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Context 1
... complete blood count and an examination of the peripheral blood smear are essential in ITP. The princi- pal features of the examination of the blood smear that were identified by the panel are the same for children and adults (Table 5). Although most patients with ITP present with platelet-related bleeding, the condition may be first detected by the incidental discovery of thrombocytopenia on routine blood counts. ...
Context 2
... each patient, thrombocytopenia must be confirmed by direct examination of the peripheral blood smear. The principal elements of the blood smear examination for ITP are described above for children and in Table 5. Particularly in older patients, evi- dence for myelodysplasia should be carefully evaluated, in- cluding the presence of the Pelger-Huet anomaly, nucleated red blood cells, schistocytes, and immature granulocytes."" ...

Citations

... Despite these advancements, many questions about ITP remain unanswered. In 1996, the American Society of Hematology released the first guidelines for diagnosing and managing patients with ITP, which have become the standard references for clinicians managing the disease [9,10]. Furthermore, it is crucial to emphasize shared decision-making in addressing treatment-related issues. ...
Article
Full-text available
Background Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their diagnosis, treatment, and outcomes. This study aims to describe the pilot phase of the national pediatric Immune Thrombocytopenia(ITP) registry (NPITP) in Iran, serving as the inaugural interpretive report. Methods This patient-centered software system was implemented as a national program across multiple pediatric centers in Iran. Several focus groups were conducted to establish a minimum data set (MDS) comprising six main classes, 14 sub-classes, and 187 data elements. Following expert consensus on the final data set, a web-based software tool was developed by the dedicated IT team, accessible online and offline via https://disreg.sbmu.ac.ir/q/ITP.html. The registry included children aged between two months and 18 years with a platelet count below 100 × 10⁹/L, based on predefined inclusion criteria. Results Within a four-month period, a total of 60 ITP patients were registered, including 41 (68.3%) newly diagnosed cases, 68 (13.6%) persistent cases, and 14 (23.3%) with chronic ITP. The mean age of the registered patients was 55.93 ± 9.72 months. The most frequently observed bleeding symptoms were petechiae (68.3%), purpura (51.6%), and ecchymosis (13.3%). Among the newly diagnosed patients, 20 (33.3%) received intravenous immunoglobulin (IVIG), 17 (28.3%) were treated with prednisolone, and 17 (28.3%) received combined IVIG and steroid therapy. Of all patients, 40 (66.7%) demonstrated a complete response to treatment, while 16 (26.7%) exhibited a partial response. Four patients (6.7%) remained unresponsive to therapy. Treatment-related complications, such as Cushing’s syndrome, edema, weight gain, hirsutism, and mood disorders, were reported in 10 patients (16.6%). However, the majority of patients (81.7%) did not experience therapy-related complications. Conclusion The pilot phase of the NPITP registry successfully implemented a web-based software tool for data collection, aiming to enhance the quality of care, facilitate clinical research, and support health service planning in the future.
... (7) For hemolytic anemia and thrombocytopenic disorders with frequent or refractory diseases, splenectomy is an effective treatment approach when the treatment of medical therapy fails. (8) Red blood cell disorders as well as thrombocytopenic disorders are common indications of splenectomy. (9) Splenectomy is also successful in the management of different hematological disorders, (10) particularly sickle cell disease (SCD) and thalassemia which are relatively common in the Middle East (11)(12)(13) and frequently occurred in Yemen. ...
Article
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Objective: To prospectively highlight the indications and outcomes of splenectomy for benign hematological disorders among patients admitted to Al-Kuwait University Hospital, Sana'a, Yemen. Methods: Patients were admitted to Al-Kuwait University Hospital, Sana'a city from Jan 2017 to Dec 2020. They were purposively selected as per inclusion criteria, including only patients with benign hematological disorders. Data were collected by a clinical case sheet in which the demographic, preoperative and postoperative variables (e.g., sex, age, primary diagnosis, indications, and hematological parameters, etc.) were collected. Results: The study included a total of 67 patients aged between 3-29 years old. 37(55.22%) patients were males and 33(44.78%) were females. Most of the study sample were thalassemic patients (40.3%) followed by sickle cell disease (SCD) patients (35.8%). The excessive recurrent blood transfusion is the main indication in thalassemic patients (n=27), hypersplenism (n=14) and sequestration crisis (n=10) in SCD patients. The splenectomy morbidity rate was 40.30% with mostly minor complications and a 3% splenectomy mortality rate was observed in the study. Conclusions: Splenectomy is an effective and reliable approach in the improvement of hematological reserve with a significantly improved effect on the hemoglobin and platelets parameters in our setting.
