A case of TAFRO syndrome after vaccination, successfully treated with cyclosporine.

BACKGROUND: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine. CASE PRESENTATION: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission. CONCLUSIONS: Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.

Hypermethylation of the FOXP3 gene regulates Tregs immunodysregulation in chronic idiopathic thrombocytopenic purpura.

BACKGROUND: Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by a breakdown of immune tolerance; in ITP, the body's immune system mistakenly attacks and destroys platelets. This study aims to investigate the role and underlying mechanisms of FOXP3 in chronic ITP. METHODS: Flow cytometry was used to detect the proportion of CD4+CD25+FOXP3+ regulatory T cells (Tregs) in CD4+CD25+ T lymphocytes from 20 patients with chronic ITP (CITP), 20 acute ITP (AITP) controls, and 20 healthy individuals.CD4+CD25+ Treg cells were isolated from peripheral blood of patients with CITP using magnetic beads and then treated with phosphate-buffered saline solution or decitabine (a methylation inhibitor) for 48 h. The levels of interleukin-2 (IL-2), IL-10, and transforming growth factor-beta1 (TGF-ß1) in the plasma and CD4+CD25+ Treg cells were assessed by Enzyme-linked-immunosorbent serologic assay and quantitative real-time polymerase chain reaction (qRT-PCR). FOXP3 level was measured by qRT-PCR and Western blot analysis. Methylation-specific PCR (MS-PCR) was adopted to detect the status of FOXP3 methylation. RESULTS: The number of Treg cells and the contents of IL-2, IL-10, and TGF-ß1 decreased in patients with CITP, compared to the AITP control group and normal group. FOXP3 expression was reduced and FOXP3 methylation increased in patients with CITP, compared to the AITP control group and normal group. Hypermethylation of FOXP3 promoter led to decrease in FOXP3 level in Treg cells. Inhibition of FOXP3 promoter hypermethylation promoted the secretion of IL-2, IL-10, and TGF-ß1 in Treg cells. CONCLUSION: The number of Treg cells in CITP patients decreased, and the hypermethylation of FOXP3 promoter led to reduction of its expression in Treg cells, thus affecting the immune functioning of Treg cells.

Gut microbiome composition and dysbiosis in immune thrombocytopenia: A review of literature.

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by excessive reticuloendothelial platelet destruction and inadequate compensatory platelet production. However, the pathogenesis of ITP is relatively complex, and its exact mechanisms and etiology have not been definitively established. The gut microbiome, namely a diverse community of symbiotic microorganisms residing in the gastrointestinal system, affects health through involvement in human metabolism, immune modulation, and maintaining physiological balance. Emerging evidence reveals that the gut microbiome composition differs in patients with ITP compared to healthy individuals, which is related with platelet count, disease duration, and response to treatment. These findings suggest that the microbiome and metabolome profiles of individuals could unveil a new pathway for aiding diagnosis, predicting prognosis, assessing treatment response, and formulating personalized therapeutic approaches for ITP. However, due to controversial reports, definitive conclusions cannot be drawn, and further investigations are needed.

Case report: Management of pregnancy-associated immune thrombocytopenia in a French bulldog with dystocia.

Introduction: The objective of this case report is to describe diagnosis and management of life-threatening immune thrombocytopenia (ITP) secondary to pregnancy in a dog with concurrent dystocia. Case summary: A 1-year 11-month old female intact French bulldog was referred for management of severe thrombocytopenia and spontaneous hemorrhage during whelping. The thrombocytopenia was progressive from approximately 32 days of gestation. In the absence of an identifiable cause for the thrombocytopenia, the patient was treated for ITP with immunosuppressive therapies and blood and plasma transfusions. The patient was also supported through dystocia until the platelet count normalized so a Caesarean section and ovariohysterectomy (OVH) could be performed. Discussion: This is the first report documenting ITP in a whelping canine. Pregnancy is a known trigger and can affect the clinical course of autoimmune diseases in women, including ITP. It is suspected that this patient's pregnancy triggered ITP, paralleling what occurs in women.

A Case of Myosin Heavy Chain 9-Related Disorder Following Splenectomy Due to Misdiagnosis of Idiopathic Thrombocytopenic Purpura.

This case study reports a patient with Myosin Heavy Chain 9 (MYH9)-related disorder (MYH9-RD) which is characterized by congenital macrothrombocytopenia, Döhle-like bodies, sensorineural hearing loss, cataracts, and glomerulopathy. Often misdiagnosed as idiopathic thrombocytopenic purpura (ITP), MYH9-RD requires accurate identification to avoid inappropriate treatments like steroids, rituximab, or splenectomy. Platelet transfusions were traditionally the only therapeutic option, but thrombopoietin receptor agonists (TPO-RA), specifically eltrombopag, have shown success in MYH9-RD treatment. The case report involves a 27-year-old male with chronic ITP post-splenectomy, revealing thrombocytopenia, mild anemia, giant platelets, kidney failure, and hearing loss. Genetic testing identified a c.287C>T; p.(Ser96Leu) variant associated with MYH9-RD. Eltrombopag treatment, initiated before the definitive diagnosis, exhibited clinical and laboratory success. The study discusses the evolving landscape of treatments for inherited thrombocytopenias, emphasizing eltrombopag's efficacy, especially post-splenectomy, and its potential application in short-term preparations for elective surgeries. The study underscores the importance of timely MYH9-RD diagnosis, preventing misdiagnoses and inappropriate treatments. Eltrombopag stands out as a potential therapeutic option, offering effective platelet count management, especially post-splenectomy, with ongoing research exploring alternative TPO-RAs. As MYH9-RDs are rare, increased awareness among healthcare professionals is crucial to ensure accurate diagnoses and optimal patient care.

