Prevalence Of Immune Thrombocytopenia Research Articles

Immune thrombocytopenia in the elderly: immunosenescent and clinical diversity.

Immune thrombocytopenia in the elderly: immunosenescent and clinical diversity.

Clinical and hematological features of childhood immune thrombocytopenia at Pediatric Center of Hue Central Hospital

Background: Immune thrombocytopenia (ITP) is the most common of the hemorrhagic diseases caused by thrombocytopenia in children. It usually occurs between the ages of 5 and 7 years old and at both sexes. It is difficult to predict ITP basing on bleeding because some severe thrombocytopenia cases have slight purpura or ecchymoses. This study aims to describe the clinical and hematological features of childhood immune thrombocytopenia Methods: This was a cross - sectional study. Patients were stratified according to age (0 to 15 years) and diagnosed ITP at the Pediatric Center of Hue central Hospital. Results: From May 2020 to March 2021, we identified 42 children diagnosed with idiopathic thrombocytopenia. Newly diagnosed ITP was the most common (66.7%) followed by chronic ITP (26.2%) and persistent ITP (7.1%). There was a slight predominance of boys to girls with the male - to - female ratio was 2:1. However, this ratio was dependent on ITP phases. The highest prevalence of immune thrombocytopenia was found under 6 years old. Children in this study usually had a history of preceding infection or vaccination. Natural hemorrhage was 83.3% (skin 95.2%). The most common type of cutaneous bleeding was petechiae (83.3%). Mild to moderate hemorrhages were dominant. Newly diagnosed ITP had less severity of hemorrhage than persistent and chronic ITP. Most children did not have a fever, hepatomegaly, splenomegaly, and lymphadenopathy. The majority of children seem to have severe thrombopenia (66.7%), but the number of platelets was not related to the severity of bleeding. Mean platelet volume was normal. Conclusions: Newly diagnosed ITP was the most common. There was a little clinical and hematological features difference between the ITP phases.

Prevalence of primary immune thrombocytopenia and related healthcare resource utilization among Texas Medicaid beneficiaries

Objective To estimate the prevalence of primary immune thrombocytopenia (ITP) and describe ITP-associated healthcare resource utilization (HRU) among Texas Medicaid beneficiaries. Methods A retrospective analysis using 2012-2015 Texas Medicaid claims data was conducted to estimate the annual prevalence of ITP. HRU was summarized for the 12-month period following initial ITP diagnosis. Logistic regression and generalized linear model were used to investigate predictors for all-cause and ITP-related HRU. Results The average annual prevalence of ITP was 17.0 per 100,000 persons; higher among females vs males (17.4 vs 13.6 per 100,000) and highest among adults aged ≥65 years (36.7 per 100,000). Among 325 patients included in the HRU analyses, 49.2% received ITP therapies. More than half of patients had at least one all-cause emergency department (ED) visit (70.5%) and/or hospitalization (56.0%). One-third (32.6%) experienced at least one ITP-related ED visit and 40.3% had at least one ITP-related hospitalization. Compared to adults aged 18-49 with ITP, children aged 0-4 (odds ratio [OR] = 3.65, p=.0008) and aged 5-17 (OR =2.68, p=.0074) were more likely to have an ITP-related hospitalization; children aged 0-4 (OR =4.36, p=.0005) and children aged 5-17 (OR =4.09, p=.0005) were more likely to have an ITP-related ED visit during the follow-up period. Conclusion There are 17 patients diagnosed with ITP for every 100,000 Texas Medicaid enrollees annually, with higher prevalence in females and the elderly. Children are more likely to experience hospitalizations and ED visits associated with ITP. ITP patients in Texas Medicaid utilize more healthcare resources compared to the general Medicaid population.