... The ASH suggests a safe platelet count of at least 50 × 10 9 /L for both vaginal delivery and cesarean section. Platelets less than 10 × 10 9 /L or platelets 10 -30 × 10 9 /L in the second/third trimester or symptomatic bleeding are indications for treatment [6] . The BCSH suggests a safe platelet count of at least 50 × 10 9 /L and 80 × 10 9 /L for vaginal delivery and cesarean section respectively. ...
... The BCSH has recommended the laparoscopic approach for splenectomy. With regards to peripartum management in patients with ITP, the risk of maternal hemorrhage is minimized by ensuring minimum platelet counts required for vaginal delivery, cesarean ~ 9 ~ section and epidural analgesia as stipulated by the ASH or BCSH guidelines [6,7] . The transplacental passage of maternal antiplatelet antibodies in pregnancy can cause fetal thrombocytopenia, and the most feared consequence of this is fetal intracranial hemorrhage during vaginal delivery. ...
... It has been shown that neonatal platelet counts fall to a nadir by day 2. By day 7, the platelet counts should have begun to rise or stabilized. ASH has recommended that infants with platelet counts below 20 × 10 9 /L or symptomatic for bleeding receive IV immunoglobulin [6] . The use of steroids is controversial due to predisposition to neonatal sepsis [12] . ...
... Apenas cerca de 5% das trombocitopenias com contagem de plaquetas abaixo de 30.000 céls/mm³ possuem um risco de hemorragia fatal em indivíduos diagnosticados com PTI (6) . Apesar da trombocitopenia poder atingir valores extremamente baixos na PTI, raramente esses pacientes desenvolvem quadros hemorrágicos graves, sendo mais comum os padrões de sangramento mucocutâneos leves a moderados. ...
Article
A Púrpura Trombocitopênica Idiopática (PTI) é uma doença autoimune caracterizada pela trombocitopenia secundária ao consumo excessivo das plaquetas, que resulta em quadros hemorrágicos de variada duração e gravidade. A cavidade oral está susceptível à presença de eventos hemorrágicos nestes pacientes, mais comumente associados à doença periodontal, ou em decorrência da realização de procedimentos odontológicos invasivos. Em virtude disso, o atendimento odontológico de pacientes portadores de discrasias sanguíneas, como a PTI, é considerado um desafio na terapêutica de cirurgiões dentistas generalistas. O Objetivo deste trabalho é relatar um caso clínico de uma paciente portadora de PTI, submetida a atendimento odontológico de urgência e tratamento periodontal, com foco nas estratégias de manejo clínico utilizadas com vistas a prover uma assistência odontológica segura e minimizar o risco de eventos hemorrágicos orais.
... Gestational thrombocytopenia (GT); -thrombocytopenia during pregnancy (PIT)-occurs late in pregnancy and is more frequent in the last few weeks of pregnancy, as well as in the last few weeks of the second trimester. PIT is generally minor (>100×10 3 μl) and goes away after birth; nevertheless, severe thrombocytopenia occurs seldom [13] . Several studies have reported fetal and/or neonatal thrombocytopenia in 4-13% GT's mothers [14] . ...
... In order to properly assess the evolution of some patients with PTCP, it was necessary to define the diagnostic criteria for ITP. The diagnosis of ITP required that the platelet count was lower than normal (<150 × 10 9 /L) and that there were no other known causes of thrombocytopenia [18,19]. In addition, at least one of the following positive diagnostic criteria were required: (1) the finding of autoantibodies fixed on the platelet surface, (2) an isotopic platelet kinetic study demonstrating a shorter than normal platelet survival, or (3) a clear therapeutic response to immunosupressors or splenectomy [17]. ...
... Acute ITP was that with an abrupt onset with severe thrombocytopenia and significant bleeding. Chronic ITP was that with mild thrombocytopenia and moderate or absent symptoms [18,19]. ...
... Some patients also had biomarkers of autoimmunity, a finding that has been previously described in other series [6,8,12,22]. Our study included healthy individuals with PTCP, with any associated disorder, but not patients with secondary PTCP, since they are entities of a different nature [3,6,10,13,18]. Secondary PTCP is caused by conditions potentially inducing cryptantibodies (referred in the Methods section as exclusion criteria). However, in line with other studies on ITP and on other ADs [17,[23][24][25], we did not consider PTCP with associated ADs as secondary, so they were included in the study. ...
