Acute Immune Thrombocytopenic Purpura Research Articles

CHARACTERISTIC OF TIM-3 EXPRESSION IN T CELL SUBSETS IN PATIENTS WITH IMMUNE THROMBOCYTOPENIC PURPURA

T cell dysregulation is a common event involved in immune thrombocytopenic purpura (ITP). Recent findings indicated that aberrant expression of immune checkpoints may be related to autoimmune disease pathogenesis as well as in ITP. However, the distribution of Tim-3 and its co-expression with exhausted phenotype in T cell subsets remain unclear. In this study, the frequency of T-cell immunoglobulin mucin 3 (TIM-3) expression and co-expression with CD244 and CD57 in CD3+, CD4+ and CD8+ T cells were evaluated in peripheral blood (PB) from 16 cases with ITP and 21 healthy individuals (HI). Overall, the frequency of Tim-3+CD3+, CD4+ and CD8+T cells in ITP is similar to HI. However, Tim-3+CD244+CD8+ and Tim-3+CD57+CD8+T cells were increased in ITP in compared with HI group. While the percentage of CD244+ and CD57+CD3+, CD4+ or CD8+T cells was similar between ITP and HI groups. Moreover, the Tim-3+CD244+CD3+ and Tim-3+CD244+CD8+ T cells in newly diagnosed ITP group were higher than that in relapsed ITP group, similar trends showed in Tim-3+CD57+CD3+T cells. Obviously, relative higher Tim-3+T cells in most T cell subsets were detected in a case with acute ITP with viral infection. The distribution of Tim-3+ T cells in different subsets was also compared between PB and bone marrow (BM). High trends of Tim-3+T cells was detected in BM group. In conclusion, we firstly characterized T cell immune disorder with higher level of Tim-3+exhausted+CD8+T cells in ITP, the effect and mechanism of such alteration in ITP is needed further investigation.

PB2246 CLINICAL FEATURES AND TREATMENT OUTCOMES OF IMMUNE THROMBOCYTOPENIC PURPURA IN INFANTS; A SINGLE CENTER RETROSPECTIVE STUDY

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.Aims:Since children of different ages may have differing clinical courses, better delination 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 year of age admitted and treated for acute ITP at Pusan National University Children's Hospital from 2009 through 2017. All of these patients less than 1 year of age were identified and enrolled. We investigated their demographics, clinical features, laboratory examinations, respond to treatment and long‐term outcomes and compared to those of children 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 x 109/L (0∼76). Minor bleeding (bleeding score 0‐2) was significantly dominant in infant compared to older ages. Eighty two out of 85 patients (96.5%) 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 rising compared to older ages (p = 0.02), and the mean time to platelet counts above 100x109/L was 6.4+4.5 days. Follow up information at 6 months was available for 85 infants. Chronic ITP at 6 month was seen less frequently in infants (4.7%) than in children 1 to 10 years of age. (23.2%. P < 0.01).Summary/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.

Hemorrhage in the Setting of Acute Severe Refractory Immune Thrombocytopenic Purpura: A Case Report

Hemorrhage in the Setting of Acute Severe Refractory Immune Thrombocytopenic Purpura: A Case Report

Immune Thrombocytopenic Purpura of Children in Black Africa: Two New Observations in Togo

Introduction: Immune thrombocytopenic purpura (ITP) is an auto immunedisorder that is very common in Europe and the USA but ispoorlydescribed in black Africa. Observations: K.A., boy of 5 years 7 months was admitted in 2012 for post-varicella acute ITP with a hemorrhagic syndrome associated with severe isolated thrombocytopenia at 7,000 platelets / mm3. S.D. is a boy of 3 years 8 months admitted in 2015 for ITP which history is marked by a delay in diagnosis despite several consultations. In both cases, complete remission was obtained under prednisone. Discussion: Recently, the rarity of ITP has also been highlighted in the USA in the black race. The probable reasons given were genetic factors, a sub-diagnosis due to the discrimination of the blacks in the accessibility to healthcare, a relative discretion of the petechi as which can go unnoticed on black skin, but also the ignorance of the affection by the practitioners in Togo. Conclusion: ITP is poorly known and poorly reported in Black Africa. The establishment of an ITP registry may increase its knowledge and impact.