Safety and efficacy of danazol in immune thrombocytopenia: a systematic review.

Background: The treatment landscape for relapsed or refractory immune thrombocytopenia (ITP) after corticosteroids is complex. Objectives: We aimed to assess the efficacy of danazol in treating ITP and evaluate the safety and adverse events following its administration. Methods: We searched the databases PubMed, EMBASE, and ClinicalTrials.gov for all published studies assessing danazol's efficacy and safety in treating ITP. The retrieved studies were screened by title and abstract, followed by full-text screening based on the eligibility requirements. The quality assessment was performed using a set of questionnaires. The data were extracted on the descriptive characteristics of the studies and participants, drug dosage, efficacy measures, and adverse effects, and the data were synthesized. Results: A total of 17 studies consisting of 901 participants were included. The overall response rate is around 61% in this analysis. Among the participants, 315 (34.9%) were men. The age of participants ranged from 16 to 86 years. Danazol combined with other pharmacologic interventions, including all-trans-retinoic acid or glucocorticoids, generated better results. The most common side effects appear to be liver injury and elevation of liver enzymes, weight gain, oligomenorrhea, amenorrhea, and myalgia. Conclusion: Danazol at low-to-medium doses was well tolerated and succeeded in improving ITP. Danazol therapy may be helpful in the treatment of chronic ITP that is corticosteroid refractory and when corticosteroids or splenectomy (or both) is contraindicated. Danazol can be considered for further research and development in treating primary immune thrombocytopenia.

Rapid, Simple and Low-Cost Analytical Method Development for Quantification of Eltrombopag Olamine in Tablet Dosage by UV Spectroscopy Method.

BACKGROUND: Eltrombopag Olamine is a drug used to treat thrombocytopenia, a disorder where blood platelet counts get lower and severe aplastic anemia. It serves as a thrombopoietin receptor agonist, which give rise to platelet production in the bone marrow. OBJECTIVES: The objective of this study is to develop a simple, specific, accurate, precise and economical Ultraviolet spectroscopy method to estimate the amount of Eltrombopag Olamine in bulk and tablet dosage form. METHODS: The developed method was performed using methanol for identification and physicochemical characterization of the drug. The validation parameters like linearity, precision, accuracy, robustness limits of detection and quantitation, and specificity were assessed as per ICH Q2 (R2). RESULTS: The maximum absorbance wavelength (λmax) of the drug was found at 247 nm in methanol. The linearity was found in the concentration range of 2-14 µg/ml with regression equation y = 0.0619x - 0.0123 and r² = 0.999. The standard addition method was used to determine the accuracy of the developed method. The result was found in the % recovery range of 98-99%. The precision was done on λmax with respect to the parameters such as repeatability, intraday, and interday. The method was found to be precise as the % RSD value was found to be <2%. The detection limit value (LOD) and quantitation limit value (LOQ) were 0.0524 µg/ml and 0.1588 µg/ml, respectively. CONCLUSION: The developed method is simple, economical, accurate and selective. The developed method was adaptable for the estimation of Eltrombopag Olamine analysis in pharmaceutical dosage form and routine quality control laboratory.

Trombosis venosa cerebral: una etiología rara / Cerebral Venous Thrombosis – A Rare Etiology

Idiopathic thrombocytopenic purpura is an immune-mediated disease that courses with thrombocytopenia. Eltrombopag is a thrombopoietin receptor agonist used as a second-line treatment for this disease to increase platelet count. The association of this drug with thrombotic events is known, however, with few cases of venous sinus thrombosis described in the literature. We present a 26-year-old female with idiopathic thrombocytopenic purpura diagnosed at the of age 14, splenectomized and previously treated with immunoglobulins, corticosteroids, and rituximab, with no response, who was started on oral estroprogestative and eltrombopag, with multiple dose increases, for 3 months. She was admitted to the emergency room with a 3-day history of severe frontal headache, with phono and photophobia, nausea, vomiting, and confusion. Physical examination was remarkable only for mild paraphasias, anomalous pauses, and difficulty in reading. Routine labs showed mild thrombocytopenia, leukocytosis, and elevated C-reactive Protein. She performed a brain computerized tomography and magnetic resonance that demonstrated extensive venous sinus thrombosis. She was admitted to the ward with eltrombopag suspension and enoxaparin 1mg/kg bid. Increasing thrombocytosis was observed for nine days, with a subsequent decrease to normal levels. Sequenced cerebral tomography showed hemorrhage reabsorption. At discharge, there was no improvement of neurological deficits and dabigatran 150mg bid was started for secondary prophylaxis. (AU)

