Effects of peripartum treatment on delivery outcomes in women with primary immune thrombocytopenia: a prospective cohort study / 中华围产医学杂志

Objective:To investigate the effects of peripartum administration of low-dose corticosteroids or intravenous immunoglobulin (IVIG) on delivery outcomes in pregnant patients with primary immune thrombocytopenia (ITP).Methods:This prospective cohort study involved pregnant women (≥34 gestational weeks) who were diagnosed with ITP in Peking University People's Hospital from January 2017 to December 2021. Their platelet counts were between 20×10 9/L to 50×10 9/L without bleeding and none of them had been treated with any medications. All patients were divided into medication group (prednisone or IVIG) and platelet transfusion group based on their preference. Differences in vaginal delivery rate, postpartum hemorrhage rate and platelet transfusion volume between the two groups were compared using t-test, Wilcoxon rank sum test and Chi-square test. Binary logistic regression was used to investigate the factors influencing the rates of vaginal delivery and postpartum hemorrhage. Multiple linear regression was used to analyze the factors influencing the platelet transfusion volume. Results:A total of 96 patients with ITP were recruited with 70 in the medication group and 26 in the platelet transfusion group. The vaginal delivery rate in the medication group was higher than that in the platelet transfusion group [60.0% (42/70) vs 30.8% (8/26), χ 2=6.49, P=0.013]. After adjusted by the proportion of multiparae and the gestational age at delivery, binary logistic regression showed that the increased vaginal delivery rate in patients undergoing the peripartum treatment ( OR=4.937, 95% CI: 1.511-16.136, P=0.008). The incidence of postpartum hemorrhage in the two groups was 22.9% (16/70) and 26.9% (7/26), respectively, but no significant difference was shown ( χ 2=0.17, P=0.789). The median platelet transfusion volume was lower in the medication group than in the platelet transfusion group [1 U(0-4 U) vs 1 U(1-3 U), Z=－2.18, P=0.029]. After adjustment of related factors including the platelet count at enrollment, obstetrical complications and anemia, multiple linear regression showed that the platelet transfusion volume was also lower in the medication group (95% CI:0.053-0.911, P=0.028). Ninety-six newborns were delivered without intracranial hemorrhage. The overall incidence of neonatal thrombocytopenia was 26.0% (25/96). There was no significant difference in birth weight, and incidence of neonatal asphyxia or thrombocytopenia between the two groups. Conclusion:Peripartum therapy in ITP patients may increase vaginal delivery rate and reduce platelet transfusion volume without causing more postpartum hemorrhage.

Concurrencia entre el síndrome de Guillain-Barré y púrpura trombocitopénica inmune posiblemente inducidos por COVID-19 prolongado / Concurrence between Guillain-Barré syndrome and immune thrombocytopenic purpura possibly induced by long COVID-19

RESUMEN Durante la infección aguda por el SARVS-CoV-2 se produce una desregulación del sistema inmune que puede durar hasta ocho meses después de controlado el cuadro agudo. Esto, sumado a otros factores, posiblemente este asociado con un aumento del riesgo de aparición y concurrencia de enfermedades autoinmunes. La aparición simultanea del síndrome de Guillain-Barré (SGB) y púrpura trombocitopénica (PTI) se ha reportado poco en la literatura, y el SGB raramente se asocia con otra enfermedad autoinmune. Presentamos el caso de un varón que luego de un mes de tener un cuadro agudo de COVID-19 moderado, presentó concurrentemente SGB y PTI con respuesta adecuada al tratamiento.

ABSTRACT During acute SARS-CoV-2 infection, there is persistent deregulation of the immune system that can last up to 8 months after the acute condition is controlled. This, added to other factors, is possibly associated with an increased risk of the appearance and concurrence of autoimmune diseases. The simultaneous occurrence of GBS and ITP has been rarely reported in the literature, and GBS is rarely associated with another autoimmune disease. We present the case of a man who, one month after his recovery from acute moderate COVID-19, presented concurrent GBS and ITP with an adequate response to treatment.