Role of TNF −308 G/A, TNFβ +252 A/G and IL10 −592 C/A and −1082 G/A SNPs in pathogenesis of Immune Thrombocytopenia Purpura in population of Gujarat, India

Immune Thrombocytopenic Purpura (ITP) is an autoimmune disease, also known as Idiopathic Thrombocytopenia Purpura is characterized by the presence of antibodies against self-platelets. The etiology of ITP remains unclear, but it is generally accepted that both environmental and genetic factors and probably also a synergistic relationship between these factors, play important roles in the development of the disease. The etiological data for Indian population is insufficient. Several reports have suggested an association of cytokine polymorphisms with ITP, especially in occurrence and progression of acute ITP to chronic ITP disease. We therefore investigated association of candidate gene viz. TNFA (−308G/A), TNFB (+252A/G) and IL10 (−592C/A; −1082G/A) polymorphisms with Immune Thrombocytopenia Purpura in Population from Gujarat state of India.To investigate this, total 154 subjects (103 control and 51 ITP patients) were selected irrespective of sex. DNA was extracted and PCR-RFLP analysis was performed to assess the association of the above mentioned SNPs in patients 51 with respect to 103 controls.Results revealed that SNP of TNFA (−308) GA genotype was significantly higher in ITP patients (p < 0.0001, OR 7.8887 and 95% CI = 3.857–16.13) as well as elevated frequencies of recessive genotype AA (p < 0.001, OR = 11.40, 95% CI = 5.176–25.11) were present. Similarly, IL10 (−1082G/A) showed significant association with ITP patients with increased GA genotype frequency (p = 0.398, OR = 2.057, 95% CI = 1.030–4.109). The homozygous recessive genotype AA was also found to be significantly high (p < 0.0013, OR = 3.088, 95% CI = 1.541–6.190). However, we did not observe any association between TNFB(+252A/G) and IL10 (−592C/A) SNPs with ITP patients.Overall, our study suggests that the GA/AA genotype of the TNFA −308G/A and IL-10 −1082 polymorphism may be a significant genetic risk factor for the pathogenesis of ITP. Moreover, TNFA and IL-10 single nucleotide polymorphisms (SNPs) are known to be associated with altered expression of these proteins which may lead to dysregulation in downstream pathways of inflammation, thereby further strengthening the possibility, of role of inflammatory pathway's components in the development of ITP.

Cytokines and immunoglobulin derangement in egyptian children with primary immune thrombocytopenic purpura

Background Immune thrombocytopenic purpura (ITP) is a heterogeneous immune-mediated disorder in which the immune system reacts with cytokine-mediated T lymphocytes and a platelet autoantigen(s). Aim The aim of this study was to detect helper T-1 and T-2 response through assessment of tumor necrosis factor (TNF)-α and interleukin (IL)-6, respectively, and immunoglobulin (Ig)M, IgG, and IgA in patients with acute and chronic ITP and to detect their possible roles as predictors of the course of the disease. Patients and methods This cross-sectional case–control study, conducted at Ain Shams University, recruited 20 patients with ITP (10 acute and 10 chronic) and 10 age-matched and sex-matched healthy controls. All were subjected to detailed clinical assessment, assessment of serum IL-6 and TNF-α using enzyme-linked immunosorbent assay, and serum IgA, IgM, and IgG using nephlometric method. Results Mean serum values of IgM and IgG were statistically lower in patients with acute ITP, and statistically higher serum values of TNF-α were detected in patients with acute and chronic ITP compared with controls. IL-6 levels were statistically higher in acute ITP when compared with chronic ITP and with controls and a higher significant value in chronic ITP than controls. There were significant positive correlations between IgM, IgG, and platelet count and a significant negative correlation between both TNF-α and IL-6 and platelet count. On follow-up of patients with acute ITP, one of them developed a chronic progressive course, and this patient was found to have low serum levels of IgA and IgG at presentation. Conclusion Children with primary ITP had dysfunction at cytokine and serum Ig level.