La púrpura trombocitopénica idiopática es una enfermedad inmunomediada que cursa con trombocitopenia. Eltrombopag es un agonista del receptor de trombopoyetina que se utiliza como tratamiento de segunda línea de esta enfermedad para aumentar el recuento de plaquetas. Sin embargo, se conoce la asociación de este fármaco con acontecimientos trombóticos, con pocos casos de trombosis del seno venoso descritos en la literatura. Presentamos el caso de una mujer de 26 años con púrpura trombocitopénica idiopática diagnosticada a los 14 años, esplenectomizada y tratada previamente con inmunoglobulinas, corticoides y rituximab, sin respuesta, a la que se inició tratamiento con estroprogestativos orales y eltrombopag, con múltiples incrementos de dosis, durante 3 meses. Ingresó en urgencias con un cuadro de 3 días de evolución de cefalea frontal intensa, con fono y fotofobia, náuseas, vómitos y confusión. En la exploración física sólo destacaban parafasias leves,pausas anómalas y dificultad para leer. Los análisis de rutina mostraron trombocitopenia leve, leucocitosis y proteína C reactiva elevada. Se le realizó una tomografía computarizada cerebral y una resonancia magnética que demostraron una extensa trombosis del seno venoso. Fue ingresada en planta con eltrombopag en suspensión y enoxaparina 1mg/kg bid. Se observó un aumento de la trombocitosis durante nueve días, con una disminución posterior a niveles normales. La tomografía cerebral secuenciada mostró reabsorción de la hemorragia. Al alta, no hubo mejoría de los déficits neurológicos y se inició dabigatrán 150mg bid para profilaxis secundaria. (AU)

Cuidados ao portador de púrpura trombocitopênica idiopática - revisão de literatura / Care for patients with idiopathic thrombocytopenic purpura - literature review

Introdução: Conhecida como púrpura trombocitopênica imunonológica, autoimune ou isoimune, a Púrpura Trombocitopênica Idiopática é uma doença hematológica frequente que pode ser aguda, mais frequente em crianças, ou crônica, quando persiste por mais de um ano, típica em adultos. Objetivo: Identificar na literatura científica dados sobre a doença púrpura trombocitopênica, quanto às manifestações clínicas, diagnóstico, tratamento e cuidados de enfermagem. Material e Método: Estudo embasado em revisão integrativa de literatura com busca realizada na base de dados da Biblioteca Virtual da Saúde, Scientific Electronic Library Online e Google Acadêmico, pelos descritores: "Púrpura Trombocitopênica Idiopática", "Cuidados de Enfermagem", e suas respectivas traduções em inglês: "IdiopaticThrombocytopenic Purpura" e "Nursing Care" em associação com operadores booleanos "OR" e "AND", para ampliar a busca dos artigos, dos últimos cinco anos. Resultados: Foram identificados 121 artigos com potencial de relevância, sendo excluídos 4 por duplicidade, totalizando em 117 artigos. Após leitura do título e resumo foram selecionados 17 artigos e excluídos 100. Em seguida, excluídos 12, por não responderem a questão norteadora do estudo, totalizando em 5 artigos para realizar a síntese qualitativa na íntegra. Há destaque quanto a importância da sistematização da assistência de enfermagem para o paciente acometido pela doença, e sobre os desafios da equipe de enfermagem no procedimento de transfusão sanguínea. Também um relato de experiência descreve como é viver com a doença e o tratamento, uma revisão integrativa sobre as manifestações clínicas da doença e a dificuldade de diagnosticar a PúrpuraTrombocitopênica Idiopática. Conclusão: Púrpura Trombocitopênica Idiopática é considerada uma doença grave e de difícil diagnóstico, pois pode ser confundida com outras doenças. O tratamento é específico, realizado com procedimentos de plasmaférese e transfusões sanguíneas em unidades especializadas e de alta complexidade assistencial. Assim, a equipe de enfermagem precisa estar preparada para os cuidados próprios e o enfermeiro, estimulado a desenvolver maneiras de cuidar e pesquisas com abordagens na doença

Introduction: Known as immunological, autoimmune or isoimmune thrombocytopenic purpura, Idiopathic Thrombocytopenic Purpura is a frequent hematological disease that can be acute, more frequent in children, or chronic, when it persists for more than a year, typical in adults. Objective: To identify in the scientific literature data on thrombocytopenic purpura, regarding clinical manifestations, diagnosis, treatment and nursing care. Material and Method: Study based on an integrative literature review with a search carried out in the database of the Virtual Health Library, Scientific Electronic Library Online and Google Scholar, by the descriptors: "Idiopathic Thrombocytopenic Purpura", "Nursing Care", and their respective English translations: "IdiopaticThrombocytopenic Purpura" and "Nursing Care" in association with Boolean operators "OR" and "AND", to expand the search for articles from the last five years. Results: 121 potentially relevant articles were identified, 4 of which were excluded due to duplicity, totaling 117 articles. After reading the title and abstract, 17 articles were selected and 100 were excluded. Then, 12 were excluded for not answering the guiding question of the study, totaling 5 articles to carry out the qualitative synthesis in full. There is emphasis on the importance of systematizing nursing care for the patient affected by the disease, and on the challenges of the nursing team in the blood transfusion procedure. Also an experience report of what it is like to live with the disease and the treatment, an integrative review on the clinical manifestations of the disease and the difficulty of diagnosing Idiopathic Thrombocytopenic Purpura. Conclusion: Idiopathic Thrombocytopenic Purpura is considered a serious disease and difficult to diagnose, as it can be confused with other diseases. The treatment is specific, carried out with plasmapheresis procedures and blood transfusions in specialized and highly complex care units. Thus, the nursing team needs to be prepared for self-care and the nurse encouraged to develop ways of caring and research with approaches to the disease