Relação entre a infecção por Helicobacter pylori e doenças hematológicas: uma revisão integrativa / The relationship between Helicobacter pylori infection and hematologic diseases: an integrative review

Helicobacter pylori, uma bactéria gram-negativa, desde 1984 vem sendo associada às doenças gástricas. A partir da década de 1990, surgiram crescentes relatos indicando a relação da infecção com manifestações extragástricas. Nesse sentido, o objetivo do estudo foi investigar, através de uma revisão integrativa, as evidências relacionadas à H. pylori e a presença de doenças hematológicas, focando na anemia por deficiência de ferro (ADF) e na púrpura trombocitopênica idiopática (PTI). Bases de dados foram consultadas com as palavras-chave e descritores Helicobacter pylori, Doenças extragástricas, Doenças hematológicas, Anemia ferropriva e Púrpura Trombocitopênica Idiopática, nos idiomas inglês, português e espanhol, combinados com o operador booleano "AND". Após a leitura de 1.964 títulos, 85 artigos atendiam aos critérios de inclusão. Com a exclusão dos artigos duplicados e pela análise dos resumos, 62 trabalhos foram selecionados e lidos na íntegra. Por fim, 27 estudos foram incluídos: 13 relacionados à ADF e 77% deles encontraram associação com H. pylori, e 14 relacionados à PTI, nos quais a relação com a bactéria foi encontrada em 93%. As evidências que associam essas doenças hematológicas com H. pylori são expressivas, portanto, mais estudos são necessários para elucidar os mecanismos relacionados e contribuir para prevenção, diagnóstico e tratamento mais eficazes.

Helicobacter pylori is a gram-negative bacterium that has been associated with gastric diseases since 1984. Since the 1990s, there have been increasing reports indicating that the infection may also be associated with extragastric manifestations. This integrative review aimed to investigate the evidence on the relationship between H. pylori and hematological diseases, specifically iron deficiency anemia (IDA) and idiopathic thrombocytopenic purpura (ITP). Databases were searched for the keywords "Helicobacter pylori," "extragastric diseases," "hematologic diseases," "iron deficiency anemia," and "idiopathic thrombocytopenic purpura" in English, Portuguese, and Spanish, combined with the boolean operator "AND." The search yielded 1,964 studies. After reading the titles, only 85 met the inclusion criteria. Sixty-two studies were selected for full-text reading after exclusion of duplicates and abstract analysis. Finally, 27 studies were included in this review. Thirteen studies addressed IDA, among which 77% found an association with H. pylori; whereas 14 studies addressed ITP, among which 93% found a relationship with H. pylori. There is strong evidence supporting the association between hematologic diseases and H. pylori. Further studies are needed to elucidate the mechanisms involved in this relationship, contributing to more effective prevention, diagnosis, and treatment.

Maternal and infant outcomes of women with non-severe primary immune thrombocytopenia during two consecutive pregnancies: a self-controlled study / 中华围产医学杂志

Objective:To understand the progress, maternal morbidity, and maternal and infant outcomes in pregnant women with non-severe primary immune thrombocytopenia (ITP) during two consecutive pregnancies.Methods:This study retrospectively analyzed the clinical data of 40 patients with non-severe ITP who had two pregnancies and were treated at Peking University People's Hospital between June 2010 and June 2020. Platelet counts at different stages of pregnancy, treatments, maternal complications and neonatal outcomes were compared with Chi-square test, Fisher's exact test, paired sample t-test, non-parametric Wilcoxon signed rank test, independent sample t-test or non-parametric Mann-Whitney U test. Results:Among the 40 patients, 18 were diagnosed before and 22 were first diagnosed during the first gestation. Platelet counts and treatments in the 18 patients prior to their first conception were not significantly different from those in the 40 patients before their second pregnancy (all P>0.05). No significant difference in the average platelet count and thrombocytopenia severity at each stage of pregnancy, and maternal bleeding score or drug treatment was observed between the two pregnancies (all P>0.05), neither in the incidence of gestational hypertension, gestational diabetes, premature rupture of membranes, premature delivery, or anemia (all P>0.05). The incidences of postpartum hemorrhage and severe postpartum hemorrhage in the second pregnancy were 30.0%(12/40) and 22.5%(9/40), respectively, which were both higher than those in the first gestation [(7.5%(3/40) and 5.0%(2/40); χ2=6.64, 5.17; P=0.010, 0.023]. The amount of postpartum hemorrhage was higher in the second pregnancy than in the first [500 ml(213-795 ml) vs 300 ml(163-400 ml), Z=－2.34, P=0.019]. There was no significant difference in birth weight, the incidence of passive ITP or intracranial hemorrhage, or mortality between the neonates of the first and second pregnancy group (all P>0.05). The lowest platelet count in neonates within one week after birth in the second pregnancy group was (202.2±106.7)×10 9/L, which was lower than that of the first [(222.5±91.8)×10 9/L, Z=－2.04, P=0.041]. Conclusions:Non-severe ITP is not worse in the second pregnancy than in the first. In women with non-severe ITP, the incidence of maternal complications is not increased in the second pregnancy, but the risk of postpartum hemorrhage and the incidence of neonatal passive immune thrombocytopenia are raised.