Association of IL-17A and IL-17F Gene Polymorphisms with Acute Immune Thrombocytopenia in Egyptian Children

Background: IL-17 is an inflammatory cytokine that plays a crucial role in many autoimmune diseases. Aim: To investigate the association of IL-17A rs2275913 and IL-17F rs763780 gene polymorphisms with acute immune thrombocytopenic purpura (ITP) in Egyptian children. Patients and methods: We examined 80 patients (male/female, 33/47; median age, 7 years old) diagnosed with acute ITP and 55 healthy controls (male/female, 28/27; median age, 7 years old). Genotyping was determined using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) technique. Results: In the acute ITP group compared to control, statistical analysis of the genotype frequencies (GG, AG, AA) of the IL-17A rs2275913 polymorphism and its alleles (A, G) showed no significant difference between the two groups (p > 0.05). Interestingly, the IL17A rs2275913 GG genotype was associated with early recovery (p = 0.04). As regard the genotype frequencies of the IL-17F rs763780 polymorphism, there was statistical significant difference in the TT and TC genotype frequencies between the case and control groups (p = 0.001 and p = 0.003, respectively). The number of IL-17F rs763780 T alleles was significantly higher in acute ITP patients as compared with children in the control group (p Conclusion: The present findings indicate that the IL-17 polymorphism IL-17F rs763780, but not IL-17A rs2275913 may be associated with a higher risk of acute ITP in Egyptian children.

Use of Single- or Two-dose Pulse Methylprednisolone in the Treatment of Acute Immune Thrombocytopenic Purpura.

Objectives:In immune thrombocytopenic purpura (ITP) treatment, the main goal is achieving the platelet level most rapidly for hemostasis. Pulse steroid therapy is common due to the rapid increase in the platelet count within the first 48 hours. Intravenous (IV) pulse steroid therapy is usually administered as a single methylprednisolone dose in the morning. Oral methylprednisolone is generally used as two divided doses due to its half-life, but there is no efficacy study for the use of pulse methylprednisolone therapy in two doses. In this study, we aimed to investigate whether the administration of single or double doses of pulse steroid treatment, which is the cheapest and most economical way to treat patients, differ in terms of platelet count increase rate.Methods:The diagnosis of acute ITP was made based on the appropriate clinical, laboratory, and bone marrow findings and platelet count <100.000/mm3. All the patients were diagnosed with bone marrow aspiration, and they were admitted to the hospital. All patients with platelet counts below 20000/mm3 and those who had wet purpura or active bleeding were treated. Patients in need of treatment were randomly divided into two treatment groups with closed envelope method. The first group was given IV pulse methylprednisolone (30 mg/kg/day for three days and 20 mg/kg/day for four days) in the early morning hours. The second group received the same daily dosages in two divided doses. Hemoglobin, white blood cell, and platelet counts were evaluated before and on the first, second, third, fifth, and seventh days of treatment. To evaluate the rate of treatment response, platelet counts over 20.000/mm3, 50.000/mm3, and 100.000/mm3 obtained on the first, second, third, and seventh days of treatment were compared.Results:Sixty patients with acute ITP diagnosis receiving pulse steroid therapy were included in the study. Platelet counts of the patients in group 2, who received pulse steroids in two doses, reached ≥20.000/mm³ on the second day [median, (2–3) days], ≥50.000/mm³ on the third day [median, (2.7–3.5) days], ≥100.000/mm³ on the fifth day [median, (3–5) days], which were significantly lower than the platelet counts of the patients in the first group on the third day [median, (2–5) days], fifth day [median, (4–7) days], and seventh day [median, (4–7) days], respectively (p<0.001, p<0.001, p=0.004).Conclusion:This study shows that administration of IV pulse steroid therapy in two doses is more effective in increasing the platelet count in early period in patients with acute ITP, especially whose platelet count is less than 20.000/mm³, and when we prefer to increase the platelet counts rapidly due to risk of intracranial hemorrhage.

