Management of Perioperative Anticoagulation in a Patient with Antiphospholipid Antibody Syndrome Undergoing Cardiac Surgery: A Case Report

Patients with Antiphospholipid syndrome (APLS) are at high risk for both bleeding and thrombotic complications during cardiac surgery involving cardiopulmonary bypass (CPB). In this case we present a patient with APLS and Immune Thrombocytopenic Purpura who successfully underwent aortic valve replacement (AVR) with CPB despite recent craniotomy for subdural hematoma evacuation. Anticoagulation for CPB was monitored by targeting an Activated Clotting Time (ACT) that was 2× the upper limit of normal. A multidisciplinary approach was essential in ensuring a safe and successful operation.

Síndrome de Evans como manifestación inicial de lupus eritematoso sistémico / Evans syndrome as an initial manifestation of systemic lupus erythematosus

El síndrome de Evans se caracteriza por la presentación simultánea de anemia hemolítica autoinmune y púrpura trombocitopénica inmune, puede presentarse como una patología aislada o como manifestación de una enfermedad sistémica. Caso Clínico: Preescolar masculino de 3 años, diagnosticado de síndrome de Evans, requirió tratamiento con corticoides e inmunoglobulina por mala respuesta inmunológica, tres meses después de su diagnóstico inicial presento afectación renal, además de presentar autoanticuerpos positivos, por lo que se estableció diagnóstico de lupus eritematoso sistémico. Conclusión: El síndrome de Evans es una entidad nosológica poco frecuente, ante su diagnóstico se debe descartar enfermedad sistémica subyacente.

Evans syndrome is characterized by the simultaneous presentation of autoimmune hemolytic anemia and immune thrombocytopenic purpura; it can be manifested as an isolated pathology or as a manifestation of a systemic disease. Clinical Case: 3-year-old preschool male, diagnosed with Evans syndrome, that required corticosteroids and immunoglobulin intravenous treatment due to poor immune response. Three months after his initial diagnosis he presents kidney affectation in addition to presenting positive auto-antibodies, with which it was established the diagnosis of systemic lupus erythematosus. Conclusion: Evans syndrome is a rare nosological entity, when the diagnosis is made an underlying systemic disease must be ruled out.

The influences of mothers with moderate and severe gestational thrombocytopenia and primary immune thrombocytopenia on neonates / 中国新生儿科杂志

Objective:To study the effects of maternal moderate and severe gestational thrombocytopenia (GT) and primary immune thrombocytopenia (ITP) on neonates.Method:From Jan 2018 to Dec 2019, pregnant women with platelet count <100×10 9/L during pregnancy admitted to our hospital were retrospectively reviewed. The infants were assigned into GT group and ITP group according to their mothers' diagnoses. The clinical outcomes were compared between the two groups. Result:Of 104 mothers with platelet count <100×10 9/L, 32 (30.8%) were diagnosed with ITP and 72 (69.2%) with GT. Gestational age (GA) of the ITP group was smaller than the GT group [(37.0±1.5) weeks vs. (38.0±2.0) weeks, P<0.05]. The maternal platelet count within 24 h before delivery (39×10 9/L vs. 86×10 9/L) and the lowest platelet count during pregnancy (17×10 9/L vs. 75×10 9/L) in the ITP group were both lower than the GT group, the differences were statistically significant ( P<0.001). The maternal platelet count after birth in ITP group were lower than the GT group (184×10 9/L vs. 277×10 9/L, P<0.01). Neonates in the ITP group have an increased tendency to develop neonatal thrombocytopenia (NT) than the GT group (43.8% vs. 6.9%, P<0.001). The platelet count on the first day after birth (92×10 9/L vs. 170×10 9/L) and the lowest platelet count (43×10 9/L vs. 103×10 9/L) of NT newborns in the ITP group were lower than the GT group ( P<0.05). No differences existed for the time needed reaching the lowest platelet count in NT newborns between the two groups [(3.5±1.2) d vs. (4.4±0.4) d, P>0.05]. Neither group had intracranial hemorrhage. Conclusion:Neonates born to pregnant mother with platelet count <100×10 9/L have a tendency to develop NT. The incidence of NT in neonates born to mothers with ITP is higher than GT, but the overall prognosis of the newborns is good.