El papel de los estimulantes de trombopoyetina (eltrombopag y romiplostim) en trombocitopenia inmune secundaria a Lupus

Introducción: la púrpura trombocitopénica inmune (PTI) es una manifestación hematológica frecuente en el lupus eritematoso sistémico (LES). Los corticoesteroides son la primera línea de manejo para la trombocitopenia moderada o grave, en conjunto con antimaláricos y otros inmunosupresores. En casos particulares, en donde la respuesta a las intervenciones iniciales no sea la adecuada, se cuenta con terapias de segunda línea. Objetivo: esta revisión narrativa se enfocará en dos medicamentos agonistas del receptor de trombopoyetina (RTPO): eltrombopag y romiplostim y en su papel en la PTI secundaria a LES. Además, se revisará su perfil farmacológico, dosis e indicaciones en el contexto de esta enfermedad. Métodos: se realizó una búsqueda de literatura en diferentes bases de datos, se analizaron artículos científicos y guías de manejo, tanto de LES como de trombocitopenia inmune, con el fin de contestar diferentes preguntas clínicas surgidas en el escenario de la práctica cotidiana. Resultados y conclusiones: el uso de los estimulantes de TPO es una alternativa terapéutica para escenarios particulares de pacientes con LES y trombocitopenia inmune. Sin embargo, son necesarios estudios enfocados en esta población específica para poder hacer recomendaciones acertadas acerca de su manejo. Los datos actuales son extrapolados de la trombocitopenia inmune primaria.

SUMMARY Introduction: Immune thrombocytopenia is a frequent hematologic manifestation in systemic lupus erythematosus (SLE). Corticosteroids are the first line of treatment for moderate to severe thrombocytopenia in this disease, in conjunction with antimalarials or other immunosuppressants. In particular cases where the response to initial interventions is not achieved, second-line therapies with different mechanisms of action are available. Objective: This narrative review will focus on two thrombopoietin receptor agonist drugs (TPO-RA): eltrombopag and romiplostim, and their role in immune thrombocytopenia secondary to SLE. In addition, its pharmacological profile, dose and indications will be reviewed in the context of this disease. Methods: A literature search was conducted in different databases; scientific articles and guidelines were analyzed, both for SLE and immune thrombocytopenia. With the purpose of answering different clinical questions that constantly arise in the scene of daily practice. Results and conclusions: The use of TPO-RA stimulants is a therapeutic alternative for particular scenarios in patients with SLE and immune thrombocytopenia, however studies focused on this particular population are necessary to be able to make strong recommendations about its utility. Current data are extrapolated from primary immune thrombocytopenia.

Clinical value of megakaryocytes in the diagnosis and treatment of children with immune thrombocytopenic purpura / 中国基层医药

Objective@#To analyze the clinical value of megakaryocytes in the diagnosis and treatment of children with immune thrombocytopenic purpura(ITP).@*Methods@#From June 2014 to January 2018, the clinical data of 110 children with ITP diagnosed and treated in Zhucheng People's Hospital Affiliated to Weifang Medical College were analyzed and followed up for more than 1 year.The children were divided into two groups according to whether the duration of the disease was morethan 12 months(chronic group and non-chronic group). Gender, age, initial course of disease, platelet count, lymphocyte count, megakaryocyte count, white blood cell count, and initial treatment regimen were analyzed and compared between the two groups.Multivariate analysis was used to analyze the independent influencing factors of chronic ITP.The clinical value of the initial diagnosis and lymphocyte counts in evaluation of the effects of chronic ITP and initial treatment were analyzed.The clinical value of megakaryocyte in the assessment of initial treatment was analyzed.@*Results@#The initial course of disease[(5.8±2.26)d]and megakaryocyte count[(210.28±98.67)/piece] in the chronic groupwere higher than those in the non-chronic group[(3.57±2.05)d, (165.26±78.35)/piece], and the lymphocyte count[(2.87±0.90)×109/L] in the chronic groupwas lower than that in the non-chronic group[(3.66±1.12)×109/L], the differences were statistically significant(t=4.824, 2.299, 3.545, all P<0.05). Megakaryocyte count was not an independent factor of chronic ITP(P>0.05). The initial course of disease was a risk factor for chronic ITP(OR=3.826), while lymphocyte count was a protective factor(OR=0.471). The initial course of disease was evaluated as AUC=0.648 for chronic ITP, with an optimal cut-off value of 4.5 days, a sensitivity of 65.4%, and a specificity of 62.5%.Lymphocyte counts was evaluated as AUC(area under the ROC curve)=0.648 for chronic ITP, the optimal cut-off value was 3.01×109/L, the sensitivity was 59.4%, and the specificity was 78.2%.The initial course of disease and lymphocyte count had no significant value in evaluation of the treatment outcome (P<0.05). The initial treatment of patients with increased megakaryocyte counts was better than those with the normal level, and the difference was statistically significant(Z=6.051, P<0.05).@*Conclusion@#The initial course of disease and lymphocyte count can help to assess the duration of ITP in children.Patients with increased bone marrow megakaryocyte counts can achieve better results at initial treatment.