Incidence and prevalence of immune thrombocytopenia under the copayment waiver policy for pediatric patients in Korea: Data from the National Health Claims Database

The purpose of this study was to investigate the epidemiology of immune thrombocytopenia (ITP) under the copayment waiver policy for pediatric patients in Korea. The data were collected from the National Health Insurance Claims Database of Korea. ITP was identified based on the diagnostic code D69.38 from the Korean Standard Classification of Diseases. Patients between one and 18 years old, who had at least one health insurance claim for ITP as a final diagnosis, from 1 January 2016, to 31 December 2017, were analyzed. Prevalent cases were defined as patients who used, at least one time, any medical services coded as D69.38. Incident cases were defined as patients who did not use D69.38 coded medical services during the prior year and were newly registered in 2017. The prevalence and incidence of ITP were 24.53 and 13.39 per 100,000 persons. The peak rates were observed in 1-year-old patients. The gender-specific prevalence of ITP was significantly higher in one-year-old males than females. According to the change-point analysis, we found that the prevalence and incidence diminished rapidly at the ages of four and three, respectively. This Korean population-based epidemiological study of ITP provided meaningful insights into the current epidemiology of ITP and demonstrated the implications of interpreting epidemiologic studies to reflect age categorizing and health care system characteristics.

Epidemiology and Management of Immune Thrombocytopenia in Adult Patients in Algeria: A Non-Interventional, Longitudinal, Nationwide Estimation Study

Background: Immune thrombocytopenia (ITP), is an acquired immune mediated disease characterized by a platelet count of <50×103/mm3, accelerated destruction of platelets from peripheral circulation, and impaired platelet production. Diagnosis is based on exclusion of other causes of thrombocytopenia. Treatment goal is to ensure sufficient platelet count to prevent bleeding. ITP occurs mainly in women, either at the onset of puberty, or in their 3rd/4th decade, with higher mortality in older patients. To date, no accurate epidemiological data on ITP is available for Algeria. This study assessed the incidence (by age, sex, diagnosis stage, and province), prevalence, characteristics, and treatment regimens received by ITP patients (pts) in Algeria. Methods: This non-interventional, longitudinal, nationwide estimation study investigated the epidemiology and care of ITP pts treated in public hospitals in Algeria from September 2017─August 2018. Data were collected at inclusion visit on a case report form. Data on bleeding score and platelet count were also collected after study termination. Pts ≥16 years (y) of age, either previously (before 1st September 2017) or newly (September 2017─August 2018) diagnosed with ITP, having given written consent, were included. The incidence and prevalence of ITP were estimated with Poisson distribution (95% confidence interval [CI]). Results: Overall 1,746 pts were listed, of whom 587 without consent. Of the 1,164 enrolled pts, 1,159 were included (male: 264 [22.8%]); 5 pts (0.4%) were excluded for major protocol deviations. Among eligible pts, 173 (14.9%) were newly and 986 (85.1%) had been previously diagnosed with ITP. No comorbidities conferring bleeding risk were found in 966 pts (83.3%). At diagnosis, median age was 36y (quartiles: 25y, 50y) and platelet count (103/mm3) was 0-30 in 124 (71.7%), 30-50 in 20 (11.6%), and 50-100 in 28 (16.2%) pts. Khellaf score was ≤8 for 45 (26.0%) and >8 for 8 (4.6%) pts; 120 pts (69.4%) had missing Khellaf score. Bleeding risk (as per WHO bleeding score) was low (grade 0) in 50 (28.9%), grade I in 24 (13.9%), grade II in 34 (19.7%), grade III in 52 (30.1%), and grade IV in 13 (7.5%) pts. 55 (31.8%) pts were asymptomatic, 58 (33.5%) were ecchymotic, and 60 (34.7%) were severely hemorrhagic. Steroids comprised the major treatment for both new (n [%]: 136 [78.6]) and previously (795 [80.7]) diagnosed ITP pts. First line ITP treatments included corticosteroids (937 [80.8]), immunoglobulins (39 [3.4]), antineoplastic agents (15 [1.3]), rituximab (14 [1.2]) and antibacterials (2 [0.2]). Last/ongoing treatments at inclusion comprised corticosteroids (712 [72.2]), rituximab (56 [5.7]), immunoglobulins (30 [3.0]), romiplostim (19 [1.9]), and antibacterials (4 [0.4]). Among previously diagnosed ITP pts, 172 (17.4%) had a splenectomy with a median time from ITP diagnosis of 19 months. Incidence of ITP in pts ≥16y was 0.85 cases (95% CI: 0.75, 0.96) per 100,000 inhabitants. Incidence increased with age from 0.5 (95% CI: 0.43, 0.68) in pts aged 15-35y to 2.4 (95% CI: 1.60, 3.51) in pts ≥75y old. Female pts had a higher incidence of ITP (1.2 [95% CI: 1.02, 1.37]) vs male pts (0.5 [95% CI: 0.43, 0.67]; male:female ratio 0.3). Incidence of ITP varied with diagnosis stage (asymptomatic: 0.4 [95% CI: 0.31, 0.45]; ecchymosis: 0.1 [95% CI: 0.10, 0.19]; severe hemorrhage: 0.1 [95% CI: 0.06, 0.13]), and province (lowest: 0.1 [95% CI: 0.02, 0.92] in a public site in Tebessa; highest: 1.6 [95% CI: 0.93, 2.89] in a public site in Sidi Bel Abbès). Prevalence of ITP was 5.7 (95% CI: 5.39, 5.93) per 100,000 inhabitants. Prevalence increased with age from 3.2 (95% CI: 2.78, 3.67) in pts aged 15-25y to 11.9 (95% CI: 9.68, 13.08) in pts aged 65-75y, then decreased in pts ≥85y old (7.6 [95% CI: 4.79, 12.06]). Female pts had a higher prevalence of ITP (8.8 [95% CI: 8.33, 9.29]) vs male pts (2.6 [95% CI: 2.34, 2.85]). Conclusion: The incidence and prevalence of ITP in Algeria were higher in females and increased with age conforming to global trends, but lower than occidental data thus requiring further investigations. Corticosteroids were the most commonly used agents in different lines of treatment; splenectomy was the second option in cases of corticosteroid failure. Rituximab and romiplostim were the last/ongoing treatment before inclusion for a small number of pts. The study highlighted the need for more data on the use of romiplostim prior to splenectomy for adult ITP pts in Algeria. Disclosures Tiaiba: Amgen Middle East:Current Employment.Saad:Amgen Inc:Current Employment.