Introducción: Conocida como púrpura trombocitopénica inmunológica, autoinmune o isoinmune, púrpura trombocitopénica idiopática es una enfermedad hematológica frecuente que puede ser aguda, más frecuente en niños, o crónica, cuando persiste por más de un año, típica en adultos. Objetivo: Identificar datos sobre la púrpura trombocitopénica en la literatura científica, en cuanto a manifestaciones clínicas, diagnóstico, tratamiento y cuidados de enfermería. Material y Método: Estudio basado en una revisión integrativa de la literatura con búsqueda realizada en la base de datos de la Biblioteca Virtual en Salud, Scientific Electronic Library Online y Google Scholar, por los descriptores: "Idiopathic Thrombocytopenic Purpura", "Nursing Care", y sus respectivas traducciones al inglés: "IdiopaticThrombocytopenic Purpura" y "Nursing Care" en asociación con los operadores booleanos "OR" y "AND", para ampliar la búsqueda de artículos de los últimos cinco años. Resultados: Se identificaron 121 artículos potencialmente relevantes, de los cuales 4 fueron excluidos por duplicidad, totalizando 117 artículos. Después de la lectura del título y el resumen, se seleccionaron 17 artículos y se excluyeron 100. Luego, 12 fueron excluidos por no responder a la pregunta guía del estudio, totalizando 5 artículos para realizar la síntesis cualitativa en su totalidad. Se destaca la importancia de sistematizar los cuidados de enfermería al paciente afectado por la enfermedad y los desafíos del equipo de enfermería en el procedimiento de transfusión sanguínea. Además, un relato de experiencia describe cómo es vivir con la enfermedad y el tratamiento, una revisión integradora sobre las manifestaciones clínicas de la enfermedad y la dificultad de diagnosticar la Púrpura Trombocitopénica Idiopática. Conclusión: La púrpura trombocitopénica idiopática es considerada una enfermedad grave, de difícil diagnóstico, ya que puede confundirse con otras enfermedades. El tratamiento es específico, realizado con procedimientos de plasmaféresis y transfusiones de sangre en unidades especializadas de alta complejidad asistencial. Así, el equipo de enfermería necesita estar preparado para el autocuidado y el enfermero incentivado a desarrollar formas de cuidar e investigar con abordajes de la enfermedad

Púrpura trombocitopénica inmunológica tras vacunación contra COVID-19

RESUMEN Se presenta el caso de un paciente masculino con púrpura trombocitopénica inmunológica que ocurre 10 días después de la aplicación de la vacuna contra la COVID-19. Se descartaron con estudios complementarios todas las causas secundarias. Con pulsos de corticoides presentó mejoría clínica y laboratorial, evolucionando favorablemente. Asumiendo la relación temporal con dicha vacuna, se presume que esta plaquetopenia fue una reacción adversa a la misma. En el país no se han notificado casos de púrpura trombocitopénica inmunológica hasta el último boletín informativo de reacciones adversas relacionadas a esta vacuna.

ABSTRACT We present the case of a male patient with immunological thrombocytopenic purpura that occurs 10 days after the application of the COVID-19 vaccine. All secondary causes were ruled out with complementary studies. With corticosteroid pulses, he presented clinical and laboratory improvement, progressing favorably. Assuming the temporal relationship with the vaccine, it is presumed that this thrombocytopenia was an adverse reaction to it. No cases of immune thrombocytopenic purpura have been reported in the country until the last bulletin of adverse reactions related to this vaccine.

Síndrome de Guillain-Barré y trombocitopenia tras la vacunación contra el SARS-CoV-2 con Moderna. Descripción de un caso / Guillain-Barré syndrome and thrombocytopenia after SARS-CoV-2 vaccination with Moderna. A case report

Introducción: La vacunación masiva contra el virus SARS-CoV-2 constituye una de las principales estrategias en la reducción de la morbimortalidad que presenta dicho virus. No obstante, a lo largo de los últimos meses, su administración también se ha relacionado con diversos efectos adversos raros, pero potencialmente graves. Caso clínico: En el presente artículo describimos el caso de un paciente que desarrolló un síndrome de Guillain-Barré y una púrpura trombocitopénica idiopática nueve días después de la vacunación con la tercera dosis contra el virus SARS-CoV-2 (Moderna), con dos dosis previas de AstraZeneca. Adicionalmente, destaca la presencia de positividad para autoanticuerpos anti-SSA/Ro60 y para anticuerpos inmunoglobulina G anti-GM1 e inmunoglobulina G anti-GM3. Conclusión: Aunque no es posible establecer una relación de causalidad entre la administración del booster de la vacuna y el desarrollo de la enfermedad, es destacable la asociación de dos procesos autoinmunes concomitantes, junto con la positividad en los autoanticuerpos anti-SSA/Ro60, lo cual se ha descrito en la bibliografía en casos de infección del virus SARS-CoV-2.(AU)