New synergistic efficacy of combination of romiplostim and steroid in refractory immune thrombocytopenia patients

No abstract available.

Effects of tripterygium wilfordii polyglycoside tablets on expression of CD4+/CD8+ and CD4+ CD25+ Treg in elderly patients with relapsing refractory immune thrombocytopenic purpura / 中国基层医药

Objective To explore the mechanism of tripterygium wilfordii polyglycoside tablets for elderly patients with relapsing refractory immune thrombocytopenic purpura (ITP),and to seek the theoretical basis for Chinese medicine treatment of this disease.Methods The clinical data of 79 patients with relapsing refractory ITP were retrospectively analyzed.According to whether the combined use of tripterygium wilfordii polyglycoside,they were divided into the control group (35 cases) and the observation group (44 cases).The control group was treated with platelet and tranexamic acid,sulfasalazine,sulforaphane sodium,hemagglutinin and other symptomatic hemostasis treatment.The observation group in symptomatic hemostasis support on the basis of treatment with tripterygium wilfordii polyglycoside tablets.The CD4+/CD8+ ratio and CD4+ CD25+ Treg expression were compared between the two groups.Results The CD4+/CD8+ ratio,CD4+ CD25+ Treg and platelet count in the control group before treatmentwere (0.96 ± 0.36),(1.21 ± 0.67) ％,(13.14 ± 6.92) × 109/L,respectively,which of the observation group were (0.92 ± 0.37),(1.19 ± 0.59) ％,(11.51 ± 6.21) × 109/L,respectively,there were no statistically significant differences between the two groups (all P ＞ 0.05).The CD4+/CD8+ ratios in peripheral blood of the observation group at 2 weeks,3 weeks and 4 weeks after treatmentwere (1.04 ±0.56),(1.55 ±0.34),(1.59 ±0.41),respectively,there were statistically significant differences between the two groups (t =9.994,9.797,all P ＜ 0.05).The CD4+ CD25+ Treg proportions in the observation group at 2 weeks,3 weeks and 4 weeks after treatmentwere (1.01 ± 0.61) ％,(1.06:±:0.57) ％,(5.92 ± 0.65) ％,respectively,there was statistically significant difference between the 4 weeks after treatment and before treatment(t =5.378,P ＜ 0.05).The CD4+/CD8+ ratios in the peripheral blood of the control group were (1.01 ±0.60),(0.89 ±0.50) and (0.96 ±0.51),respectively,and the CD4+ CD25+ Treg in control group at 2 weeks,3 weeks and 4 weeks after treatment proportions were (0.99 ±0.72)％,(1.15 ±0.66)％,(1.22 ±0.56)％,respectively,there were no statistically significant differences between before and after treatment (all P ＞0.05).There were statistically significant differences in the CD4+/CD8+ ratio and CD4+ CD25+ Treg expression between the observation group and control group at 4 weeks after treatment (t =8.589,P ＜ 0.01;t =2.369,P ＜ 0.05).There was no statistically significant difference in the platelet count between the two groups(P ＞ 0.05),but the symptoms of bleeding of the observation group was lighter at 3 weeks after treatment.Conclusion Tripterygium wilfordii polyglycoside improves the expression of CD4+/CD8+ and CD4+ CD25+ Treg in peripheral blood of elderly patients with relapsed or refractory ITP.It is an ideal drug for the treatment of relapsed and refractory ITP in the elderly,it is worth further study.

The Effects of Helicobacter pylori Eradication Therapy for Chronic Idiopathic Thrombocytopenic Purpura

