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.

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.

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.

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.

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.

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.

Clinical Dilemmas in Immune Thrombocytopenic Purpura With Diffuse Alveolar Hemorrhage: Diagnosis, Treatment, and Outcomes.

This report elucidates a unique case of a 39-year-old female with immune thrombocytopenic purpura (ITP) who developed a rare and severe complication: diffuse alveolar hemorrhage (DAH). Despite initial treatments for ITP, the patient experienced fluctuating platelet (PLT) counts and shortness of breath, which were later identified as symptoms of DAH. An urgent splenectomy improved the patient's platelet counts and overall condition. This case underscores the imperative to recognize DAH as a possible ITP complication, requiring clinicians' vigilance for prompt diagnosis and intervention. The intricate nature of ITP in adults necessitates individualized, patient-centered treatment approaches to enhance outcomes. This report provides invaluable insights into the clinical understanding and management of ITP and its complications through detailed analysis and documentation of the patient's treatment journey.

Dengue Infection Triggering Concurrent Thrombotic Thrombocytopenic Purpura in a Case of Chronic Idiopathic Thrombocytopenic Purpura.

We present a case report detailing the medical history of a 53-year-old female who had a well-established 10-year history of idiopathic thrombocytopenic purpura (ITP). The patient presented with fever and gum bleeding, prompting a series of laboratory investigations. These examinations revealed concurrent thrombocytopenia and hemolytic anemia, alongside a positive test result for serum dengue IgM antibodies. Initial treatment for the patient involved intravenous administration of glucocorticoids and intravenous immunoglobulin. Regrettably, this therapeutic intervention did not yield a favorable response. Subsequent clinical developments, including the onset of generalized tonic-clonic seizures, raised suspicions of thrombotic thrombocytopenic purpura (TTP). A notable diagnostic indicator was the elevated PLASMIC score (platelet count; combined hemolysis variable; absence of active cancer; absence of stem-cell or solid-organ transplant; mean corpuscular volume; international normalized ratio; creatinine), reinforcing the consideration of TTP. To confirm the diagnosis, ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) enzyme levels were assessed and found to be low. Consequently, the patient was diagnosed with TTP. Plasmapheresis was administered, resulting in a positive clinical response after two cycles. Notably, the patient experienced a resolution of thrombocytopenia and hemolytic anemia. Following successful treatment, the patient was discharged with a prescription for immunosuppressants. This case underscores the critical importance of including TTP as a potential differential diagnosis when encountering patients with chronic ITP. TTP is characterized by its acute and life-threatening nature, often deviating from the typical clinical presentation. The application of the PLASMIC score serves as a valuable tool in guiding decision-making processes when TTP is suspected.

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

Graves' disease-induced immune thrombocytopenic purpura in an African female: a case report.

BACKGROUND: Immune thrombocytopenic purpura is a condition associated with an unusual, unexplained, and sometimes very severe reduction in the level of platelets in the blood. Though documented, its association with Graves' disease is not very common and can easily be missed or misdiagnosed, leading to excessive bleeding and mortality. Treatment with steroids and antithyroid medications has been shown to be beneficial in correcting thrombocytopenia in these patients, although the response is varied. We report on a patient with Graves' disease who presents with immune thrombocytopenic purpura. CASE PRESENTATION: A 37-year-old Ghanaian female presented to our hospital's emergency department with a complaint of palpitations, difficulty breathing, easy fatigue, and headaches. She had been referred from a peripheral hospital as a case of thrombocytopenia, severe anemia, and anterior neck swelling. She was diagnosed with Graves' disease 2 years ago, became euthyroid during treatment, but defaulted. On further examination and investigation, she was diagnosed with immune thrombocytopenic purpura and was also found to have elevated free T3 and T4, and suppressed thyroid stimulating hormone. She also had high thyroid autoantibodies. She was initially started on oral prednisolone but there was no stabilization of platelets until methimazole was introduced, which improved and normalized her platelet count. CONCLUSION: The association of Graves' disease with immune thrombocytopenic purpura, though documented, is uncommon, and very few cases have been reported thus far. There have not been any reported cases in Ghana or Sub-Saharan Africa and hence, clinicians should be aware of this association when investigating immune thrombocytopenic purpura and should consider Graves' disease as a possible cause. From this study, we observed that there was no improvement in platelet count following the use of corticosteroid therapy until methimazole was started.

Spinal subdural hematoma in a patient with immune thrombocytopenic purpura following microvascular decompression: a rare case report.