Prevalence of H. pylori in Immune Thrombocytopenic Purpura (ITP) in a tertiary care center, South India

Introduction: Immune thrombocytopenic purpura (ITP) has been linked with Helicobacter Pylori infection. Objectives: The objective of the present study was to determine the prevalence of H pylori infection in adult ITP patients in a tertiary care center in South India. Methods: 50 adult patients with ITP in the Department Hematology, Madras Medical College were recruited for a cross -sectional study over a period of 6 months. Biopsy from the antrum was subjected to Rapid urease test to detect H. pylori. A total of 50 patients participated in the study. Result: The prevalence of H. pylori infection in ITP patients was 36%. The majority of patients were male (78%). Conclusion: The diagnosis of H. pylori in ITP patients may be considered in high prevalent areas.

Clinical Features and Treatment Outcomes of Immune Thrombocytopenic Purpura in Infants: A Single Center Retrospective Study / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) in children less than one year of age is less well characterized compared to ITP in toddlers and school-age children. Since children of different ages may have differing clinical courses, better delineation of the natural history of ITP in infants is needed. METHODS: We retrospectively reviewed the admission records of 248 consecutive pediatric patients between 1 month and 15 years of age who were admitted and treated for acute ITP at Pusan National University Children's Hospital from 2009 through 2017. All patients less than 1 year of age were identified and enrolled in this study. We investigated their demographics, clinical features, laboratory examinations, response to treatment, and long-term outcomes and made a comparison to those of children aged 1 to 10 years of age. RESULTS: Ninety nine infants were identified. Male to female ratio was highest in infants and decreased with age. Seventy nine (79.8%) of the 99 infant were found to be under 6 months old. The median platelet counts at diagnosis was 6×10⁹/L. Minor bleeding (bleeding score 0–2) was significantly dominant in infant compared to older subjects. Eighty two (96.5%) out of 85 patients achieved complete remission after initial intravenous immunoglobulin (IVIG) treatment. The relapse rate after initial CR was significantly lower than older ages (P=0.003). The platelet count after IVIG treatment in infant showed more rapid response compared to older subjects (P=0.04). Follow up information at 12 months was available for 70 infants. Chronic ITP at 12 month was seen less frequently in infants than in children 1 to 10 years of age (1.4% vs. 20.2%, P<0.001). CONCLUSION: Infants with acute ITP respond more favorably to IVIG treatment and are less likely to develop chronic ITP compared to children 1 to 10 years of age.

Persistent thrombocytopenia following dengue fever: What should we do?

@#Thrombocytopenia is a common laboratory finding in dengue infection. However, it usually resolves as the patient recovers from the infection. Persistent thrombocytopenia following dengue infection requires further investigation. Here, we present a case of immune thrombocytopenic purpura (ITP) following dengue infection complicated by intracranial bleeding.

Efectividad de Rituximab en el tratamiento de citopenias autoinmunes refractarias en adultos / Effectiveness of Rituximab in the treatment of cytopenias refractory autoimmune in adults

The term autoimmune cytopenias is referred to a heterogeneous group of diseases characterized by a reduced peripheral blood cell counts in one or more cellular series, because an immunological disorder. The first line therapy is steroids, followed by splenectomy or immunesupressant therapy in non-responders. Rituximab is an anti CD20 monoclonal antibody used as a third line in refractory patients or as an alternative to splenectomy. We present a retrospective study of nine patients with autoimmune cytopenias treated in a public hospital setting with rituximab. Five patients with the diagnosis of inmune thrombocytopenic purpura received it, all of them achieved hematological response (4 complete and one partial). The median time to the best response was 6 weeks, staying in this category after 6 months of follow up. Four patients with autoimmune hemolytic anemia received rituximab, three of them achieving partial response and one was lost from follow up. No severe adverse effects related to rituximab were registered.