BACKGROUND/AIMS: The aim of this study was to evaluate the ability of Helicobacter pylori eradication treatment to increase platelet counts in Korean patients with chronic idiopathic thrombocytopenic purpura (ITP). METHODS: A total of 102 patients were evaluated against two criteria. First, those diagnosed with H. pylori infections in whom eradication was successful were assigned to the H. pylori-positive and -eradicated group (n=39), whereas those diagnosed with H. pylori infections in whom eradication failed were assigned to the H. pylori-positive and -non-eradicated group (n=3), and those without H. pylori infections were assigned to the H. pylori-negative group (n=60). Second, patients with complete remission in whom the platelet recovery effect was maintained over the average follow-up period of 6 months after eradication therapy were defined as the responder group (n=58), whereas those with partial or no response were defined as the nonresponder group (n=44). RESULTS: The platelet counts of the H. pylori-positive and -eradicated group were significantly increased 6 months after eradication therapy compared to those of the H. pylori-positive and -non-eradicated group and the H. pylori-negative group (43.2±29.1 to 155.3±68.7×10(3)/μL vs 42.5±28.1 to 79.8±59.7×10(3)/μL vs 43.1±28.9 to 81.2±62.2×10(3)/μL; p=0.041). The eradication therapy success rate in the responder group was 100.0% (39/39), in contrast to the nonresponder group (0%, 0/3) (p<0.001). CONCLUSIONS: H. pylori eradication therapy was related to increased platelet count, and successful eradication affected the increased platelet count in Korean patients with chronic ITP.

Clinical efficacy of laparoscopic splenectomy for immune thrombocytopenic purpura / 中华消化外科杂志

Objective To investigate the clinical efficacy of laparoscopic splenectomy (LS) for immune thrombocytopenic purpura (ITP).Methods The clinical data of 84 patients with ITP who underwent LS at the First Affiliated Hospital of Chongqing Medical University from January 2007 to January 2014 were retrospectively analyzed.The potential prognosing indexes (gender,age,course of diseas,preoperative response to steroid,preoperative PLT,operation time,volume of intraoperative blood loss and postoperative PLT peak within 7 days) were collected and analyzed.The evaluation of surgical efficacy was done according to the American Society of Hematology 2011 evidence-based practice guideline for ITP.The complete response and partial response were defined as effective and no response as void.All of the patients were followed up by outpatient examination and telephone interview till December 2014,the follow-up information included platelet count,bleeding performance,presence of long-term complications and postoperative recurrence,etc.Measurement data with normal distribution were presented as x ± s,and skew distribution data were described as M (range).The postoperative long-term effective rate was analyzed by Kaplan-Meier method.The univariate analysis and multivariate analysis were done using the chi-square test and Logistic regression model,respectively.Results The operation time of 84 patients was (154 ±40)minutes with a median volume of intraoperative blood loss as 200 mL (10-1 000 mL).Lienculus was detected in 11 patients and resected.Seven patients had complications and recovered after symptomatic and supportive treatment.There was no perioperative death.Among the 84 patients who were followed up for an average follow-up time of 51 months (12-96 months),45 patients had complete response,18 patients had partial response and 21 had no response,without serious spontaneous visceral and intracranial hemorrhage.Eighteen partial-response patients underwent symptomatic medical therapy and 21 no-response patients took orally prednisolone,among which 13 had PLT ＞ 30 × 109/L stably.During the follow-up,only one complete-response patient died of lung cancer with systemic metastasis at 2 years after surgery.Four patients had pneumonia,1 noresponse female patient had pyothorax repeatedly for a long time,and they all recovered after symptomatic and supportive treatments.There were no serious complications and overwhelming postsplenectomy infection in other patients.The 1-,3-,5-,7-year effective rates were 82.1％,77.6％,72.5％ and 67.9％,respectively.Univariate analysis showed that preoperative response to steroid,preoperative PLT and postoperative PLT peak within 7 days were related factors affecting long-term effecacy of patients with ITP (x2=5.600,6.006,21.733,P ＜ 0.05),but gender,age,course of disease,operation time and volume of intraoperative blood loss were not related factors affecting long-term effecacy of patients with ITP (x2=0.018,2.684,0.000,0.064,0.397,P ＞ 0.05).Multivariate analysis showed that preoperative response to steroid and postoperative PLT peak within 7 days ≥300 × 109/L were independent protective factors affecting long-term efficacy (OR =5.426,19.454,95％ confidence interval:1.220-24.129,4.704-80.449,P ＜ 0.05).Conclusions LS is safe and feasible for the treatment of ITP with a high long-term effective rate.Preoperative response to steroid and postoperative PLT peak within 7 days may be the predictors of LS for ITP.

Immune thrombocytopenic purpura in a patient with renal cell carcinoma

No abstract available.