Introduction: The massive vaccination against the SARS-CoV-2 virus has demonstrated to be one of the major measures for the reduction of the morbidity and mortality that this virus causes. However, during the last months the administration of the vaccine has been also associated with some rare, but life-threatening, adverse effects. Case report: In this article we describe the case of a patient that developed a Guillain-Barré syndrome and an Idiopathic thrombocytopenic purpura nine days after the vaccination with the third dose for the SARS-CoV-2 virus (Moderna). He had received previously two doses of the AstraZeneca vaccine. Moreover, the patient was positive for auto-antibodies anti-SSA/Ro60 and auto-antibodies IgG anti-GM1 and IgG anti-GM3. Discussion: Even though it is not possible to stablish a clear relation of causality between the administration of the vaccine booster for SARS-CoV-2 and the diseases developed by the patient, the association of two concomitant autoimmune processes is remarkable. As well as the positivity for the auto-antibodies anti-SSA/Ro60, which have been described in the bibliography in cases of SARS-CoV-2 infection.(AU)

Púrpura trombocitopénica idiopática (pti): su diagnóstico a través de lesiones en cavidad oral / Purple Idiopathic Thrombocytopenic (Itp): diagnosis through lesions in the oral cavity

La PTI es una alteración hemorrágica de instalación súbdita, adquirida, que se manifiesta inicialmente con petequias, equimosis o hematomas en piel y mucosas, sangrado nasal y gingival, sin causa aparente. La mucosa bucal puede ser el sitio donde las lesiones se observen con frecuencia y por primera vez. Se reporta el caso de un paciente masculino de 28 años de edad, con manifestaciones clínicas de un cuadro purpúrico, se describen signos, síntomas, terapéutica y manejo estomatológico (AU)

PTI is a hemorrhagic alteration of sudden installation, acquired, which manifests initially with petechiae, esquimosis or bruises on skin and mucosae, nasal and gingival bleeding without apparent cause. Bucal mucosae can be the site where lesions are observed with frequency, and for the first time. The case of a male patient with 28 years of age with clinical manifestationsofpurpuric syndrome is reported, signs, symptoms, therapeutic and stomatological handling are described (AU)

Bazo accesorio secundario a trombocitopenia autoinmune primaria refractaria - Un reto diagnóstico / Accessory spleen resection in refractory primary autoimmune thrombocytopenia - a diagnostic challenge

Resumen En la trombocitopenia autoinmune primaria hay formación de autoanticuerpos contra las membranas plaquetarias causando destrucción plaquetaria esplénica y compromiso clínico consistente en petequias, hemorragias y trombocitopenia. Cuando falla el manejo médico con corticoides e inmunoglobulinas está indicada la realización de esplenectomía, que funciona para remisión parcial de la enfermedad persistente. Realizar la esplenectomía hace necesario el estudio de un bazo accesorio o material esplénico recurrente, que comúnmente se asocia con la pobre respuesta a tratamiento y recaídas de la enfermedad. Se presenta el caso de una paciente de 27 años y antecedentes de trombocitopenia autoinmune primaria quien requirió esplenectomía, con adecuada evolución postoperatoria y remisión de los síntomas por dos años. Posteriormente, presentó recaída clínica y paraclínica, con plaquetas en 11 000 cel/uL, en contexto de trombocitopenia severa sintomática, presencia de gingivorragia, petequias en los miembros inferiores y el abdomen. Se realizó búsqueda activa de bazo supernumerario por medio de estudios complementarios, los cuales evidenciaron bazo accesorio localizado en el ligamento gastro cólico, que fue resecado por vía laparoscópica, con adecuada respuesta y remisión sintomática de la trombocitopenia autoinmune primaria refractaria. En pacientes esplenectomizados con púrpura trombocitopénica idiopática que tengan recaída de su enfermedad se requiere búsqueda activa de bazos supernumerarios, puesto que es una causa frecuente de su reaparición.

Abstract In primary autoimmune thrombocytopenia there is formation of autoantibodies against the splenic platelet membranes causing platelet destruction with clinical deterioration consisting of petechiae, haemorrhages and thrombocytopenia. When the medical management with corticoids and immunoglobulins fails, it is indicated the accomplishment of splenectomy that works for partial remission of the persistent disease. Performing splenectomy requires the study of an accessory spleen or the recurrent splenic material, that is commonly associated with poor response to treatment and frequent relapses of primary autoimmune thrombocytopenia. A case is presented in a 27-year-old patient with a history of primary autoimmune thrombocytopenia requiring splenectomy, with adequate postoperative evolution and remission of symptoms for two years who later presents clinical and paraclinical relapse with platelets at 11,000 cells/uL in the context of severe symptomatic thrombocytopenia, with the presence of gingivorrhagia, petechiae in the lower limbs and abdomen. Active search of supernumerary spleen was performed by means of complementary studies that showed an accessory spleen located in the gastrocolic ligament, which was resected by laparoscopy with adequate symptomatic remission response of the refractory primary autoimmune thrombocytopenia. In splenectomized patients with idiopathic thrombocytopenic purpura who have relapsed their disease, they require an active search for supernumerary spleens since it is a frequent cause of their reappearance.