Comparison of the Therapeutic Effect of Anti-D IG and IVIG in Children with Acute Immune Thrombocytopenic Purpura Attending a Children’s Medical Center: A Randomized, Double-Blind, Controlled Clinical Trial

Background: This study aimed at comparing the therapeutic effect of anti-D immunoglobulin (anti-D IG) and Intravenous Immunoglobulin (IVIG) in children with acute immune thrombocytopenic purpura (ITP). Methods: This Randomized double-blind, controlled clinical trial was conducted on 98 children attending a Children’s medical center during year 2008. The selected participants were allocated to IVIG and anti-D IG groups using the balanced-block randomization method. The platelet count, hemoglobin level, and side effects of the medications were evaluated on days 1, 3, 7, 14, and 21 after drug injection. Results: After drug injection, the platelet count increased in both groups (P 0.05). The hemoglobin level also decreased after injection in both groups (P 0.05). Regarding the ability of the drugs to increase the platelet count, 56% of the patients in the anti-D IG group and 52% of the children in the IVIG groups had platelet counts of more than 20 000 during the 24 hours after drug administration with no difference between the 2 groups (P = 0.836). The incidence of drug adverse effects, including fever and chills (4.1% in the anti-D group versus 10.4% in the IVIG group), severe hemolysis (4.5% in the anti D group versus 0% in the IVIG group) and headache (6.25% in the anti-D group versus 4.1% in the IVIG group), had no significant difference between the two groups (P > 0.05). Conclusions: Intravenous Immunoglobulin and anti-D have similar effectiveness in the treatment of children with acute ITP. Considering the fewer side effects of anti-D, it may be a suitable replacement for IVIG in ITP patients.

Evaluation of miR-21 and miR-150 expression in immune thrombocytopenic purpura pathogenesis: a case-control study

Immune thrombocytopenic purpura (ITP) is a common autoimmune disorder diagnosed with thrombocytopenia and bleeding symptoms due to production of autoantibodies (Abs) against platelets. Nowadays, microRNAs are known as novel biomarkers for diagnosis of diseases. The aim of this study was to investigate the expression levels of miR-21 and miR-150 in ITP patients and determine the role of these miRNAs in ITP pathogenesis. Thirty newly diagnosed patients with acute ITP and 30 healthy subjects( age and sex matched) as controls were enrolled in this study. The expression level of miR-21 and miR-150 was investigated using Real-time-PCR. Comparison of demographic characteristics of the cases was done using independent t-test and chi-square test. Comparison of the expression level of miR-21 and miR-150 with the related parameters was done using independent t-test or Mann–Whitney and Kruskal–Wallis test. Spearman rho correlation coefficient was used to investigate the relationship between the expression of miR-21 and miR-150 with demographic characteristics. The expression of miR-21, 150 in the patients was not different compared with the control group in general. A significant relationship between the expression of miR-21 with hemoglobin, hematocrit and red blood cell hemoglobin concentration was observed. Expression of miR-21 and miR-150 is not associated with pathogenesis of acute ITP and can involve the synergistic role of other miRNAs. Investigation of miR-21 and miR-150 expression along with other miRNAs and cytokines can be helpful in diagnosis and pathogenesis of ITP.

Acute Kidney Injury, Immune Thrombocytopenic Purpura, and the Infection That Binds Them Together: Disseminated Histoplasmosis.

