaciones, resultó en un aumento de la concentración de hemoglobina, hematocrito y niveles de plaquetas. Los neutrófilos fueron responsables de la leucocitosis preoperatoria en pacientes con PTI, y la neutrofilia mejoró mediante esplenectomía y también la suspensión de la terapia con esteroides en algunos pacientes. La disminución de NLR y PLR apoyó el hallazgo de una disminución de la inflamación en la esplenectomía en pacientes con PTI. La esplenectomía resultó en un cambio en los porcentajes de leucocitos a favor de los basófilos en pacientes con PTI. CONCLUSIONES: La esplenectomía, independientemente de las indicaciones, resultó en un aumento de la concentración de hemoglobina, niveles de hematocrito y plaquetas, recuentos de linfocitos, monocitos y eosinófilos. Una disminución de la inflamación en la esplenectomía en pacientes con PTI resultó en un cambio en los porcentajes de leucocitos a favor de los basófilos.

Clínica neurológica fluctuante: ¿Llamo al neurólogo o al hematólogo?

Objetivos: Resaltar el papel del laboratorio clínico y la pronta comunicación con el servicio de Hematología en el diagnóstico y la rapidez en la instauración del tratamiento de una patología hematológica tan urgente como la Púrpura Trombótica Trombocitopénica (PTT). Caso clínico: Varón de edad avanzada derivado a Urgencias hospitalarias por su médico de Atención Primaria por trastorno de emisión del lenguaje, asimetría facial y disminución de fuerza en extremidad superior, por lo que se activa el código ictus. Sin embargo, los hallazgos de laboratorio (anemia y trombocitopenia, creatinina, bilirrubina total y LDH elevadas, test de Coombs directo negativo) y presencia de esquistocitos en el frotis de sangre periférica, acaban derivando en un diagnóstico completamente diferente: microangiopatía trombótica tipo PTT. Conclusiones: La primera orientación diagnóstica de ictus hemisférico izquierdo fue rechazada ante los signos de anemia hemolítica no autoinmune, trombocitopenia sin causa aparente y presencia de esquistocitos. No debemos olvidar que esta patología puede cursar con afectación multiorgánica y características clínicas muy variables. Aunque su diagnóstico definitivo se basa en la determinación de actividad de ADAMTS-13, debido a la elevada mortalidad es necesario instaurar tratamiento de forma inmediata tras su sospecha.

Management of primary immune thrombocytopenia. A comparison between two historical cohorts.

INTRODUCTION: In recent years, there have been changes in the management of patients with primary immune thrombocytopenia. In this study, a review is presented of the characteristics and outcomes of children with primary immune thrombocytopenia in a children's hospital (Hospital Infantil Niño Jesús). Moreover, an analysis is made of the changes in the care of these patients diagnosed before and after 2011, when new guidelines were published by the Spanish Society of Paediatric Haematology Oncology (SEHOP). MATERIAL AND METHODS: Data from a cohort of primary immune thrombocytopenia patients followed up in this hospital have been retrospectively reviewed. The statistical package used for the analysis was SPSS Statistics 22.0 (IBM Corp., Chicago, IL, USA). RESULTS: A review is presented on the clinical data from 235 paediatric patients diagnosed with primary immune thrombocytopenia. It was observed that some features at diagnosis, such as age younger than five years and a previous history of infection, influenced the probability of cure. Regarding the changes in the management of patients since 2011, the steroid doses received during the first month and the first year, and the number of days corresponding to the patient's first admission have both significantly decreased. Splenectomies were also significantly reduced. CONCLUSIONS: Since 2011, there have been changes in the medical care of our primary immune thrombocytopenia patients: they receive lower doses of steroids, they stay fewer days in the hospital, and the number of splenectomies has decreased without increasing bleeding or worsening the clinical evolution. Furthermore, it was observed that age younger than 5 years and a history of infection prior to diagnosis were related to higher chances of recovery.

Immune thrombocytopenia secondary to COVID-19: a case report. / Trombocitopenia inmune secundaria a COVID-19: reporte de un caso.

BACKGROUND: The SARS-CoV-2 virus, which emerged in late 2019 in Wuhan province, China, has shown a wide spectrum of manifestations, some of which it shares with some autoimmune disorders. Until now, the association of SARS-CoV-2 with immune thrombocytopenia (ITP) is poorly understood, since the mechanisms that explain the complex relationship between these two prothrombotic states are still unknown. CLINICAL CASE: It is presented the case of a 66-year-old woman, with no chronic disease history, who arrived at the Emergency Department due to respiratory symptoms. It was carried out a polymerase chain reaction with reverse transcriptase, which was positive for SARS-CoV-2. Isolated thrombocytopenia was found in laboratory studies, which was protocolized by ruling out other causes and finally diagnosing ITP secondary to COVID-19. Intravenous steroids treatment was given observing a complete recovery in platelet levels. CONCLUSIONS: Until now, the association of IPT and COVID-19 is unclear and presents challenges in treatment. Steroids continue to be the first choice with the indication to reduce their dose and duration to the minimum necessary. Other second-line treatments present more questions than answers in the context of this pandemic.