Racial Variation in ITP Prevalence and Rate of Chronic Disease Suggests Biological Differences

Background: Immune thrombocytopenia (ITP) is the most common cause of acquired immune platelet destruction in children. Clinical symptoms range from asymptomatic to significant and even life-threatening bleeding, fatigue, and reduced health-related quality of life. About 75% of affected children experience spontaneous remission, with the remainder developing chronic ITP. Our clinical observations suggest a decreased prevalence of ITP among Black children, although no available studies have evaluated racial or ethnic predisposition to ITP or to chronic disease. We hypothesized that biological differences in Black children alter the prevalence of ITP, relative to the general population, and may affect disease course. Methods: A retrospective analysis evaluating race and ethnicity of all children with ITP treated at Texas Children's Hospital (TCH, Houston, TX) from January 2015-July 2019 was performed, and compared to both the Houston metropolitan area and the TCH Cancer Center 2018 race and ethnicity data. Of the 699 unique patients, race and ethnicity data were unavailable for 24, and 2 patients were excluded (1 with leukemia, 1 with bone marrow failure). The remaining patients were categorized as (1) White, non-Hispanic; (2) Black, non-Hispanic; (3) Hispanic; and (4) Other. Hispanic patients included those who self-identified as (1) White, Hispanic; (2) Black, Hispanic; (3) Asian, Hispanic; (4) Multi-race, Hispanic; and (5) Unknown race, Hispanic. Demographic data was then collected in a second ITP population derived from the Children's Hospital of Philadelphia (CHOP, Philadelphia, PA) and the surrounding metropolitan area. To match the distribution reported in the Houston metro area data, a comparison was conducted focusing on the proportion of Black non-Hispanic patients in each cohort. A chi-squared test with Yates correction was utilized to compare nonparametric categorical data using GraphPad Prism version 8.0.1 for Windows, GraphPad Software, San Diego, California, USA. A p-value of <0.05 was statistically significant. Results: At TCH, there were 673 evaluable ITP patients. Classifying by race only, 42 were Black (6.2%), 564 White (83.8%), and 67 Other (Asian, Mixed Race, Native American, 9.9%). There was a significantly smaller percentage of ITP patients identified as Black, non-Hispanic (n= 40, 5.9%) relative to both the Texas Children's Cancer Center (16% Black, non-Hispanic) and the Houston metropolitan area populations (16% Black, non-Hispanic, p<0.0001). These data are significant given that Texas Children's Hospital sees the majority of children in the Houston metropolitan area, and the demographic data for the Cancer Center accurately reflect the Houston population (see Table). Black, non-Hispanic patients with ITP were more likely to have chronic disease (40% chronic, n=16) than the expected 20-25% in the overall pediatric ITP population. Many of the patients described as Black, non-Hispanic had secondary ITP (20.0%, n=8), most commonly due to systemic lupus erythematosus (SLE), a condition more common amongst Black patients. These findings were consistent at CHOP, with 7% of the 311 included patients classified as Black (n=22), 76% White (n=236) and 17% Other (n=54 including Asian, Middle Eastern, Mixed Race and Native American/Pacific Islander). Again, these proportions are in contrast to the general CHOP patient population (22% Black, 55.5% White and 22.5% other) and the Philadelphia metro population (42.5% Black, 41.5% White, 16% Other). The frequency of chronic ITP was also increased in Black patients in the CHOP cohort (39%, n=7). Conclusions: This analysis suggests a significant difference in the prevalence of ITP by race in children at two large tertiary care centers in the US. We found that Black children were less likely to develop ITP but those who did were more likely to develop chronic disease relative to other races. Race and ethnicity data at both institutions more closely matched their surrounding metropolitan