Prevalencia y caracterización de las enfermedades autoinmunitarias en pacientes mayores de 13 años en un hospital de Colombia / Prevalence and characterization of autoimmune diseases in patients older than 13 years in a hospital of Colombia

Resumen ANTECEDENTES Las enfermedades autoinmunitarias afectan alrededor de 3 a 5% de los seres humanos, especialmente a las mujeres. Por su poca prevalencia se desconocen los datos clínicos locales del comportamiento de esta enfermedad. OBJETIVO Encontrar la prevalencia de las enfermedades autoinmunitarias en el Hospital Universitario de Santander, Santander, Colombia, así como realizar la caracterización clínica y sociodemográfica de las enfermedades. MATERIAL Y MÉTODO Estudio observacional, descriptivo de corte transversal, retrospectivo de la prevalencia de enfermedades autoinmunitarias en el Hospital Universitario de Santander entre 2012 y 2016 en pacientes mayores de 13 años que ingresaron a la institución. RESULTADOS Se encontraron 1463 enfermedades autoinmunitarias con prevalencia de 378 casos por cada 100,000 pacientes mayores de 13 años, atendidos en el Hospital Universitario de Santander y la población con enfermedad autoinmunitaria fue de 0.4%. Las enfermedades más prevalentes fueron la artritis reumatoide, psoriasis, enfermedad de Graves, púrpura trombocitopénica inmunitaria y lupus eritematoso sistémico y los órganos más afectados fueron la piel, la glándula tiroides, el sistema hematológico, el páncreas y el sistema nervioso. CONCLUSIONES Los datos encontrados sugieren que la prevalencia en nuestro medio es similar a la de otras poblaciones; sin embargo, estas prevalencias son multifactoriales y pueden variar entre las diferentes poblaciones.

Abstract BACKGROUND Autoimmune diseases affect around 3-5% of human beings, especially women. Due to its low prevalence, local clinical data on the behavior of this disease are unknown. OBJECTIVE To find the prevalence of autoimmune diseases in the University Hospital of Santander, as well as to carry out the clinical and sociodemographic characterization of the diseases. MATERIAL AND METHOD An observational, descriptive and retrospective cross-sectional study on the prevalence of autoimmune diseases was done at the University Hospital of Santander between 2012 and 2016 in patients over 13 years of age who entered to the institution. RESULTS A total of 1463 autoimmune diseases were found, with a prevalence of 378 cases per 100,000 patients over 13 years of age treated at the University Hospital of Santander and the population with autoimmune disease was 0.4%. The most prevalent diseases were rheumatoid arthritis, psoriasis, Graves' disease, immune thrombocytopenic purpura and systemic lupus erythematosus and the most affected organs were the skin, thyroid gland, hematology system, pancreas and nervous system. CONCLUSIONS The data suggest that the prevalence in our environment is similar to that of studies in other populations; however, these prevalences are multifactorial and may vary among different populations.

The Efficacy of High Dose Dexamethasone Therapy in Children with Immune Thrombocytopenic Purpura / 임상소아혈액종양

BACKGROUND: Few studies of high dose dexamethasone (HD-DXM) therapy in children with immune thrombocytopenic purpura (ITP) have been reported. The purpose of this study is to investigate efficacy and safety of repeated HD-DXM therapy as second-line treatment of ITP in childhood. METHODS: We retrospectively analyzed the medical records of patients < 18 years of age with primary ITP who received more than 2 cycles of HD-DXM therapy from May 2004 to January 2018. HD-DXM was given orally in 4-day pulses every 28 days as a 20–40 mg/1.73 m² daily dose. RESULTS: A total of 26 patients (male, 19; female, 7) were enrolled and their median age was 6 years (range, 1–15). All patients had received previous treatment for ITP. A median 6 cycles (range, 2–19) of HD-DXM was given. On the beginning of HD-DXM therapy, three patients satisfied the criteria for newly diagnosed ITP, 16 for persistent ITP and 7 for chronic ITP. Relapse-free survival (RFS) of responders (n=9) after the last HD-DXM cycle was estimated to be 38.1±17.2%, lasting for a median 9.1 months (range, 5.6–46.2). According to response after the 2nd cycle, RFS of responders (n=13) was significantly higher than non-responders (23.1±11.7% vs. 7.7%±7.4%, P=0.001). The most common adverse event was irritability (30.8%), followed by fatigue (19.2%). CONCLUSION: HD-DXM therapy in children was relatively tolerated and response after therapy was acceptable. More courses of HD-DXM may be feasible in responders after two cycles of HD-DXM.