INTRODUCCIÓN: el virus del SARS-CoV-2, el cual surgió a finales de 2019 en la provincia de Wuhan, China, ha mostrado un espectro amplio de manifestaciones, algunas de las cuales comparte con algunos trastornos autoinmunes. La asociación del SARS-CoV-2 con la trombocitopenia inmune (TIP) es hasta ahora poco entendida, pues todavía no se conocen los mecanismos que expliquen la compleja relación entre estos dos estados protrombóticos. CASO CLÍNICO: se presenta el caso de una mujer de 66 años, sin antecedentes crónicos, que acudió al Servicio de Urgencias por sintomatología respiratoria. Se realizó prueba de reacción en cadena de la polimerasa con transcriptasa reversa, la cual fue positiva para SARS-CoV-2. En los estudios de laboratorio se encontró trombocitopenia aislada que se protocolizó, por lo que se descartaron otras causas de esta y se diagnosticó TIP secundaria a COVID-19. Se inició tratamiento con esteroides intravenosos y se obtuvo una respuesta completa. CONCLUSIONES: la asociación de TIP y COVID-19 es hasta ahora poco clara y plantea retos en el tratamiento. Los esteroides continúan siendo de primera elección con la indicación de disminuir su dosis y duración a las mínimas necesarias. Otros tratamientos de segunda línea plantean más dudas que respuestas en el contexto de esta pandemia.

[Primary immune thrombocytopenia: Experience of a specialised clinic]. / Trombocitopenia inmune primaria: experiencia de una consulta especializada.

INTRODUCTION: Although primary immune thrombocytopenia (ITP) is rare in childhood, it is the most frequent cause of thrombocytopenia. There have been attempts to establish risk factors to predict the progression of the disease in order to optimise its management, which has changed in recent years due to, among other reasons, specialised care. MATERIAL AND METHODS: A retrospective, observational and analytical study was conducted on patients diagnosed with ITP over a 3-year period in a Paediatric Haematology specialist clinic. RESULTS: From the epidemiological, clinical and analytical point of view, the characteristics of this group are similar to others. Most of the patients (23/31, 74.2%) had ITP for less than 12 months, with there being no serious complications related to the disease or the treatment received. It was established that risk factors were related to being slowly evolving (lower event-free survival (EFS)) with no statistical significance, female gender, age over 10 years, leukopenia absence of initial severe thrombocytopenia, and non-specialised care. The absence of a history of infection was significantly related to a lower EFS. CONCLUSIONS: The epidemiological and analytical risk factors for a slowly evolving ITP are the same that described in the literature. Patients treated before the beginning of specialised care also had a lower EFS. These data seem to support the current recommendation that rare diseases should be managed in specialised units.

Assessment of the importance of platelet transfusion in patients with severe dengue: a systematic review / Evaluación de la importancia de la transfusión de plaquetas en pacientes con dengue grave: una revisión sistemática

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)

Trombocitopenia inmunitaria, como efecto adverso a vacuna frente a COVID-19, descripción de dos casos y revisión de bibliografía / Immune thrombocytopenia, as an adverse effect of the COVID-19 vaccine. Two 2 case studies and literature review

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)

Alteraciones hematológicas en el paciente gran quemado

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.

[Protocol for the study and treatment of primary immune thrombocytopenia: PTI-2018]. / Protocolo de estudio y tratamiento de la trombocitopenia inmune primaria: PTI-2018.

Primary immune thrombocytopenia, formerly known as immune thrombocytopenic purpura, is a disease for which the clinical and therapeutic management has always been controversial. The ITP working group of the Spanish Society of Paediatric Haematology and Oncology has updated its guidelines for diagnosis and treatment of primary immune thrombocytopenia in children, based on current guidelines, bibliographic review, clinical assays, and member consensus. The main objective is to reduce clinical variability in diagnostic and therapeutic procedures, in order to obtain best clinical results with minimal adverse events and good quality of life.

Thrombocytopenia in pregnancy, a challenge in the intensive care unit (ICU). / Trombocitopenia en el embarazo, un desafío en la unidad de cuidados intensivos (UCI).

Thrombocytopenia is a common alteration during pregnancy and the multiple possible aetiologies make the differential diagnosis challenging. Physiological changes of pregnancy can mask severe conditions until advanced stages. This case refers to a pregnant woman who presented fever and thrombocytopenia during labour. In the postpartum period she developed rapidly progressive hemodynamic instability requiring admission to the ICU. There was progression to acute kidney injury, persistent thrombocytopenia and platelet dysfunction. The differential diagnosis included DIC in the context of sepsis, PPH and other causes of thrombotic microangiopathies in pregnancy.

Validez de herramientas diagnósticas en pacientes pediátricos hospitalizados con diagnóstico presuntivo de dengue en un Hospital de Referencia de Paraguay / Validity of diagnostic tools in pediatric patients hospitalized with a presumptive diagnosis of dengue in a Reference Hospital in Paraguay

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.

Anormalidades hematológicas en enfermedad hepática, fisiopatología y consideraciones terapéuticas / Hematologic disorders in liver failure: pathophysiology and therapeutic considerations

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.

Aplasia medular asociada a osimertinib: a propósito de un caso / Bone marrow aplasia associated with osimertinib: a case report

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)

MYH9 Associated nephropathy. / Nefropatía asociada a mutación del gen MYH9.

MYH9 related diseases are caused by mutations in the MYH9 gene and constitute a rare group of genetic entities. Its inheritance follows an autosomal dominant pattern. The MYH9 gene, encodes the nonmuscle myosin heavy chain IIA, expressed in different tissues and especially in podocytes and mesangial cells. The disorder is characterized by the presence of macrothrombocytopenia, leukocyte inclusions and a variable risk of developing renal failure, hearing loss and early-onset cataracts. We describe the case of a 27-year-old Caucasian woman, diagnosed initially with idiopathic thrombocytopenic purpura. After a detailed family history and the appearance of renal involvement and hearing loss, genetic testing allowed to make the diagnosis of nephropathy associated with MYH9 mutation. This case is an example of the delayed diagnosis of uncommon diseases and highlights the usefulness genetic testing. A review of the disease is provided.