areas, suggesting the observed differences are not due to care or access barriers. These findings support the possibility that damaging genetic variants, altered gene expression and/or other biological differences in immune response may lead to increased risk of ITP in White children, or protective factors result in reduced prevalence of ITP in Black children. Further research to explore these differences is ongoing. Figure Disclosures Lambert: CSL Behring: Consultancy; Amgen: Consultancy, Other; Bayer: Other: Ad boards; Novartis: Other: Ad boards, Research Funding; Shionogi: Consultancy; Kedrion: Consultancy; Sysmex: Consultancy; AstraZeneca: Research Funding; PDSA: Research Funding. Despotovic:Dova: Honoraria; Novartis: Research Funding; Amgen: Research Funding.

Incidence of Primary Immune Thrombocytopenia (ITP) in Adults in One Region of Russia

Introduction. Primary immune thrombocytopenia is a rare disease1. The incidence of ITP is not well estimated in Russia and worldwide. In adults it varies from 1,6 to 3,9/100 000 person-years2-3. The gender and age-associated results are discussed and differ in several investigations4-6.Study objectives: evaluation of the incidence of primary immune thrombocytopenia in adults in one region of RussiaPatients and methods. The data source is the Registry of the patients with primary ITP in Russia. 272 adult patients: 77 males (28%) and 195 females (72%), age from 16 to 89 years (median 44 years) with ITP (ICD-10 code D69.3), newly diagnosed cases during the period from 12 Jan 2014 to 24 May 2016.Results. 221 (81%) cases were newly diagnosed in 12 regions of Russia in which registration was performed most actively - more than 5 cases for the duration of the study. But only one region was selected for the first evaluation of epidemiological characteristics because of the number of reasons. There is one hematological central clinic in this region in which diagnosis of ITP can be verified and patients with ITP are treated and monitored most properly. The early started and fully performed registration process can be regarded as covered most part of region population in this target region.86 cases (27 male, 59 female) were registered in the target region. The gender-age distribution was following: male: age <41 = 10 (37%), age <41-60 = 7 (26%), age >60 = 10 (37%); female: age <29 = 10 (49%), age <41-60 = 15 (25%), age >60 = 15 (25%).The estimated incidence rate in the target region is shown in table 1.The estimated incidence rates in gender-age strata in the target region are demonstrated in table 2.Conclusion. Overall ITP incidence in one region of Russia is 3.20/100 000 person-years. It is compatible to the incidence in other European countries. Our data demonstrate the rise of incidence rate in males with age and its decrease with age in female population.Literature.1) Rodeghiero F., Stasi R., Gernsheimer T., Michel M., Provan D., Arnold D.M., et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood. 2009; 113(11): 2386--93. doi: 10.1182/blood-2008-07-162503.2) Terrell DR, Beebe LA, Vesely SK, Neas BR, Segal JB, George JN. The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports. Am J Hematol. 2010; 85(3): 174-180.3) Moulis G, Palmaro A, Montastruc J-L, Godeau B, Lapeyre-Mestre M, Sailler L. Epidemiology of incident immune thrombocytopenia: a natiowide population-based study in France. Blood. 2014; 124(22): 3308-3315.4) Segal JB, Powe NR. Prevalence of immune thrombocytopenia: analyses of administrative data. J Thromb Haemost 2006; 4: 2377-835) Schoonen WM, Kucera G, Coelson J, et al. Epidemiology of immune thrombocytopenic purpura in the General Practise Research Database. Br J Haematol 2009; 145(2): 235-244.6) Lisukov I.A., Maschan A.A., Shamardina A.V., Chagorova T.V., Davydkin I.L., Sycheva T.M., et al. Immune thrombocytopenia: clinical manifestations and response to therapy. Intermediate analysis of data of the Russian register of patients with primary immune thrombocytopenia and review of literature. Oncogematologiya. 2013; 2: 61--9]. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