Complications of idiopathic thrombocytopenic purpura in pregnancy: a review of literature / Complicações da púrpura trombocitopênica idiopática na gravidez: uma revisão da literatura

ABSTRACT INTRODUCTION: Idiopathic thrombocytopenic purpura (ITP) is an acquired immune disorder that causes a reduction in platelet count, called thrombocytopenia. ITP during pregnancy usually presents some complications that may impair the outcome of pregnancy. OBJECTIVE: This literature review aimed to identify the main complications of ITP in pregnancy and its consequences. METHODOLOGY: The bibliographic search was performed through scientific articles available in the Scielo and PubMed databases, of which 64 articles were selected, both in Portuguese and English. RESULTS: The risk of postpartum hemorrhage, placental abruption, and neonatal thrombocytopenia are some complications that may occur during pregnancy. CONCLUSION: Pregnant women must be properly monitored during pregnancy so that there are no major complications.

RESUMEN INTRODUCCIÓN: La púrpura trombocitopénica idiopática (PTI) es una enfermedad autoinmune adquirida que causa un bajo conteo de plaquetas en la sangre denominado trombocitopenia. La PTI durante el embarazo normalmente presenta algunas complicaciones que pueden afectar el resultado del embarazo. OBJETIVO: Esta revisión de literatura tuvo como objeto identificar las principales complicaciones de la PTI en el embarazo y sus consecuencias. MÉTODO: Se llevó a cabo una búsqueda bibliográfica de artículos científicos disponibles en las bases de datos Scielo y PubMed. Se seleccionaron 64 artículos, tanto en inglés como en portugués. RESULTADOS: El riesgo de hemorragia posparto, desprendimiento prematuro de placenta y trombocitopenia neonatal son algunas complicaciones que pueden ocurrir en la gestación. CONCLUSIÓN: Las mujeres embarazadas deben ser debidamente supervisadas durante el embarazo para que no ocurran mayores complicaciones.

RESUMO INTRODUÇÃO: A púrpura trombocitopênica idiopática (PTI) é uma doença autoimune adquirida que causa redução na contagem de plaquetas denominada trombocitopenia. A PTI durante a gestação normalmente apresenta algumas complicações que podem afetar o desfecho da gravidez. OBJETIVO: Esta revisão da literatura teve como objetivo identificar as principais complicações da PTI na gravidez e suas consequências. METODOLOGIA: A pesquisa bibliográfica foi realizada por meio de artigos científicos disponíveis nas bases de dados Scielo e PubMed. Foram selecionados 64 artigos, tanto em inglês quanto em português. RESULTADOS: O risco de hemorragia pós-parto, descolamento prematuro da placenta e trombocitopenia neonatal são algumas complicações que podem ocorrer na gestação. CONCLUSÃO: As gestantes devem ser devidamente acompanhadas durante a gestação para que não ocorram maiores complicações.

A preliminary study on the diagnostic value of infrared thermography in children with idiopathic thrombocytopenic purpura / 生物医学工程学杂志

Idiopathic thrombocytopenic purpura (ITP) is a common bloody disease with a high incidence in children, but its diagnostic method is exclusive diagnosis, and the existing detection techniques are mostly invasive, which may cause secondary injury to patients and also may increase the risk of disease. In order to make up for the lack of the detection method, this study made a preliminary exploration on the diagnosis of children's ITP from the perspective of infrared thermography. In this study, a total of 11 healthy children and 22 ITP children's frontal infrared thermal images were collected, and the pattern characteristic (PFD), average temperature (Troi) and maximum temperature (MAX) characteristics of 7 target areas were extracted. The weighted PFD parameters were correlated with the platelet count commonly used in clinical diagnosis, and the sensitivity and specificity of the weighted PFD parameters for children's ITP were calculated through the receiver operating characteristic curve (ROC). The final results showed that the difference of the weighted PFD parameters between healthy children and ITP children was statistically significant, and the parameters negatively correlated with platelet count. Under the ROC curve, the area under the curve (AUC) of this parameter is as high as 92.1%. Based on the research results of this paper, infrared thermography can clearly show the difference between ITP children and healthy children. It is hoped that the methods proposed in this paper can non-invasively and objectively describe the characteristics of ITP infrared thermal imaging of children, and provide a new ideas for ITP diagnosis.

Exploration on connotation of Zhigancao Decoction formula syndrome from the perspective of modern pathophysiology and severe cases of critical care and its clinical efficacy on cardioversion,maintenance of sinus rhythm,hemostasis,increasing platelets count,and tonifying deficiency / 中国中药杂志