Poor Prognosis in Idiopathic Thrombocytopenic Purpura-associated Retinopathy

PURPOSE: To report a case of retinopathy in a patient with chronically resistant idiopathic thrombocytopenic purpura (ITP) associated with a poor prognosis. CASE SUMMARY: A 52-year-old female presented with a complaint of decreased visual acuity, which was 0.63 in both eyes. The patient had received a splenectomy, was receiving systemic treatment for chronic ITP, and had a history of diabetes mellitus and hypertension. Multiple retinal and subretinal hemorrhages and Roth spots were found on fundus examination. Fluorescein angiography revealed microaneurysms and hemorrhages in all four quadrants of the retina. The patient's platelet count was 38,000/µL. The patient was diagnosed with non-proliferative diabetic retinopathy and ITP-associated retinopathy, and underwent panretinal photocoagulation. Sudden visual loss was noted 4 months later, at which time the patient's visual acuity was 0.1 in both eyes, and her platelet count was 7,000/µL. For 2 years, the patient's platelet count was not controlled, and remained between 12,000–19,000/µL despite active medical treatment. Macular edema did not improve with intravitreal triamcinolone, dexamethasone, and bevacizumab. Retinal hemorrhages were not absorbed and visual acuity decreased to light perception in the right eye and counting fingers in the left eye. CONCLUSIONS: ITP-associated retinopathy was associated with a poor prognosis when the systemic disease was not controlled.

Mechanism of amifostine combined with low dose cyclosporine in refractory immune thrombocytopenia / 中国生化药物杂志

Objective To explore the effect of amifostine combined with low-dose cyclosporine in treatment of refractory immune thrombocytopenia effect and related mechanisms.Methods 60 cases of refractory immune thrombocytopenia patients using parallel randomized controlled groups, divided into three groups, 20 cases in each group, amifostine group were treated with amifostine, cyclosporine group were treated with cyclosporine, amifostine+CSA group received amifostine+cyclosporine A treatment.The platelet count, platelet membrane glycoprotein antibody, lymphocyte subsets and bone marrow megakaryocyte count were observed and compared.Results After different treatment of three, six months, the level of platelet count of patients in three groups were compared with the group before treatment were significantly increased, and the treatment of platelet count level of amifostine group and cyclosporine group were significantly lower than that of amifostine +CSA group, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.The total efficacy of amifostine+CSA group was significantly higher than the other two groups, the difference was statistically significant ( P<0.05 ) , there was no significant difference between amifostine group and cyclosporine group.After the treatment, the platelet membrane glycoprotein GPIIb/IIIa antibody levels in three groups were significantly increased, and ring the detection level of amifostine+CSA group after treatment was significantly higher than the other two groups, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.After treatment, the three groups of CD4 +, CD4 +/CD25 +and CD4 +/CD8 +levels were significantly increased, CD8 +decreased significantly, the difference was statistically significant (P<0.05).And the level of change after treatment with amifostine +cyclosporine group was significantly higher than that of the other two groups, the difference was statistically significant (P<0.05), there was no significant difference between amifostine group and cyclosporine group.After treatment, the number of bone marrow megakaryocytes in the three groups was significantly lower than that before treatment , the level of count after treatment with amifostine +cyclosporine was significantly lower than that of the other two groups, the difference was statistically significant (P<0.05).there was no significant difference between amifostine group and cyclosporine group.The adverse reactions of amifostine group and amifostine+CSA group were significantly lower than that in cyclosporine group, the difference was statistically significant (P<0.05).there was no significant difference between amifostine group and amifostine+CSA group.Conclusion Amifostine combined with low dose of cyclosporine in treatment of refractory immune thrombocytopenia can play a synergistic effect, improve the therapeutic effect, and effectively reduce the dosage and adverse reactions.