French Observatory of Adult' Chronic Immune Thrombocytopenia (ITP) Treated By Thrombopoietin Receptor Agonists (TPO-RAs)

▪Immune Thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated low platelet count (<100x109/L) with a variable risk of bleeding. The prevalence of ITP in France is 25/100000. While drug therapy is indicated in patients with platelet count less than 30x109/L and or with bleeding symptoms, discussions remain about therapeutic strategy to be implemented. The SATURNE study aimed to describe the current therapeutic management of adult ITP patients, to focus on TPO-RAs treated patients, and to assess changes in real life treatment strategy since TPO-RAs' approval.This study was carried out in referral and non-referral centersby hematologists and internists from hospitals or clinics in France. Enrolled patients were adults suffering from persistent (3 to 12 months after diagnosis) or chronic (>12 months) ITP. Patients with a newly diagnosed ITP (<3 months) and patients with secondary ITP (viral infection, lupus, etc.) were excluded.Data were collected online by investigators through an eCRF at inclusion (M0). A subgroup of patients initiating a TPO-RAs treatment during the study period was followed up at M3, M6, M12, M18 and M24. Only M0 data are presented in this abstract as interim analysis.Overall, 48 investigators included 333 patients (278 with chronic ITP and 55 with persistent ITP) over a 19 months period (2012 to 2013). Figure 1 displays the main characteristics including comorbidities and laboratory tests performed at diagnosis. Half ofthe patients (53%) had bleeding manifestations at ITP diagnosis; 10% at time of inclusion. ITP was mainly diagnosed by a hematologist (53%) or an internist (32%) and less frequently by a general practitioner (11%).Patients completed a median of 2 treatment lines before entering the study. Figure 2 shows treatment-lines distribution according to the ITP phase. Most patients had been treated with corticosteroids ± intravenous immunoglobulin (IVIG) (83%) as 1st line treatment. Rituximab was the preferred 2nd line option, far prior to splenectomy (44% vs 14%).A total of 144 patients (123 chronic/ 21 persistent ITP) received TPO-RAs (39% romiplostim/ 33% eltrombopag / 15% both / 13% non specified): 6%, 20%, 34%, 28% and 12% respectively as a 1st line treatment, 2nd, 3rd, 4th, 5th and beyond. At inclusion 75% were still on TPO-RAs. Recently diagnosed patients received 2nd line TPO-RAs in higher proportions: 40% (of 46 patients diagnosed <2 years) vs 15% (of 65 patients diagnosed 2-5 yrs ago) and 7% (of 72 patients diagnosed >5 yrs). TPO-RAs became the 3rd line most used treatment (over 76% for diag. <2 yrs). In parallel, the use of splenectomy decreased from 31% (diag. >5 yrs) to 9% (<2 yrs) in 2nd line, and from 16% to 5% in 3rd line. TPO-RAs treated patients had a more severe ITP, in particular at diagnosis. More patients in this group showed platelet counts less than 30.109/L (71% vs 51%, p<0.0001 at diagnosis / 23% vs 10%, p<0.001 at inclusion), and bleeding manifestations (64% vs 44%, p<0.001 at diagnosis/12% vs 8%, p=0.24 at inclusion). They received an average of 3.2 lines of treatment (against 1.7 in TPO-RAs' non-treated patients, p<0.0001).The SATURNE study supports epidemiological trends observed in current practice in terms of patients and ITP characteristics, and