Untreated human immunodeficiency virus (HIV) can be complicated by opportunistic infections, including disseminated histoplasmosis (DH). Although endemic to portions of the United States and usually benign, DH can rarely act as an opportunistic infection in immunocompromised patients presenting with uncommon complications such as acute kidney injury and idiopathic thrombocytopenic purpura. We report a rare presentation of DH presenting with acute kidney injury and immune thrombocytopenic purpura in an immunocompromised patient with HIV.

Study of CD4+, CD8+, and natural killer cells (CD16+, CD56+) in children with immune thrombocytopenic purpura

Objective/backgroundTo assess the percentage of CD4+, CD8+, and natural killer cells (CD16+, CD56+) in children with immune thrombocytopenic purpura (ITP) at presentation and study their impact on disease chronicity. MethodsThis case–control study was conducted at the Pediatric Hematology and Oncology Unit, Menoufia University Hospital (tertiary care center in Egypt). The study was held on 30 children presenting with ITP; they were followed-up and classified into two groups: 15 children with acute ITP; and 15 children with chronic ITP. Patients were compared to a group of 15 healthy children of matched age and sex. Measurements of CD4+, CD8+, and natural killer cells (CD16+, CD56+) by flow cytometry were assessed and compared in these groups. ResultsCD4+ and CD4+/CD8+ were significantly lower in acute and chronic patients than the control group (p<0.05 and p<0.001, respectively), with no significant difference between acute and chronic patients (p>0.05). However, CD8+ was significantly higher in acute and chronic patients than the control group (p<0.05), with no significant difference between acute and chronic patients (p>0.05). Natural killer cell percent was significantly lower in acute patients than the control group (p<0.001), with no significant difference between chronic and control groups (p>0.05). ConclusionITP is associated with immunity dysfunction denoted by the increase in cytotoxic T lymphocytes and the decrease in natural killer cells.

Investigation of celiac disease followed by immune thrombocytopenic purpura diagnosis in patients and comparison with literature.

OBJECTIVE:Celiac disease (CD) and Immune thrombocytopenic purpura (ITP) may occur together as a result of similar autoimmune mechanisms. The aim of this study was to assess the frequency of CD in a group of ITP patients and in the literature.METHODS:A total of 29 patients in Pamukkale University Faculty of Medicine Hospital Pediatric Hematology and Oncology Department with ITP were included in the study. Test was performed for the antibodies related to CD. Positive result for celiac antibodies was confirmed with biopsy. The results were compared with the literature.RESULTS:Of the study group, 13 patients (44.8%) were female and 16 (55.2%) were male. The mean age was 7.2±4.7 years and mean platelet count at the time of admission was 13,440±11,110/mm3 (range: 2000-41,000/mm3). Twelve patients (41.4%) were diagnosed as acute ITP, 6 patients (20.7%) as persistent ITP, and 11 patients (37.9%) as chronic ITP, according to the duration of thrombocytopenia. Antibody positivity was detected in 1 patient. Histological evaluation was compatible with CD. Results were compared with studies regarding the prevalence of CD in the population. No significant difference was found.CONCLUSION:Although it is not necessary to perform CD test in every case of ITP, the presence of differential diagnosis of CD is important to prevent unnecessary treatment, especially in ITP patients with growth retardation or malabsorption findings.

Challenge of Using Antiplatelet Drugs in Patients with Primary Immune Thrombocytopenia and Recently Implanted Coronary Stents-Splenectomy as a Therapeutic Option