Zhigancao decoction recorded in Treatise on Febrile Disease by Zhang Zhongjing in the Han dynasty have been widely used in treating palpitation and irregular pulse by traditional Chinese medicine physicians for thousands of years. It is all known that Zhigancao Decoction is used to treat consumptive disease. However,why it has been used to treat exogenous febrile disease? According to studies,Fumai Decoctions in Treatise on Differentiation and Treatment of Epidemic Febrile Disease,that was modified based on Zhigancao Decoction,have their names without reality. Serious defects,including unclear diagnosis,curative effect,and prognosis,have been found in ancient and modern medical records about Zhigancao Decoction. The indications of Zhigancao Decoction include atrial premature beats,ventricular premature beats,and viral myocarditis; tachyarrhythmia( supraventricular tachycardia,atrial fibrillation)with long interval or conduction block,during or after severe infection or high fever; chronic consumptive disease due to tumor after radiotherapy and chemotherapy,malignant fluid state of tumor,hematopathy,terminal stage of heart failure after major operation,and acute hemorrhage after control of severe infection and other major diseases; cough,phlegm and asthma due to chronic obstructive pulmonary disease,pulmonary interstitial fibrosis,lung cancer,after lung cancer surgery; increased heart rate and decreased blood pressure due to insufficient capacity after acute blood loss; the symptoms included palpitation,chest tightness,sweating,lassitude,lacking in strength,shortness of breath,syncope,sudden death,cough,expectoration,excessive phlegm,clear and dilute sputum,emaciation,dry and haggard skin,constipation,haemorrhagic,uterine bleeding,enjoy sweet taste,red tongue without moss,knotted pulse,intermittent pulse,thready rapid pulse,and weak pulse. Besides,Zhigancao Decoction has effect on cardioversion and maintenance of sinus rhythm without thrombosis in persistent atrial fibrillation and permanent atrial fibrillation. Zhigancao Decoction could stop bleeding soon for acute upper gastrointestinal bleeding,and achieve positivity of occult blood test; Zhigancao Decoction could promote thrombocytopenia for idiopathic thrombocytopenic purpura,with the number of platelets 1×109/L. Zhigancao Decoction could promote the rise of granulocytic,erythroid and megakaryocytic hematopoietic lines in unexplained severe anemia,thrombocytopenia,and leukocyte reduction. Zhigancao Decoction could treat cough,asthma,and chest tightness in lung cancer and after lung cancer surgery; chronic consumptive disease due to lung cancer after lung cancer surgery,hematopathy and acute blood loss,which all belonged to the scope of consumptive disease. Zhigancao Decoction could ascend platelets,which was considered as " oriental interleukins" for the ancients. Zhigancao Decoction possesses dual-directional regulation on anticoagulant and hemostasis,which was considered as " oriental low molecular heparin" and " oriental proton pump inhibitors". Large dose of Rehmannia glutinosa is the key of the efficacy of Zhigancao Decoction. This study is expected to enrich the guidelines for modern medical diagnosis and treatment. However,the clinical evidence,relevant genes and targeting network need to be deepened in future studies. In conclusion,it may be a shortcut to restore and explain Zhigancao Decoction formula syndromes based on modern pathophysiology and severe cases of critical care.

Trombocitopenia inmune. Guía de diagnóstico y tratamiento / Guideline for diagnosis and treatment of immune thrombocytopenia

El manejo de la trombocitopenia inmune es motivo de discusión en lo concerniente a evolución,diagnóstico, pronóstico y tratamiento. Se han publicado arias guías que expresan distintas opiniones de expertos, pero no existe aún consenso mundial sobre cuál es el manejo más adecuado de la enfermedad. Esta guía establece los criterios para definir el diagnóstico; detalla el plan de estudios de laboratorio por realizar inicialmente; plantea los distintos diagnósticos diferenciales; desarrolla aspectos relativos a evolución y pronóstico, y enumera los tratamientos disponibles para las formas agudas y las crónicas, así como para el manejo de las emergencias y en algunas situaciones especiales.

Management, outcome, diagnosis, prognosis and treatment of immune thrombocytopenia are controversial. Several guidelines stating different experts' opinions have been published; however, no worldwide consensus regarding the management of the disease has still been reached. This guideline defines diagnostic criteria, states initial laboratory tests, establishes differential diagnosis, develops topics concerning outcome and prognosis, and enumerates available treatments for acute and chronic disease, as well as for management of life-threatening bleeding.

Clinical, immunological and genetic characteristic of patients with clinical phenotype associated to LRBA-deficiency in Colombia / Características clínicas, inmunológicas y genéticas de pacientes con el fenotipo asociado a deficiencia LRBA en Colombia

Abstract Background: LPS-responsive beige -like anchor protein (LRBA) deficiency is a primary immunodeficiency disease caused by loss of LRBA protein expression, due to biallelic mutations in LRBA gene. LRBA deficiency patients exhibit a clinically heterogeneous syndrome. The main clinical complication of LRBA deficiency is immune dysregulation. Furthermore, hypogammaglobulinemia is found in more than half of patients with LRBA-deficiency. To date, no patients with this condition have been reported in Colombia Objective: To evaluate the expression of the LRBA protein in patients from Colombia with clinical phenotype associated to LRBA-deficiency. Methods: In the present study the LRBA-expression in patients from Colombia with clinical phenotype associated to LRBA-deficiency was evaluated. After then, the clinical, the immunological characteristics and the possible genetic variants in LRBA or other genes associated with the immune system in patients that exhibit decrease protein expression was evaluated. Results: In total, 112 patients with different clinical manifestations associated to the clinical LRBA phenotype were evaluated. The LRBA expression varies greatly between different healthy donors and patients. Despite the great variability in the LRBA expression, six patients with a decrease in LRBA protein expression were observed. However, no pathogenic or possible pathogenic biallelic variants in LRBA, or in genes related with the immune system were found. Conclusion: LRBA expression varies greatly between different healthy donors and patients. Reduction LRBA-expression in 6 patients without homozygous mutations in LRBA or in associated genes with the immune system was observed. These results suggest the other genetic, epigenetic or environmental mechanisms, that might be regulated the LRBA-expression.