provides current data on comorbidities.The results highlight the increasing use of TPO-RAs as 2nd and 3rd lines for ITP treatment and the decrease of splenectomy use over time. Initiated in 2012, respectively 1 and 2 years after eltrombopag and romiplostim approval, the SATURNE study points out the changes of the management of adult ITP in France.Table 1N=333Age57 ± 20yrWomen190 (57%)Main ITP characteristicsChronic/Persistent ITP278 (84%)/55 (16%)Mean ITP duration*6 ±8yrPlatelet count* - diagnosis - inclusion33±31.109/L 100±83.109/LHemorrhagic manifestations- diagnosis- baseline176 (53%) 32 (10%)White blood cell*8±3.109/LHemoglobin*14±5g/dlGlobular volume*90±6flComorbidities since ITP diagnosisAt least once36%Hypertension17%Diabetes8%Benign/malignant tumors8%Cardiovascular disease6%Diagnostic tests performed at ITP onsetViral serology tests96%Blood smear93%Blood coagulation93%Marrow aspirate78%Antiplatelet antibody52%ITP treatment (at least once since diagnosis all lines combined)Corticosteroids and/or IVIG275 (83%)Rituximab146 (44%)TPO-RAs- eltrombopag- and/or romiplostim144 (43%) 69 (48%) 78 (54%)Splenectomy59 (18%)*mean ± SD [Display omitted] DisclosuresMichel:Roche: Research Funding; AMGEN: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; GSK: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees. Adoue:GSK: Other: Symposium presentations; AMGEN: Other: Symposium presentations; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; OCTAPHARMA: Other: Symposium presentations; LFB: Other: Symposium presentations; PFIZER: Other: Symposium presentations; ACTELION: Other: Symposium presentations. Cheze:Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees. Coppo:Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees. Leclerc-Teffahi:Novartis: Employment. Fernandes:Novartis: Other: CRO. Texier:Novartis: Other: CRO.

Immune thrombocytopenia: epidemiological and clinical features of 216 patients in northwestern Turkey.

We evaluated the clinical features, treatment modalities, treatment responses, and prognosis of our patients with immune thrombocytopenia (ITP). Furthermore, we estimated the frequency of ITP in the Thrace region of Turkey. Two hundred sixteen patients diagnosed with ITP between 2000 and 2012 at our center were retrospectively evaluated. Patients' clinical features, treatments, and responses to treatment modalities were recorded. The mean annual incidence of ITP was 2.92/100,000 (95%CI: 1.57-4.27). The overall prevalence of ITP was 35.1/100,000 (95%CI: 30.3-39.8). The administration of first-line therapy resulted in complete remission (CR) in 76.5 % of patients and partial remission (PR) in 13.6 %. After 5 years, 33 % of patients who were responsive to first-line therapy were still in relapse-free remission. Of patients who were given second-line therapy, CR was obtained in 71.3 % and PR in 14.9 %. The duration of relapse-free remission was longer with splenectomy than with steroids (p < 0.001). Five years after splenectomy, 62 % of patients were in relapse-free remission; contrarily, this was lower with steroids (36 % at 5 years). The annual incidence and prevalence of ITP in northwestern Turkey was similar to data from western countries-at the lower limit for some countries. Effective treatment strategies seem to be steroids as first-line therapy and splenectomy in refractory cases.