Application of flow cytommetry detection of platelet associated antibody in patients with immune thrombocytopenic pupura / 国际检验医学杂志

Objective To investigate the value of the flow cytometry(FCM)for detecting platelet associated antibody in the di-agnosis of immune thrombocytopenia(ITP).Methods Platelet associated antibody,expression rate and the fluorescence intensity of platelet-associated immunoglobulin(PAIg)were measured in 51 patients with ITP(23 cases of newly diagnosed ITP and 28 cases of persistent ITP),21 patients with non-ITP and 60 healthy individuals.The correlation between the detection results with the platelet and megakaryocyte counts was performed;the expression rates of PAIg before and after treatment were compared.Results The fluorescence positive percentage and mean fluorescence intensity of IgG,Ig A and IgM in the patients group were significantly high-er than those in the control group with statistical difference(P 0.05);the difference between the autoimmune disease group and the healthy control group had statistical significance(P <0.01 );in the persistent ITP group,PAIg in the complete response group had statistical difference between the groups before treament.PAIg in the response group also had statistical difference between the groups before treament (P <0.05).Conclusion FCM for detecting PAIg can be used in the diagnosis,differential diagnosis and the disease condition monitoring of ITP.

Effect of Shenmai injection on Treg cells of idiopathic thrombocytopenic purpura / 中国基层医药

Objective To explore the effect of Shenmai injection on profile and function of CD4+CD2+5 Treg cells in ITP patients.Methods 78 ITP patients were selected and divided into the two groups, each group in 39 cases.The control group were given prednisone,while the observation group were given Shenmai injection additionally. CD4+CD2+5 FoxP3 +Treg cell counts were detected before and after treatment with flow cytometry,and the serum IL-2, IL-4 and IFN-γcontents were detected with ELISA kit.Results The effective rate of 56.41%and total effective rate of 92.31% in the observation group were higher than those of 30.77% and 71.79% in the control group (χ2 =4.222,3.933,all P<0.05).Furthermore,after the treatment,the CD2+5 FoxP3+Treg cell counts of 30.66%vs 24.10%and 7.49%vs 5.15%and serum IL-2,IL-4 and IFN-γcontents of (15.6 ±4.3)ng/mL vs (10.3 ±3.1)ng/mL, (9.5 ±2.7)ng/mL vs (13.9 ±3.4)ng/mL,(37.1 ±4.3)ng/mL vs (31.2 ±4.9)ng/mL in both the two groups increased(P<0.05);while all indexes′levels of the observation group were higher than those of the control group after treatment(χ2 =4.329,4.012,4.568,4.105,all P<0.05).Conclusion The defeat of CD4+CD2+5 Treg cells may be involved in the process of ITP,and Shenmai injection may be able to play a role in treatment through this link.

Correlation study of regulary B cell and effective Th cell in children with chronic idiopathic thrombocytopenic purpura / 国际检验医学杂志

Objective To study the changes of the peripheral blood regulatory B cells and related cytokines in children patients with chronic idiopathic thrombocytopenic purpura (CITP),and to analyze their correlations with effective Th cells,and to discuss their roles in the pathogenesis of CITP.Methods The peripheral blood mononuclear cells (PBMC)were separated in 30 cases of CITP,then percentages of Th1 ,Th17,Th22 and Breg cells were detected by the flow cytometry(FCM).IFN-γ,IL-17,IL-22 and IL-10 levels in culture supernatant were measured by the ELISA method.Results Compared with the control group,the proportions of the peripheral blood Th1 ,Th17,Th22 subsets in CITP children were increased[(18.4±4.7)% vs.(10.82±4.4)%;(2.42±1.02 )% vs.(1 .23±0.42)%;(0.79±0.24)% vs.(0.26±0.11)%],the proportion of Breg cells was reduced significantly,the differ-ences had statistical significance[(0.83±0.21)% vs.(1 .89±0.12)%,P <0.05].In CITP children,the IFN-γ,IL-17,IL-22 levels in culture supernatant were higher than those in the control group[(278.2±121.2)pg/mL vs.(181.8 ±82.2)pg/mL;(214.8 ± 100.5)pg/mL vs.(161 .4±67.8)pg/mL;(122.16±22.2)pg/mL vs.(90.8±31.1)pg/mL],but the IL-10 level was lower than that in the healthy control group[(27.4±12.6)pg/mL vs.(46.1±16.2)pg/mL),differences between them had statistical signifi-cance(P <0.05).Besides,there was a positive correlation between the proportion of Breg cells and cytokine IL-10 level(r=0.617, P <0.05 ),however,there was a negative correlation between the proportion of Breg cells with Th1 ,Th17 and Th22 cells (P <0.05),between IL-10 level with IFN-γ,IL-17 and IL-22 levels(P <0.05).Conclusion The downregulation of Breg cells proportion may participate in the immune disorder mechanism of effective Th cells in CITP children,which can provide a new idea for the im-munoregulation therapy in CITP children.