Article
Full-text available
The platelet antibodies that cause pseudothrombocytopenia (PTCP) act only in vitro and do not produce clinical bleeding. Most studies on PTCP have focused on improving differential diagnosis with true thrombocytopenia but studies on the characteristics of patients with PTCP are limited. In this study, we aimed to evaluate the clinical and biological characteristics of 192 patients with PTCP. In addition to general variables, we evaluated automated and microscopic platelet counts, platelet clumps, platelet diameters, immature platelet fraction (IPF), and platelet antibodies. Adult women accounted for the largest subgroup of patients (n=82; 42.7%) and 67 patients (34.9%) were grouped into families. Forty-four patients (22.9%) had one or more associated autoimmune disorders (ADs); 39 relatives of these patients (19.8%) had ADs and 45 relatives (23.4%) had immune thrombocytopenia (ITP) or unspecified thrombocytopenia. Platelet cryptantibodies and/or autoantibodies were positive in 56 patients (30.1%). Most patients (n=169; 80%) had automated platelet counts >80×10⁹/L. In all patients, microscopic platelet counts were ≥150×10⁹/L. The platelet clump index (% increase in microscopic platelet count compared to automatic count) ranged from 30 to >7000%. Platelet diameters and IPF parameters were significantly greater in the PTCP versus healthy controls (p<0.001). A total of 17 patients (8.8%) had had previous ITP or the PTCP evolved into ITP. Our data suggest that PTCP should be considered a situation of autoimmunity; the assessment of platelet clumps has a high diagnostic value; the close association between ITP and PTCP suggests that these conditions could be different phases of the same process.
... Other causes include genetic factors (immune genes-FcR, immune syndromes, platelet antigens) and susceptibility to the initial event (infection, inflammation) [33]. Immune thrombocytopenia is characterized by an increased risk of mucocutaneous bleeding with a low platelet count [34][35][36][37][38]. ...
... The diagnosis of immune thrombocytopenia is made by excluding other factors that cause thrombocytopenia. The annual incidence of ITP in adults is 1.6-6.6 per 100,000 people [31][32][33][34], more prevalent in women in the middle age group (30-60 years), and approximately equal for the sexes in all ages [35,36]. The hypothesized initial cause of ITP has increased destruction of platelets at a rate that exceeds the production by the bone marrow. ...
Article
Full-text available
Haemostasis disorders are serious pathologies that could put dental and surgical procedures at risk as they are associated with postoperative bleeding, which in some circumstances could be prolonged and dangerous for the patient. In-depth knowledge of the problems associated with coagulation pathologies and the suitable specific procedures should be implemented in dental practice. A good awareness of the clinical protocols to be used in these circumstances may help reduce operator stress and increase patient compliance. Collaboration with the haematologist is always recommended to establish an adequate treatment plan, both regarding the administration of therapies that promote haemostasis and for assessing the operative risk. Hereby, we summarize the congenital and hereditary pathologies that lead to haemostasis disorders, which can be found in patients undergoing dental procedures. The purpose of this narrative review is to frame the diseases from a clinical, anamnestic, and etiopathological standpoint, as well as to evaluate an operative approach to the pathology under consideration, with particular attention to anaesthesia manoeuvres and post-surgical haemostasis, to avoid hematoma formation and uncontrolled bleeding which can lead procedure failure up and even death. Of note, it is likewise important to educate the patient about prevention, to keep the oral cavity healthy and avoid invasive procedures, limiting the number of operative sessions.
... ; https://doi.org/10.1101/2023.01. 16.23284594 doi: medRxiv preprint deviation; skewed distribution calculates median and interquartile range. ...
... [15] The American Society of Hematology (ASH) first published guidelines for ITP in 1996, recommending glucocorticoids, IVIg, and splenectomy as initial treatment, and accessory splenectomy, danazol, and azathioprine as complementary treatment. [16] In 2019, ASH updated the ITP guidelines, recommending glucocorticoids, IVIg as initial treatment and rituximab, eltrombopag, romiplostim, and splenectomy as complementary treatment. [17] It is the large number of clinical trials on rituximab, eltrombopag, romiplostim . ...