The Epidemiology Of Immune Thrombocytopenia and Clinical Features Of Patients At a Single Center In Northwestern Turkey

Introduction There is only a few data about the epidemiology of immune thrombocytopenia (ITP). Until now, no data about the frequency of ITP in Turkey has been reported. It is quite difficult to perform an epidemiologic study because there are multiple pathogenic mechanisms in ITP and no laboratory or clinical data is characteristic for the diagnosis. We determined the epidemiology of ITP in northwestern region of Turkey and we evaluated the clinical features in our ITP patients. Methods Twohundred-and-sixteen patients diagnosed with ITP between 2000-2012 at our center were retrospectively evaluated. Our hospital has been the only tertiary referral center for hematological diseases (benign and malignant) for a mixed rural and urban population of 616000 people (316000 males, 300000 females) for longer than 16 years. The incidence rates and prevalence per 100000 poulation aged ≥16 years were calculated. Results Of 216 ITP patients, 159 (73.6%) were females and 57 (26.4%) were males (female/male: 2.8). The mean annual incidence of ITP was 2.92/100000 (95%CI: 1.57-4.27) and the overall prevalence was 35.1/100000 (95%CI: 30.3-39.8). The prevalence in women (53/100000, 95%CI: 44.8-61.2) was higher than the prevalence in men (18/100000, 95%CI: 15.4-20.6). The mean age at the time of diagnosis was 42.3 years (median age: 40, range:8-87). Of 162 ITP patients who were given first-line therapy, there was complete response (CR) in 124 (76.5%) and partial response (PR) in 22 (13.6%) (Table 1). Seventythree (50%) of the 146 patients who obtained response (CR+PR) with first-line therapy relapsed at a median of 6 months (range: 2-98 months). The median follow-up in patients who did not relapse was 11 months (range: 2-108 months). The frequency of relapse-free remission in patients responsive to first-line therapy was 61% at one year and 52% at 5 years. Thirtynine patients (48.1%) who were responsive to second-line therapy (CR+PR) relapsed at a median of 5.5 months (range: 2-83 months). Twentyfive patients (53.2%) who were steroid-responders relapsed at a median of 6 months (range: 2-83 months). Ten patients (34.5%) who were responsive to splenectomy relapsed at a median of 6.5 months (range: 2-54 months). Splenectomy was performed in 49 ITP patients. In 30 patients, it was the second-line treatment modality; in 13 patients, it was third-line; and in 6, it was fourth-line. Splenectomized patients were followed up for a median of 35.5 months (range: 2-187 months). The median duration from diagnosis until splenectomy was 7 months (range: 2-102). Of 49 patients who underwent splenectomy, 43 (87.8%) had CR and one (2%) had PR. Nine of the 44 patients (20.5%) relapsed at a median of 24 months (range: 5.5-141). The median duration of remission in 35 patients (79.5%) who did not relapse was 30.5 months (range: 2-84 months). When splenectomy and steroids were compared as second-line treatment options, CR rate was higher with splenectomy (p=0.002) and total response (CR+PR) tended to be better (96.7% vs. 82.5%, p=0.09). Patients responsive to steroids as second-line therapy tended to relapse more than patients treated with splenectomy (53.2% vs. 33.3%, p=0.088). When relapse-free remission durations with splenectomy and steroids were compared, it was seen that it was longer with splenectomy (p&lt;0.001). The relapse-free remission rates after splenectomy were 90% at 1 year and 62% at 5 years. These rates were lower with steroid therapy (45% at 1 year, 36% at 5 years). Conclusions The annual incidence and prevalence of ITP in northwestern Turkey was similar to data from western countries –at the lower limit for some countries. Effective treatment strategies seem to be steroids as first-line therapy and splenectomy in refractory cases. Disclosures: No relevant conflicts of interest to declare.