Anticorpos antifosfolípides em adultos com púrpura trombocitopênica imune / Assessment of antiphospholipid antibodies in adult patients with immune thrombocytopenic purpura

Introdução: a púrpura trombocitopênica imune (PTI) é doença autoimune adquirida, caracterizada por trombocitopenia. A PTI em adultos usualmente apresenta evolução crônica e o diagnóstico é sugerido pela exclusão de outras causas de trombocitopenia. Anticorpos antifosfolípides (AAF) com perda fetal ou trombose vascular definem a síndrome antifosfolípide (SAF). AAFs também podem ser identificados em portadores adultos de PTI. O objetivo deste estudo foi avaliar as associações entre AAF e PTI e entre PTI AAF positivo e TV. Método: trata-se de estudo de coorte incluindo pacientes com PTI atendidos em um ambulatório de um hospital público em Belo Horizonte, entre 1981 e 2006. Os dados relativos ao diagnóstico e ao acompanhamento foram extraídos de prontuários médicos, de laudos laboratoriais e por pesquisa telefônica. Resultados: foram diagnosticados 65 adultos com PTI, dos quais 28 (43,1%) foram avaliados para AAF. Cinco pacientes foram AAF positivo (18% dos pacientes avaliados, intervalo de confiança de 95% - 7,3% a 33,9%). Não houve diferença entre os grupos AAF positivo e AAF negativo em relação à idade e evolução clínica. Houve tendência ao predomínio de pacientes masculinos no grupo AAF positivo (valor p 0,08, teste qui- -quadrado). Nenhum evento trombótico foi observado em 956 meses acumulados de observação. Conclusão: observou-se AAF em 18% dos pacientes com PTI de adultos, mas não se constatou evento trombótico em pacientes com PTI.

Introduction: Immune Thrombocytopenic Purpura (ITP) is an acquired autoimmune disease characterized by low-platelet counts. ITP in adults usually runs a chronic course and the diagnosis is suggested by ruling out other diseases that may cause thrombocytopenia. Antiphospholipid antibodies (aPL) may be associated with vascular thrombosis or fetal loss as defined by the Antiphospholipid Syndrome (APS). aPL may also be found in adul ITP. The aim of this study was to evaluate the association between aPL and ITP between aPL and venous thrombosis. Material and methods: The study is a cohort comprising adult IPT patients who attended a large public hematological unit in Belo Horizonte, Minas Gerais, from 1981 to 2006. Data on diagnosis and follow-up were abstracted from medical record, laboratory databases and by telephone interviews. Results and discussion: A total of 65 adults were identified with adult ITP of whom 28 (43,1%) had aPL tested. Five ITP patients were aPL positive (18%, 95% CI 7.3 to 33.9). There was no difference between aPL positive and negative ITP patients regarding age and clinical evolution but there was a tren towards male overrepresentation in aPL positive ITP patients (p value 0.08, chi-squared test). No venous thromboembosis was observed in 956 cumulative months of observation. Conclusion: We observed aPL in 18% of adult ITP patients but no thrombosis in adult ITP patients.

The value of anti-von Willebrand factor antibody in the mechanism of idiopathic thrombotic thrombocytopenic purpura / 中华检验医学杂志

Objective This study is aimed at determining whether anti-von Willebrand factor (VWF) autoantibodies are present in the plasma of idiopathic thrombotic thrombocytopenic purpura (TTP) patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies,and at examining whether murine monoclonal antibodies (mAbs) against human VWF decrease the susceptibility of VWF to ADAMTS13 in vitro.Methods Anti-VWF autoantibodies and ultralarge VWF (UL-VWF) multimers were measured in plasma samples of 53 adult patients with idiopathic TTP by enzyme-linked immunosorbent assay and sodium dodecylsulphate-agarose gel electrophoresis,respectively.Moreover,the effects of eight murine mAbs to different human VWF domains on VWF cleavage by ADAMTS13 were evaluated under fluid shear stress and static/denaturing conditions,respectively.Results Anti-VWF antibodies and UL-VWF multimers were detected in two TTP patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13antibodies.The SZ34,an anti-VWF mAb,inhibited VWF proteolysis mediated by ADAMTS13 under flow,but not static conditions.Conclusion Anti-VWF antibody may be one of the causes of idiopathic TTP with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies.