Background: Immune thrombocytopenic purpura (ITP) in the patients with implanted coronary stents is related with serious risks of haemorrhage related to dual antiplatelet therapy on the one hand and stent thrombosis if antiplatelet therapy is interrupted on the other hand. Therefore, the main objective in these patients is the correction of thrombocytopenia and continuous use of antiplatelet drugs. Case report: We present the patient with implanted stents after acute myocardial infarction (AMI) and severe ITP who was successfully treated with splenectomy. After the patient experienced AMI, primary percutaneous coronary intervention (PCI) with implantation of stents has been performed. Thrombocytopenia (21 × 109/L) was registered for the first time after PCI intervention when it has been noticed a massive hematoma of whole right arm at the site of radial artery puncture. Immediately after the intervention dual antiplatelet therapy and prednisone has been started. Since corticosteroids and azathioprine treatment proved unsuccessful (platelet count <10 × 109/L), the patient has been prepared for splenectomy with intravenous immunoglobulins. As the platelet count was in stable range (40-50 × 109/L) after splenectomy, antiplatelet therapy has been readministered safely. Conclusion: As there are no definitive guidelines for treatment of patients with ITP and implanted stents, the treatment should be individualized to minimize risk of hemorrhagic as well as thrombotic complications. Our case suggests that splenectomy is an available and safe treatment for these patients. However, decision on splenectomy is a challenge; and before the surgical intervention the risk-benefit assessment should be considered.

Use of the Platelet Indices for Differential Diagnosis of Pediatric Immune Thrombocytopenic Purpura ( ITP )

IntroductionThe prognosis of pediatric immune thrombocytopenic purpura (ITP) has a favorable prognosis, but it is difficult to predict the course of this disease at the time of diagnosis. Recent advances of automated blood cell analyzer enable to measure platelet indices such as mean platelet volume (MPV), platelet size deviation width (PDW) and platelet-large cell ratio (P-LCR). These have brought about some information for thrombocytopenia. We studied the significance of such platelet indices in the differential diagnosis of immune thrombocytopenic purpura (ITP) and their ability to discriminate the clinical courses of ITP.MethodsNinety-three patients were enrolled this study at our institution from 2004 to 2006. Their ages ranged from 1 to 17 years (media 4.3 years). Of 93, patients were divided into two goups for analysis, according to the diagnosis of ITP. 34 were diagnosed as ITP and 59 were diagnosed various blood diseases other than ITP. In these 59 non-ITP patients, five were with thrombocytopenia (platelet count < 100.0 x 109/L), the other without thrombocytopenia. Of 34 ITP patients, 29 were acute subtype and 5 were chronic. The Sysmex-XE2100 automated blood cell analyzer (Sysmex, Kobe, Japan) was used to measure the blood parameters, including the platelet indices.ResultsAccording to MPV, PDW and P-LCR, there were no significant differences between ITP patients and non-ITP. In children with ITP, there were significant inverse correlations between the platelet count and evaluated parameters, MPV (R2=0.376), PDW (R2=0.26) and P-LCR(R2=0.338). But no significant correlations were found between the platelet count and these evaluated platelet indices in non-ITP. Also, we have found all platelet indices were significantly higher in chronic ITP than in acute ITP. In particular, PDW (9.910 vs 13.080) and P-LCR (21.071 vs 33.220) showed marked differences between the two groups.ConclusionsITP is considered as a result of hyper-destruction of platelets. Increase of platelet count is regarded as decrease of destruction of platelets. Our data suggested that platelet indices, MPV, PDW and P-LCR showed the useful information for the degree of platelet destruction and the ability to predict the clinical courses of ITP. DisclosuresNo relevant conflicts of interest to declare.

Differential Expression of T-bet and GATA3 in Egyptian Children with Idiopathic Thrombocytopenic Purpura.