Preprint
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Objective: The purpose is to clarify the overall situation of clinical related to primary immune thrombocytopenia (ITP), to evaluate the difference between published clinical trials and unpublished trials, and to evaluate the relevant information of trial publication. Methods: Search the ClinicalTrials.gov database on March 20, 2022 to identify ITP clinical trials and obtain relevant data. Publications in PubMed were searched using standardized methods to identify the publication of completed clinical trials. Results: Of 341 trials identified, interventional trials were the most common (74.2%, n=253). Interventional trials and observational trials differ in the main research content (odds ratio (OR) 0.06, 95% confidence interval (CI) 0.03-0.015, P=0.000). In terms of published articles, there are few trials involving non drugs (OR 0.23, CI 0.08-0.64, P = 0.005). There were fewer trials with less than 10 participants (OR 1.52, CI 1.06-2.20, P = 0.024). Of the 167 completed trials, 93 (55.7%) were published and 48 (28.7%) uploaded results. Conclusion: If the main research content involves drugs, trials with a larger number of people are more likely to be published. The publication rate of ITP clinical trials is high, but the submission rate of database results is low. Therefore, more attention should be paid to the submission of clinical trial results in the later stage.
... However, 3-5% of children with ITP face a greater risk of bleeding, resulting in significant morbidity and mortality [7], and children with severe thrombocytopenia (platelet counts below the threshold of approximately 30 × 10 9 /L) require investigation and treatment. Importantly, about 20% of children with newly diagnosed ITP may develop chronic thrombocytopenia status (e.g., platelet counts < 100 × 10 9 /L persisting after 12 months) [3,[8][9][10][11]. ...
Article
Full-text available
Childhood immune thrombocytopenia (ITP; platelet count < 100 × 109/L) is the most common bleeding disorder in children. A total of 3–5% of children with ITP face a greater risk of bleeding, resulting in significant morbidity and mortality. Childhood ITP is often benign and self-limited; however, children with severe ITP (platelet count < 30 × 109/L) require investigation and monitoring. In addition, 20% of ITP patients may not go into remission (platelet counts < 100 × 109/L by 12 months after diagnosis) and may develop chronic ITP. The early identifying predictors associated with the resolution of severe ITP at the time of diagnosis may be helpful for family guidance. However, there is still controversy about the associations between the clinical factors at the time of initial diagnosis and the definitions of disease remission assessed at different timepoints after diagnosis. This retrospective study aimed to analyze the shared clinical factors among the disease remission definitions at three arbitrarily set timepoints—3, 6, and 12 months after diagnosis. This study retrieved records for hospitalized children aged under 18 years and diagnosed with ITP from the hospital registry in a tertiary university hospital. Clinical variables were recorded by reviewing the medical records with structured data entry for ITP admission. The serial follow-up platelet counts within 12 months after diagnosis were recorded. The times of ITP remission were identified by experienced pediatric hematologists. Patients with mild-form ITP (platelet counts ≥ 30 × 109/L) at diagnosis or who were lost to follow-up within 3 months were excluded. From 1988 to 2019, 546 children were enrolled, and a total of 497 children with severe ITP were included in the further analysis. In total, one (0.2%) died of an intracranial hemorrhage, 363 (73.2%) children went into remission at 3 months, 40 (8.1%) went into remission between 6 and 12 months, and 104 (20.9%) developed chronic ITP. The shared significant predictors for remission by the third, sixth, and twelfth months included pre-adolescent age (<10 years) at diagnosis, abrupt onset (duration of symptoms prior to admission ≤ 2 weeks), and speedy recovery (platelet count > 100 × 109/L at 1 month post diagnosis). ITP patients with positive viral serology tests or vaccination within 4 weeks had trends of delayed remission. In conclusion, diagnosis before preadolescent age, abrupt onset, and speedy recovery may share favorable factors for the remission of childhood ITP assessed at different timepoints.
... Elle se rapproche des formes adultes par une évolution chronique. Elle touche 20% des filles de plus de 10 ans avec une moyenne d'âge de 7,8 ans mais un taux de [8]. Le diagnostic est donc basé sur l'histoire de la maladie, l'examen physique, la formule sanguine complète et le frottis sanguin qui vont permettre d'exclure d'autres causes de thrombopénie. ...
Article
Full-text available
Immune thrombocytopenia (ITP) is one of the main causes of childhood thrombocytopenia. We report the case of a 12-month-old infant with a complicated ITP of severe anemia. The management was done according to the accepted recommendations and the outcome was favorable. The purpose of this case report is to draw attention on a diagnosis which is not often mentioned in our context because the color of the skin makes petechiae barely visible. However, this disease can lead to death in case of severe acute external bleeding.