Clinical study of recombinant human interleukin-11 combined with ciclosporin in treatment of glucocorticoid invalid idiopathic thrombocytopenic purpura / 中国医师进修杂志

ObjectiveTo observe the effect of recombinant human interleukin-11 combined with ciclosporin in treatment of giucocorticoid invalid idiopathic thrombocytopenic purpura.MethodsFrom August 2009 to August 2011,35 cases with glucocorticoid invalid idiopathic thrombocytopenic purpura patients were divided into two groups according to the treatment method:13 cases in control group were treated with ciclosporin and 22 cases in observation group were treated with recombinant human interleukin-11 at basis of above method.The clinical effect and platelet counts between two groups were compared.Results The total effective rate in observation group was 90.9％ (20/22),which was significantly higher than that in control group [53.8％ (7/13)] (P ＜0.05).After treatment,the platelet counts in both two groups were significantly increased [control group:( 112.5 ± 15.4) × 109/t vs.(13.2 ± 1.8 ) × 109/L; observation group:( 132.7 ± 22.3 ) × 109/L vs.(12.9 ± 1.6) × 109/L] (P ＜ 0.05 ).Moreover,the platelet counts after treatment in observation group was significantly more than that in control group (P ＜ 0.05).ConclusionRecombinant human interleukin-11combined with ciclosporin in treatment of glucocorticoid invalid idiopathic thrombocytopenic purpura is a good treatment scheme,which can be applied in clinic.

Efficacy of rituximab in treatment of children with refractory idiopathic thrombocytopenic purpura / 中华全科医师杂志

Fifty children with refractory idiopathic thrombocytopenic purpura (RITP) were treated with rituximab (n =26) or vincristine (n =24).The response rate,adverse reaction and recurrence rate in two groups were compared.The CD19 +/CD20 + B cells in peripheral blood were detected by flow cytometry in 26 patients before and after RITP treatment.The response rates of rituximab group was significantly higher than that of vincristine group ( 69.2％ vs. 37.5％,x2 =9.74,P ＜ 0.01 ). There was no significant difference in the rate of adverse reaction between two groups ( 11.5％ vs.8.3％,x2 =0.62,P ＞ 0.05 ).The recurrence rate of rituximab group ( 22.2％ ) was significantly lower than that of vincristine group (55.6％,x2 =7.24,P ＜ 0.05 ).In rituximab group the platelet count after treatment was significantly higher than that before treatment ( t =12.48,P ＜0.01 ),and the ratio of CD19 +/CD20 + B cells decreased significantly after treatment compare to that before treatment ( t =6.71,P ＜0.05 ).Rituximab is effective in treatment of refractory idiopathic thrombocytopenic purpura in children,which may be associated with decreased B cells in peripheral bloos.

A Case of Pulmonary Thromboembolism in a Patient with Idiopathic Thrombocytopenic Purpura / 대한내과학회지

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune blood disorder characterized by thrombocytopenia. Common clinical manifestations include bleeding events. Rarely, thrombotic complications may develop in ITP. A 43-year-old man was admitted with dyspnea. His platelet count at admission was 48,000/mm3. The patient had a history of ITP diagnosed 12 years earlier and had been treated with low-dose steroids. Two months before admission, he had been diagnosed with deep vein thrombosis and treated only with clopidogrel due to severe thrombocytopenia. Chest computed tomography showed filling defects in both pulmonary arteries. In the workup for precipitating factors, only lupus anticoagulant was positive. The concomitant administration of warfarin and methylprednisolone was used to treat the pulmonary thromboembolism and ITP, respectively. Six months later, the lupus anticoagulant test remained positive. The patient was confirmed to have a pulmonary thromboembolism due to antiphospholipid syndrome, which might be related to the underlying ITP. After 10 months, his symptoms and radiological findings had improved.

Clinical Implications of Elevated Antiphospholipid Antibodies in Adult Patients with Primary Immune Thrombocytopenia

BACKGROUND/AIMS: Antiphospholipid antibodies (aPL) have been detected in various proportions of patients with primary immune thrombocytopenia (ITP), but the clinical significance of this is debatable. The present study aimed to determine the frequency and clinical implications of elevated aPL in adult patients with ITP. METHODS: We prospectively studied newly diagnosed adult patients with ITP who were enrolled between January 2003 and December 2008 at Chungnam National University Hospital. They were evaluated for the presence of lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) at diagnosis and were followed for the development of thrombosis. RESULTS: Seventy consecutive patients with ITP (median age, 48 years; range, 18 to 79) were enrolled. Twenty patients (28.5%) were positive for aPL at the time of diagnosis: aCL alone in 15 (75%), aCL and LA in two (10%), and LA alone in three (15%). Patients who had platelet counts < 50,000/microL were administered oral prednisolone with or without intravenous immune globulin. No difference was found between the aPL-positive and -negative groups regarding gender, initial platelet count, and response to the therapy. After a median follow-up of 20 months (range, 2 to 68), two of 20 patients who were aPL-positive (10%) developed thrombosis, whereas no thrombotic event was found among those who were aPL-negative. CONCLUSIONS: Our data suggest that aPL levels should be determined at the initial presentation of ITP and that patients found to be aPL-positive should receive closer follow-up for thrombotic events.