GATA3 and T-box (T-bet) expressed in T-cells are transcriptional factors that play a critical role in development of Th2 and Th1 immunity respectively. GATA3 is expressed during Th2 differentiation and T-bet is expressed exclusively in Th1 cells. Thus, a balance between GATA3 and T-bet is believed to control Th2/Th1 polarization. Therefore, the high expression of T-bet and low expression of GATA3 indicate the existence of Th1 polarization in children with acute immune thrombocytopenic purpura (ITP). This might be related to the regulation of T-bet and GATA3. The objective of this work was to study the expression of transcriptional factors T-bet and GATA3 m RNA in children with idiopathic thrombocytopenic purpura and correlate it with clinical findings, laboratory findings, and outcome of patients. In this study the expression of T-bet and GATA3 genes was analysed in 20 normal healthy individuals and 40 children with ITP (20 acute and 20 persistent) using reverse transcriptase polymerase chain reaction to investigate a possible relation, association or correlation with the type of ITP and prognosis. T-bet was expressed significantly in 60% of acute ITP children (12/20) (P value 0.001) and not expressed in persistent ITP children (0/20), while GATA3 was expressed in 25% of persistent ITP patients (5/20) (P value 0.017) and not expressed in acute ITP patients (0/20). Both genes were not detected in healthy controls. We concluded that the high expression of T-bet and the low expression of GATA3 indicate the existence of Th1 polarization in children with acute ITP. This might be related to the regulation of T-bet and GATA3. Intensive studies of abnormal cytokine profiles in ITP have led to cytokine therapies that exploit the effects of IFN-γ on Th2 cells, but such therapies are often ineffective to develop safe and effective therapeutic tools. Targeting specific molecules such as T-bet and GATA3 may be a novel therapeutic tool in ITP.

A New Look into Childhood Acute ITP: The Relationship Between Thrombin Generation and Thrombocytopenia

Introduction: Immune thrombocytopenic purpura (ITP) is the most common cause of primary isolated thrombocytopenia of childhood. Although ITP results in profound thrombocytopenia, the severity of bleeding in this disease does not correlate well with the platelet count suggesting a compensatory procoagulant state. The primary aim of this study is to evaluate thrombin generation in childhood acute ITP patients compared to age-matched controls and examine procoagulant activity in relation to bleeding severity in childhood acute ITP.Methods: After IRB consent was approved, platelet poor plasma was isolated from acute ITP patients at the time of initial diagnosis and serially for up to 6 months post-diagnosis. A single sample was obtained from age-matched controls. Thrombin generation assays were run via the Calibrated Automated Thrombogram (CAT) using reagent containing 1pM tissue factor and 4uM phospholipid. Peak thrombin levels were measured and compared to control samples using paired t-test analysis. Clinical bleeding severity was assessed using a modified overall Buchanan bleeding score at each time point (0=no bleeding, 1=minor, 2=mild, 3=moderate, 4=severe, 5=life threatening bleeding).Results: Samples from 19 childhood acute ITP patients, mean age 7 years, and 8 controls, mean age 5 years, were assessed. The figure below demonstrates that despite profound thrombocytopenia, the peak thrombin values of acute ITP patients were higher than controls throughout the first 3-4 months following diagnosis. ITP assays were significantly different from controls at the following time-points: 1-2 weeks (p =0.001), 3-4 weeks (p =0.022), 5-8 weeks (p =0.0009), 9-12 weeks (p =0.004), and 13-16 weeks (p =0.006) post-diagnosis. Peak thrombin values returned to values similar to controls 17 weeks from diagnosis. With the onset of acute ITP, higher bleeding scores were related to more severe thrombocytopenia. In ITP patients, thrombin peak values remained elevated regardless of resolving thrombocytopenia and improved bleeding scores (see figure).Conclusion: To our knowledge, this is the first study to assess longitudinal thrombin generation using the CAT in childhood ITP patients. In a highly significant pattern, acute ITP patients demonstrated sustained elevated peak thrombin levels compared to controls, even at acute onset of disease. Certainly, thrombin release, and subsequent thrombin generation play a compensatory role to decrease bleeding among childhood ITP patients when the platelet count remains in the 10-50,000 range. But, based on bleeding scores, this elevated thrombin may not be able to overcome the bleeding phenotype at presentation when platelets are most profoundly decreased or absent. Greater understanding of the etiology of this thrombin potential in childhood acute ITP is critical, as the thrombocytopenia in this disorder may be triggered or linked to a larger post infectious or inflammatory coagulopathy. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Evaluation of the Hemostatic Disorders in Adolescent Girls with Menorrhagia: Experiences from a Tertiary Referral Hospital.