Púrpura trombocitopênica imunológica crônica e infecção pelo helicobacter pylori: respostaplaquetária ao tratamento de erradicação da bactéria

A Púrpura Trombocitopênica Imunológica Crônica (PTIc) é uma afecção causada por resposta auto-imune decorrente da sensibilização das plaquetas por auto anticorpos antiplaquetários, causando lise das plaquetas. A infecção pelo H. pylori tem sido mostrada como provável fator para o desenvolvimento de PTIc, com possível relação de resposta plaquetária na PTIc após o tratamento de erradicação da bactéria. O objetivo foi avaliar possíveis efeitos da erradicação do Helicobacter pylori no número de plaquetas dos pacientes com PTIc atendidos no Hospital Universitário Walter Cantídio. Foram convidados a participar do estudo 29 pacientes com PTIc, 15 permaneceram e todos foram tratados para o H. pylori, sendo acompanhados por um período de 6 meses a um ano para a avaliação clínica e contagem de plaquetas. Todos realizaram endoscopia digestiva para diagnóstico da infecção e, após o tratamento, realizaram teste respiratório para confirmação da erradicação. Foi realizado PCR para averiguar a presença do gene cagA do H. pylori. Dos 15 pacientes tratados 01 (6,6%) era do gênero masculino e 14 (93,3%) foram do gênero feminino, a média de idade foi de 47,7 anos (27-68), O tempo médio de doença foi de 7,43 anos (1-25)...

The Chronic Immune Thrombocytopenic Purpura ITPc) is a condition caused by autoimmune response resulting awareness of platelets by self antiplatelet antibodies, causing lysis of platelets. Infection with H. pylori has been shown to likely factor for the development of PTIc, with possible platelet response relationship in PTIc after treatment of bacterial eradication. The objective was to evaluate possible effects of eradication of Helicobacter pylori in the number of platelets of patients with PTIC patients at the University Hospital Walter Cantídio. Were invited to participate in the study 29 patients with PTIc, 15 remained and all were treatedfor H. pylori and were followed for a period of 6 months to a year for clinical evaluation and platelet count. All patients underwent endoscopy for diagnosis of infection and, after treatment, held breath test to confirm eradication. PCR was performed forthe presence of H. pyloricagA gene. Of the 15 patients 01 (6.6%) were male and 14 (93.3%) were female, the mean age was 47.7 years (27-68), the mean disease duration was 7.43 years (1-25)...

Neutrophil to Lymphocyte Ratio (NLR) as a Predictive Marker for Prognosis in the Patients with Immune Thrombocytopenic Purpura (ITP) / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting low platelet count in peripheral blood without any other abnormalities in other blood cell lineage. There were many studies that investigated the mechanism of ITP and the factors related to its chronicity, but much remains controversial. Neutrophil to lymphocyte ratio (NLR) is considered an indicator representing the severity of acute or chronic inflammation. The aim of this study was to assess whether NLR can predict the outcome of ITP and its chronicity. METHODS: Patients who were diagnosed as ITP at Dong-A University Hospital between March 2000 and March 2016 were analyzed retrospectively. NLR results and other medical records at diagnosis and after treatment were analyzed and compared between two groups; patients with single episode and chronic ITP group. RESULTS: Of a total of 67 patients, 39 patients (58.21%) were chronic ITP. Patients with single episode showed low NLR at diagnosis than patients with chronic ITP (1.23±1.38 vs. 1.54±1.15, P=0.42), but NLR after recovery were significantly high (1.58±1.71 vs. 1.31±0.76, P=0.21). However, patients with chronic ITP showed a trend of high NLR at the time of relapse. CONCLUSION: Chronic ITP patients showed higher NLR at the time of diagnosis compared with the patients with single episode. Also, the NLR at the time of relapse showed higher level compared to those at diagnosis. Relationship between NLR and chronic ITP need to be further studied.