Prevalence of primary immune thrombocytopenia in Oklahoma

To determine the prevalence of immune thrombocytopenia (ITP) in Oklahoma regardless of age, clinical characteristics, insurance status, and source of health care. Patients with ITP were identified by the administrative code ICD-9-CM 287.3 in Oklahoma hematologists' offices for a 2-year period, 2003-2004. Prevalence was estimated separately for children (<16 years old) and adults because of their distinct clinical characteristics. Oklahoma census data for 2000 was used as the denominator. Eighty-seven (94%) of 93 eligible Oklahoma hematologists participated; 620 patients with ITP were identified. The average annual prevalences were as follows: 8.1 (95% CI: 6.7-9.5) per 100,000 children, 12.1 (95% CI: 11.1-13.0) per 100,000 adults, and 11.2 (95% CI: 10.4-12.0) per 100,000 population. Among children and adults less than age 70 years, the prevalence was greater among women. Among adults aged 70 years and older, the prevalence was greater among men. The highest prevalence of ITP was among men age 80 years and older. These data document for the first time the prevalence of ITP regardless of age, clinical characteristics, insurance status, and source of health care. The methodology developed for this prevalence analysis may be adaptable for epidemiologic studies of other uncommon disorders which lack specific diagnostic criteria and are treated primarily by medical specialists. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.

Prevalence of Diagnosed Adult Immune Thrombocytopenia in the United Kingdom

Data regarding the prevalence of immune thrombocytopenia (ITP) is limited, and is derived from North American population-based analyses. Therefore, the authors conducted the first study outside the United States (US) using the United Kingdom (UK) General Practice Research Database (GPRD) to estimate the adult prevalence of ITP in the UK. This study estimated the diagnosed prevalence of ITP in the adult population in UK using the GPRD from January 1, 1992 to December 31, 2009. The unadjusted, overall 18-year period prevalence was 50.29/100,000 (95% CI: 48.51, 52.06). The age- and gender-adjusted, overall 18-year period prevalence was 50.00/100,000 (95% CI: 49.20, 50.90). ITP prevalence was lower in adults aged 18-49 years of age (30.09/100,000, 95% CI: 28.27, 31.90) than in older adults aged 50-64 years of age (58.22/100,000, 95% CI: 53.88, 62.57) or ≥65 years of age (93.80/100,000, 95% CI: 88.76, 98.85). Prevalence was higher among females (59.32/100,000, 95% CI: 56.63, 62.01) than in males (40.66/100,000, 95% CI: 38.36, 42.96). Prevalence in the GPRD increased over time (1992 [16.33/100,000, 95% CI: 13.70, 19.00], 2000 [36.93/100,000, 95% CI: 34.50, 39.30], and 2009 [58.49/100,000, 95% CI: 55.80, 61.20]). This new analysis of general practice in the UK provides robust prevalence estimates of diagnosed ITP among adults in Europe. ITP prevalence is higher in women and increases with age and over time.

The incidence of thrombocytopenia in children with Cornelia de Lange syndrome

Thrombocytopenia was first reported in Cornelia de Lange syndrome (CdLS) by Froster in 1993. Despite early reports, thrombocytopenia has been rarely reported in this disorder. We performed a retrospective analysis of a large cohort of patients with CdLS. We calculated prevalence of thrombocytopenia in three subsets of this cohort: the entire cohort (n = 1,740), a subset of subjects with substantial clinical records (n = 695) and a subset of subjects with clinical information regarding platelet counts (n = 85). This analysis revealed that 15 have had thrombocytopenia (18% of those with available blood counts); seven had immune thrombocytopenia (ITP). The reported prevalence of pediatric ITP is between 5 and 13 per 100,000 persons. The prevalence of ITP in this cohort is between 7/1,740 and 7/85, giving a relative risk of ITP of between 30 (CI 12-77) and 633 (CI 259-1,549). Contrary to the reported cases in the literature, none of our patients have had progression of the thrombocytopenia nor have they developed other cytopenias. All 15 patients with thromobocytopenia had CdLS based on clinical criteria. Of the 10 patients tested for mutations in NIBPL, 8 had mutations identified. These data support an increased incidence of thrombocytopenia and ITP in CdLS. Subsequently, patients are at risk for spontaneous hemorrhage, and likely increased risk secondary to the high frequency of self-injurious behavior. Although further studies are needed to better define the scope of the problem and to define the mechanisms of thrombocytopenia in CdLS, we would recommend screening for thrombocytopenia upon diagnosis and at 5-year intervals thereafter.