Bleeding disorders are a common cause of menorrhagia in the adolescent age group. We aimed to evaluate the incidence of hemostatic disorders, using clinical and laboratory findings of bleeding disorders in adolescent girls with menorrhagia. A retrospective chart review used to evaluate adolescent girls with menorrhagia who were referred to Yuzuncu Yil University Pediatric Hematology clinic between January 2010 and December 2014. Out of 52 patients referred for investigation, 50 patients were included in the study. The mean age and mean menarche age were 14.8±1.42 (range: 12-17) and 12.47±0.55, respectively. In 42% (n=21) of patients, anemia was detected. In 22% (n=11) of patients, a bleeding disorder was detected: five cases with von Willebrand disease, two cases with acute immune thrombocytopenic purpura, one case with Bernard-Soulier syndrome, one case with Glanzmann thrombasthenia, one case with aplastic anemia and one case with factor X deficiency. The remaining 39 out of the 50 patients were finally diagnosed with dysfunctional uterine bleeding. When compared the patients with bleeding disorders and without bleeding disorders, bleeding from other sites, including gingival bleeding or epistaxis, low platelet counts and prolonged activated partial thromboplastin time were found statistically more frequent in patients with bleeding disorders (p<0.05). Menorrhagia in adolescents is frequently associated with underlying bleeding disorders. Adolescents with heavy menstrual bleeding and a history of nose or gingival bleeding should be evaluated for congenital bleeding disorders.

Investigation of mean platelet volume as a predictor of acute versus chronic immune thrombocytopenic purpura in children: An exploratory analysis

Current difficulty remains in predicting acute versus chronic immune thrombocytopenic purpura (ITP) in children at the time of diagnosis. The purpose of this exploratory retrospective study was to determine the relationship between mean platelet value (MPV) and initial platelet count at time of diagnosis on prediction of acute versus chronic ITP among pediatric patients. When odds ratios were calculated, initial platelet count less than 10,000/μl was the only statistically significant predictor of acute ITP ( i.e. , case resolution). MPV < 8 fl was not a statistically significant predictor of acute ITP, although clinical trends suggested that those with acute ITP had lower MPV at time of diagnosis. This exploratory study does suggest that multi-center prospective study designs are needed for further study to describe if there is a relationship between MPV in determining acute versus chronic ITP. Adequately predicting acute versus chronic ITP at diagnosis could have numerous implications for children and families as they prepare for treatment and help nurses deliver patient-centered interventions.

A Case of Term Pregnant Woman with Pseudothrombocytopenia: An Overlooked Phenomenon

Objective: To report a case of pregnant woman with low thrombocyte count which in fact has spurious thrombocytopenia. Methods: A 40-week pregnant woman was admitted to our obstetric clinic with complaint and signs of active labour. She was hospitalized and followed for the vaginal birth. Results: On laboratory examination, the low platelet (Plt) count as 42 x 103/mm was measured by using blood samples with ethylenediaminetetraacetic acid (EDTA). Patient had no history for echymosis, bleeding disorders and bruising. She underwent a differential diagnosis for thrombocytopenia in term pregnancy for preeclampsia, HELLP syndrome, acute fatty liver disease or immune thrombocytopenic purpura and no pathology was found. The peripheral blood smear showed platelet (plt) clustering with normal count as 181 x 103/mm. She diagnosed as pseudothrombocytopenia. The patient delivered a healthy baby with vaginal way and had no bleeding complication in pospartum period. Conclusion: Thrombocytopenia in pregnancy is a challenging and important issue for obstetrician. Case Report Erbil and Kamuran; BJMMR, 6(12): 1196-1200, 2015; Article no.BJMMR.2015.299 1197 We aimed to point out that low Plt count doesn’t indicate always a serious disease in term pregnancy. Pseudothrombocytopenia should be kept in mind which may cause unnecessary laboratory examination and treatments.