Neutrophil to Lymphocyte Ratio (NLR) as a Predictive Marker for Prognosis in the Patients with Immune Thrombocytopenic Purpura (ITP) / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting low platelet count in peripheral blood without any other abnormalities in other blood cell lineage. There were many studies that investigated the mechanism of ITP and the factors related to its chronicity, but much remains controversial. Neutrophil to lymphocyte ratio (NLR) is considered an indicator representing the severity of acute or chronic inflammation. The aim of this study was to assess whether NLR can predict the outcome of ITP and its chronicity.METHODS: Patients who were diagnosed as ITP at Dong-A University Hospital between March 2000 and March 2016 were analyzed retrospectively. NLR results and other medical records at diagnosis and after treatment were analyzed and compared between two groups; patients with single episode and chronic ITP group.RESULTS: Of a total of 67 patients, 39 patients (58.21%) were chronic ITP. Patients with single episode showed low NLR at diagnosis than patients with chronic ITP (1.23±1.38 vs. 1.54±1.15, P=0.42), but NLR after recovery were significantly high (1.58±1.71 vs. 1.31±0.76, P=0.21). However, patients with chronic ITP showed a trend of high NLR at the time of relapse.CONCLUSION: Chronic ITP patients showed higher NLR at the time of diagnosis compared with the patients with single episode. Also, the NLR at the time of relapse showed higher level compared to those at diagnosis. Relationship between NLR and chronic ITP need to be further studied.

Factors Predicting Response to Splenectomy in Patient with Immune Thrombocytopenia Purpura

OBJECTIVE: Splenectomy has been proposed to be the standard therapy for patients with steroid refractory immune thrombocytopenic purpura (ITP). This study aimed to describe valuable factors predicting the effect of splenectomy in patients with ITP. METHODS: A total of 51 adult patients who underwent splenectomy for steroid refractory ITP were evaluated their medical records retrospectively. The response to the treatment was classified on the basis of the platelet count. RESULTS: The responding group included 35 patients (68.8%), the partial-responding group included 4 patients (7.8%), and non-responding group was 12 patients (23.5%). On univariate analysis, the response of splenectomy correlated with only the intravenous immune globulin (IVIG) response (66.7%, P=0.006), but hemolysis, autoantibody, a presence of accessory spleen, the response of steroid were not significantly associated with the effect of splenectomy. On multivariate analysis, the response of IVIG and the amount of platelet transfusion were independent variables of the response of splenectomy. CONCLUSION: Patients with ITP who have good responses to IVIG are likely to have a good or favorable responses to splenectomy.

Establishment and evaluation of heat sheng model of rat primary immune thrombocytopenic purpura / 中国比较医学杂志

Objective To establish a rat model at the same time in accordance with the “hot sheng syndrome” of traditional Chinese medicine and primary immune thrombocytopenic purpura of peripheral blood platelet reduction. Methods Using back multi-point injection of 20% dry yeast suspension on SD rats and 1∶4 dilution of rabbit anti SD rats platelet serum (APS) by intraperitoneal injection to establish a primary immune thrombocytopenic purpura “heat sheng”rat model.And observing rats of TCM syndrome characteristics, hemogram, myelogram and serotonin (5-HT) level of the temperature regulating center in thalamus.Results After injection of 2 h ~6 h temperature and daily water of the model group rats increased significantly,toe purper showed in fourth day of modeling and intestinal mucosal bleeding in thirty day of modeling(P <0.05);Platelet count in peripheral blood decreased significantly, bone marrow megakaryocyte number reduced significantly((P <0.05);5-HT level of the temperature regulating center of brain increased significantly((P <0.05).Conclusions The study of the primary immune thrombocytopenic purpura heat sheng rat model of combination of disease and syndrome reflected basically the pathological characteristics of purpura caused by “heat sheng” in primary immune thrombocytopenic purpura rat mode.