Resumen Antecedentes: la deficiencia de LRBA (del inglés, LPS-responsive beige -like anchor protein) es una inmunodeficiencia primaria causada por la pérdida de la expresión de la proteína LRBA, debido a mutaciones bialélicas en el gen LRBA. Los pacientes con deficiencia de LRBA exhiben un síndrome clínicamente heterogéneo. La principal complicación clínica de la deficiencia de LRBA es la desregulación inmune. Además, la hipogammaglobulinemia se encuentra en más de la mitad de los pacientes con deficiencia de LRBA. Hasta la fecha, no se han reportado pacientes con esta afección en Colombia Objetivo: Evaluar la expresión de la proteína LRBA en pacientes de Colombia con fenotipo clínico asociado a deficiencia de LRBA Métodos: En el presente estudio se evaluó la expresión de LRBA en pacientes de Colombia con fenotipo clínico asociado a deficiencia de LRBA. Después de eso, se evaluaron las características clínicas, inmunológicas y las posibles variantes genéticas en LRBA o en otros genes asociadados con el sistema inmune en pacientes que exhiben una disminución de la expresión de la proteína. Resultados: En total, se evaluaron 112 pacientes con diferentes manifestaciones clínicas asociadas al fenotipo clínico LRBA. La expresión de LRBA varía mucho entre diferentes donantes sanos y pacientes. A pesar de la gran variabilidad en la expresión de LRBA, se observaron seis pacientes con una disminución en la expresión de la proteína LRBA. Sin embargo, no se encontraron variantes bialélicas patógenas o posibles patógenas en LRBA, o en genes relacionados con el sistema inmune. Conclusión: La expresión de LRBA varía mucho entre diferentes donantes sanos y pacientes. Se observó reducción de la expresión de LRBA en 6 pacientes sin mutaciones homocigotas en LRBA o en genes asociados. Estos resultados sugieren los otros mecanismos genéticos, por ejemplo epigenéticos o ambientales, que podrían estar regulados por la expresión de LRBA

Laparoscopic splenectomy for patients with idiopathic thrombocytopenic purpura and Helicobacter pylori / 中华肝胆外科杂志

Objective To evaluate the short-term and long-term outcomes of laparoscopic splenectomy combined with eradication of Helicobacter pylori (HP) in patients with idiopathic thrombocytopenic purpura (ITP).Methods 72 patients with ITP were divided into three groups:the eradication of Hp group (group A),the untreated or failure group (group B),and the Hp-negative group (group C).Results Hppositive Patients (group A and group B) were significantly shorter in the course of disease before splenectomy (26.7± 13.8 months vs.45.2±22.1 months,P＜0.05),and lower in platelet counts peak within 7 days after splenectomy (134.9±53.9) × 109/L vs.(250.9± 160.5) × 109/L,P＜0.05) than Hp-negative patients.After discharge from hospital,in 28 patients who received infection therapy against Hp by taking amoxicillin,CLA,omeprazole for one month,21 (75.0％) patients had the Hp eradicated,but in 7 patients the eradication failed.The PLT between these 2 groups of patients were (189.6± 114.8)× 109/L vs.(124.0±45.7) × 109/L,(P＜0.05).The long-term outcomes in platelet counts and remission rates after spleuectomy of the three groups of patients were (149.7±90.6) × 109/L,76.1％ (group A);(98.5±64.1) × 109/L,66.6％(group B);(172.4± 102.0)× 109/L,80.0％ (group C).The platelet count in group B was significantly lower than group C (P＜0.05).There was no statistical significance between group A and group C (P＞0.05).There was no significant difference in the remission rates in the three groups.Conclusions Eradication of Hp improved the short-term and long-term outcomes of Hp-positive ITP patients after splenectomy.

Effect of Montelukast on T-lymphocyte subsets and advanced oxidation protein products in immune thrombopenic purpura model mice / 中国免疫学杂志

Objective:To investigate the effect of Montelukast on T-lymphocyte subsets, cytokines and advanced oxidation protein products ( AOPP ) in immune thrombopenic purpura ( ITP ) model mice. To analyze the principle of the treatment by Montelukast. Methods: Forty ITP mice were randomly divided into control group,model group,Montelukast low dose group(3 mg/kg) and Montelukast high dose group(12 mg/kg). ITP model mice were successive administration for 14 days after building models for 7 days. Platelet counts,the index of thymus and spleen were calculated. T-lymphocyte subsets were detected by flow cytometry. IL-6,TNF-α,AOPP were detected by enzyme linked immunosorbent assays. Results: Comparison with control group,the PLT,thymus index and spleen index,CD8+,IL-6,TNF-α,AOPP of model group mice were significantly increased (P＜0. 05) while CD3+,CD4+,CD4+/CD8+were significantly decreased (P＜0. 05). Comparison with model group,PLT,thymus index and spleen index,CD8+,IL-6,TNF-α,AOPP of low dose group and high douse group mice were significantly decreased (P＜0. 05) while CD3+,CD4+,CD4+/CD8+were significantly increased (P＜0. 05). Conclusion: Montelukast can cure ITP regulate immune disorders,eliminate accumulation of AOPP and reduce level of IL-6 and TNF-α.