This article reports a case of spontaneous spinal subdural hematoma (SSDH) after brain surgery in a patient with immune thrombocytopenic purpura (ITP), reviews the relevant literature, and discusses the etiology, pathogenesis, and clinical features of SSDH in patients with ITP. A male patient in his early 50 s with an 8-year history of ITP and suffering from coexistent hemifacial spasm and trigeminal neuralgia underwent microvascular decompression in our department. His preoperative corrected platelet count was within the normal range. On postoperative day 2, the patient complained of acute low back pain and sciatica. Lumbar magnetic resonance imaging demonstrated an SSDH extending from L3 to L4 with a significantly decreased platelet count (30.0 × 109/L). The pain was gradually relieved after 2 weeks of conservative treatment, and no neurological deficit occurred during the 1-year follow-up. Brain surgery may increase the risk of postoperative SSDH in patients with ITP. Clinicians planning brain surgery must conduct a rigorous assessment through detailed physical examination, laboratory tests, and medical history records and maintain perioperative platelet counts within the normal range to prevent various risks associated with spinal cord compression.

Immune thrombocytopenia in infants: a retrospective study with comparison to toddlers.

Introduction: Immune thrombocytopenia (ITP) is the most common cause of acquired bleeding in childhood, but little is known about the clinical course and outcomes in infants with ITP. Methods: This is a retrospective study of all infants (1-12 months of age) and toddlers (13-47 months of age) diagnosed with ITP from a single centre during a 13-year period. The following data were compared between the two patients groups: demographics, severity of bleeding, platelet counts, duration of illness, development of chronic ITP, treatment and association with recent vaccination. Results: Twenty-two infants and 30 toddlers were diagnosed and followed up for ITP during the study period. Infants with ITP generally had minor or mild bleeding (19, 86.4%) and seldom required treatment (7, 31.8%), and their thrombocytopenia resolved at a mean of 1.90 months after diagnosis. Besides age, the sex ratio, severity of bleeding, platelet counts and proportion that required treatment were comparable between infants and toddlers. Fewer infants developed chronic ITP (1/22 vs. 9/30, P = 0.032), but more infants had a history of vaccination in the preceding 6 weeks prior to diagnosis of ITP (13/22 vs. 1/30, P < 0.001). Conclusion: ITP in infants is almost always a self-limiting and transient illness, and the majority of cases do not require treatment.

Advances in management of pediatric chronic immune thrombocytopenia: a narrative review.

Immune thrombocytopenia (ITP) is a disease in which thrombocytopenia occurs because of immune-mediated platelet destruction and decreased platelet production. Although many pediatric patients with ITP experience spontaneous remission or reach remission within 12 months of first-line therapy, approximately 20% progress to chronic ITP. Patients who do not respond to first-line treatment or experience frequent relapses are of great concern to physicians. This review summarizes recent treatments for second-line treatment of pediatric chronic ITP.

Immune thrombocytopenic purpura secondary to COVID-19 vaccination: A systematic review.

INTRODUCTION: This systematic review aimed to retrieve patients diagnosed with de novo immune thrombocytopenic purpura (ITP) after COVID-19 immunization to determine their epidemiological characteristics, clinical course, therapeutic strategies, and outcome. MATERIALS AND METHODS: We conducted the review using four major databases, comprising PubMed, Scopus, Web of Science, and the Cochrane library, until April 2022. A systematic search was performed in duplicate to access eligible articles in English. Furthermore, a manual search was applied to the chosen papers' references to enhance the search sensitivity. Data were extracted and analyzed with the SPSS 20.1 software. RESULTS: A total of 77 patients with de novo COVID-19 vaccine-associated ITP were identified from 41 studies, including 31 case reports and 10 case series. The median age of patients who developed COVID-19 vaccine-associated ITP was 54 years (IQR 36-72 years). The mRNA-based COVID-19 vaccines, including BNT16B2b2 and mRNA-1273, were most implicated (75.4%). Those were followed by the adenovirus vector-based vaccines, inclusive of ChAdOx1 nCoV-19 and vAd26.COV2.S. No report was found relating ITP to other COVID-19 vaccines. Most cases (79.2%) developed ITP after the first dose of COVID-19 vaccination. 75% of the patients developed ITP within 12 days of vaccination, indicating a shorter lag time compared to ITP after routine childhood vaccinations. Sixty-seven patients (87%) patients were hospitalized. The management pattern was similar to primary ITP, and systemic glucocorticoids, IVIg, or both were the basis of the treatment in most patients. Most patients achieved therapeutic goals; only two individuals required a secondary admission, and one patient who presented with intracranial hemorrhage died of the complication. CONCLUSIONS: De novo ITP is a rare complication of COVID-19 vaccination, and corresponding reports belong to mRNA-based and adenovirus vector-based vaccines, in order of frequency. This frequency pattern may be related to the scale of administration of individual vaccines and their potency in inducing autoimmunity. The more the COVID-19 vaccine is potent to induce antigenic challenge, the shorter the lag time would be. Most patients had a benign course and responded to typical treatments of primary ITP.