Expression of DNA methyltransferase mRNA in children with immune thrombocytopenia / 临床儿科杂志

Objective To study the relationship between DNA methylation and pathogenesis of childhood immune thrombocytopenic purpura (ITP) by examining the expression of DNA methyltransferase 1(Dnmt1) and DNA methyltransferase 3a (Dnmt3a) mRNA in peripheral blood lymphocytes of the children with ITP. Methods Expression of Dnmt 1 and Dnmt3a mRNA in the peripheral blood lymphocytes in 36 children with newly diagnosed ITP and 26 healthy children were detected using RT-PCR. Results Dnmt1 mRNA expression in peripheral blood lymphocytes in children diagnosed with ITP was 3.02±0.49, significantly lower than 4.58±0.52 in the control group (t=11.95, P<0.001). Dnmt3a mRNA expression in peripheral blood lymphocytes in children diagnosed with ITP was 1.49±0.44, signiifcantly lower than 2.41±0.32 in the control group (t=9.12, P<0.001). Conclusions Children with newly diagnosed ITP have lower DNA methylation status in peripheral blood lymphocytes as compared to that in healthy children. The DNA methylation may play an important role in the etiology of acute ITP in children.

Association of CD4+CD25+FoxP3+ regulatory T cells with natural course of childhood chronic immune thrombocytopenic purpura / 소아과

PURPOSE: The purpose of this study was to determine the frequency of CD4+CD25+FoxP3+ regulatory T cells (Treg) in the peripheral blood of patients with childhood chronic immune thrombocytopenic purpura (ITP) exhibiting thrombocytopenia and spontaneous remission. The findings of this study indicate the possibility of predicting spontaneous recovery and pathogenesis of childhood chronic ITP. METHODS: Eleven children with chronic ITP (seven thrombocytopenic and four spontaneous remission cases; mean age, 8.8 years; range, 1.7-14.9 years) were enrolled in this study. Five healthy children and eight healthy adults were included as controls. The frequency of Treg was evaluated by flow cytometry in the peripheral blood. RESULTS: In this study, four patients (36%) achieved spontaneous remission within 2.8 years (mean year; range, 1.0-4.4 years). The frequency of Treg was significantly lower in patients with persisting thrombocytopenia (0.13%+/-0.09%, P<0.05), than that in the patients with spontaneous remission (0.30%+/-0.02%), healthy adults controls (0.55%+/-0.44%), and healthy children controls (0.46%+/-0.26%). A significantly positive correlation was found between the frequency of Treg and the platelet count in children. CONCLUSION: These data suggest that a lower frequency of Treg contributes to the breakdown of self-tolerance, and may form the basis for future development of specific immunomodulatory therapies. Furthermore, Treg frequency has prognostic implication toward the natural course and long-term outcomes of childhood chronic ITP.

Efficacy and safety of eltrombopag in adult refractory immune thrombocytopenia

BACKGROUND: Eltrombopag is a thrombopoietin receptor agonist with excellent treatment outcomes in immune thrombocytopenia (ITP). Here, we analyzed the dose of eltrombopag required to achieve and maintain safe platelet counts in Korean ITP patients. METHODS: Adult refractory ITP patients ( or =50,000 cells/microL). After achieving the target platelet count, the dose of concomitant ITP medications and eltrombopag was reduced to identify the lowest effective dose required to maintain the platelet count. RESULTS: Among 18 patients, 66.7% achieved complete response, 5.6% achieved platelet counts between 50,000 and 100,000 cells/microL, and 27.8% failed to achieve the target platelet count. The median ITP duration was significantly shorter in patients who achieved the target platelet count. The initial dose required to achieve the target platelet count was 25 mg/d. The adjusted maintenance doses were 25 mg twice per week or 25 mg/d. After discontinuation, 83.3% relapsed, and the median relapse-free survival was 15 days. Two relapsed and 1 failed patient switched to romiplostim. The response to romiplostim was similar to eltrombopag. During eltrombopag treatment, 38.9% showed hepatobiliary laboratory anomalies. Among 9 follow-up bone marrow examinations, 1 revealed fibrosis after 1 year of treatment. CONCLUSION: Eltrombopag was well tolerated with excellent treatment outcomes in refractory adult ITP patients. Low-dose eltrombopag effectively maintained the target platelet count. However, some patients required longer or higher-dose treatment to maintain the target platelet count, especially in heavily pretreated or longer ITP cases.