A Case of Off-Pump Coronary Artery Bypass Grafting Following High-Dose Dexamethasone Therapy in a Patient with Idiopathic Thrombocytopenic Purpura / 日本心臓血管外科学会雑誌

Man in his 70s, who had suffered from idiopathic thrombocytopenic purpura (ITP), was admitted to our hospital with chest pain at rest. Coronary angiography revealed obstruction of the right coronary artery and triple vessel disease. Because a bleeding tendency was expected during coronary artery bypass grafting, we performed percutaneous coronary intervention to the culprit lesion first, and then intravenous immunoglobulin and high dose dexamethasone were tried. His platelet count rose from 49,000 to 103,000/mm3, so we performed coronary artery bypass grafting. The patient had no postoperative hemorrhagic complications. We believe that high dose dexamethasone therapy is useful for patients with ITP who need surgery immediately.

Splenectomy in patients with immune (idiopathic) thrombocytopenic purpura (ITP) appears to be protective against developing aortic valve disease.

BACKGROUND: Immune thrombocytopenia (ITP) has been shown to be independently associated with aortic valve disease (AVD). However, whether ITP patients who have undergone splenectomy are also at increased risk for AVD has not been researched. The goal of this study was to evaluate any association between AVD and splenectomy in patients with ITP. METHOD: We used the Nationwide Inpatient Sample from 2005 to 2014 as 10 consecutive years randomly selected. Using ICD-9 codes for AVD, ITP, and splenectomy, a total of 108,434 patients were identified with ITP, 4,282 of which had undergone splenectomy. We performed uni- and multivariate analysis adjusting for baseline characteristics. RESULTS: Univariate analysis revealed a significantly lower rate of AVD in ITP patients with splenectomy compared to no splenectomy in 2007, 2009, and 2010 with a trend of this association during the other years. For example, in 2007, 0.6% of ITP patients with history of splenectomy had AVD versus 2.0% of ITP patients without splenectomy (OR, 0.29; 95% CI, 0.09-0.91; P = 0.02). Similarly, in 2010, 0.2% of ITP patients with history of splenectomy had AVD versus 1.9% of ITP patients without splenectomy (OR, 0.13; 95% CI, 0.02-0.92; P = 0.02). After adjusting for age, gender, race, diabetes, hypertension, hyperlipidemia, and tobacco use, we confirmed that ITP patients with splenectomy have no association with prevalence of aortic valve disease (2005: OR, 0.48; 95% CI, 0.18-1.30; P = 0.15; 2014: OR, 0.88; 95% CI, 0.36-2.16; P = 0.77). CONCLUSION: Based on a large inpatient database, our previous finding of ITP patients' association with AVD is only present in patients without splenectomy, and splenectomy appears to exert a protective effect on developing aortic valve disease in ITP patients, warranting further investigation.

Sheng-Xue-Xiao-Ban Capsule-induced ischemic colitis and pulmonary embolism in an idiopathic thrombocytopenic purpura patient: a rare case report.

Background: Sheng-Xue-Xiao-Ban Capsule (SXXBC), as a classic Chinese traditional medicine comprised of natural indigo, cortex moutan, forsythia, herba agrimoniae, and licorice, exhibits a heat-clearing and detoxicating function, hemostasis, and stasis dissipation, which is widely applied to treat idiopathic thrombocytopenic purpura (ITP). However, report on ischemic colitis and pulmonary embolism induced by SXXBC therapy is never disclosed. We report the case of an ITP patient who received SXXBC for ascending platelets that then induced ischemic colitis and pulmonary embolism. Case Description: A 74-year-old female patient was admitted in June 2021 due to "bleeding in stool for 1 day," she was then re-admitted in July 2021 due to "repeated bleeding in stool for 2 days". Abdominal computed tomography (CT), colonoscopy, and a pathological examination suggested ischemic colitis according to the American College of Gastroenterology (ACG) clinical guidelines. Pulmonary artery CT angiography suggested pulmonary embolism reflected by multiple filling defects, and the patient presented with shortness of breath. It was noted that the patient had started taken SXXBC for ascending platelets 2 months before the onset of hematochezia. After the diagnosis of hematochezia was made, the patient received phenethylamine and carbazochrome for hemostasis, mesalazine enteric-coated tablets for anti-inflammation, and SXXBC was stopped. The hematochezia then ceased, and the ischemic colitis was attenuated. Afterwards, low-molecular-weight heparin was administered, followed by a 3-week treatment of rivaroxaban anticoagulant, which was taken orally after discharge. The pulmonary embolism was then obviously ameliorated. After excluding other causes, the patient was diagnosed with SXXBC-induced ischemic colitis complicated by pulmonary embolism. After conducting research, we came to the view that natural indigo, which is the main component of SXXBC, contributed to the patient's illness. Conclusions: Ischemic colitis complicated with pulmonary embolism are rare; however, close attention such as regular abdominal CT test needs to be paid and preventive steps such as anti-coagulant treatment could to be taken (if symptoms occur) when treating patients with SXXBC.