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Immune thrombocytopenia (ITP) continues to intrigue pediatricians and hematologists alike. Patients can have a dramatic presentation with wide-spread bleeds over a few days. There is an aura and fear of intra-cranial hemorrhage that drives the physician to recommend and the patient's family to accept drug treatment. Difference of opinion among phys...
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Background
Immune thrombocytopenia (ITP) is a rare disorder characterized by low platelet counts and an increased tendency to bleed. The goal of ITP therapy is to treat or prevent bleeding. Actual rates of bleeding are unknown. Clinical trial data may not reflect real-world bleeding rates because of the inclusion of highly refractory patients and m...
Citations
... It should be noted that symptomatic bleeding is rare, except in severe cases of ITP. Common symptoms in children include gum bleeding, epistaxis, purpura, and easy bruising [25]. Petechiae, purpura, and ecchymosis were the most frequently reported bleeding manifestations in the NPITP registry. ...
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.
... The diagnosis of ITP is typically made based on clinical presentation and laboratory findings and is a diagnosis of exclusion. 4 Treating children with ITP aims to raise their platelet count to a safer level that is less risky to prevent severe bleeding, particularly ICH. 5 Since the 1950s, corticosteroids have been effectively utilized. The effects of corticosteroids are caused by a reduction in the production of antiplatelet antibodies and a decrease in the clearance of opsonized platelets. ...
Key Clinical Message
This case report documents the unusual co‐occurrence of immune thrombocytopena (ITP) and chickenpox in a 15‐year‐old girl. Initial symptoms included shortness of breath, chest pain, and heavy menstrual bleeding. Laboratory results revealed significant anemia and thrombocytopenia. Treatment involved blood transfusions, prednisolone, and iron supplementation. The patient's vesicular skin rash emerged 8 weeks later, prompting the combined diagnosis of ITP and chickenpox. Antiviral treatments, blood transfusions, and supportive care were used in the course of treatment, leading to full recovery. This case emphasizes the importance of prompt diagnosis, appropriate management, and regular follow‐up for patients with both chickenpox and ITP. The coexistence of chickenpox and ITP poses a clinical challenge due to the complex interaction between the viral infection and the immune system. The exact mechanism linking these two conditions remains unclear, making it a baffling case that warrants investigation and further understanding. As low is the occurrence of hemorrhagic chickenpox, the presentation of simultaneous chicken pox with or following ITP was found to be rarer, and thus is this enigmatic case presented. Healthcare providers should remain vigilant about such co‐occurrences to prevent complications. In order to improve treatment for instances with comparable clinical presentations and advance our collective knowledge, further study is required to better understand the mechanisms relating viral infections and ITP.
... [56] It is a safer, less invasive, and faster intervention than operative splenectomy in a sick thrombocytopenic patient. [57] Availability of expertise and data regarding its use in ITP is limited. Splenic irradiation is utilized as an alternative to splenectomy to increase the platelet count in patients with life-threatening bleeding. ...
... These patients need to be vaccinated against encapsulated organism and need to continue long-term antibiotic prophylaxis. [57] Newer agents that have been tried are listed in Table 4. ...
J Appl Sci Clin Pract 2021;2:33-41.
... Typical clinical features include sudden onset of skin bleeds along with pallor proportionate to bleeds and absence of fever, lymphadenopathy, hepatosplenomegaly, bone pains. 7 No confirmatory diagnostic tests are available. Complete blood counts show isolated thrombocytopenia with peripheral smear showing giant platelets. ...
Immune Thrombocytopenia (ITP) is a hematologic disorder characterized by immune mediated destruction of platelets leading to isolated thrombocytopenia. It presents as sudden onset of acute self-limiting episodes of bleeds which are usually minor but may present with intracranial hemorrhage. ITP is a clinical diagnosis of exclusion. No confirmatory diagnostic tests are available. Management depends on severity of bleeding rather than platelet counts. Here is a case report of a 16 years old female patient presenting with multiple somatic complaints and no history of bleeding manifestations with isolated thrombocytopenia and hematologic malignancy ruled out.
... Symptoms usually appear if the platelet count drops below 20×10 9 /L. Usually, the lower the 1 2 1 1 1 3 platelet count is, the more severe the condition is [3][4][5]. ITP is diagnosed after excluding other causes of thrombocytopenia. ...
Background
Immune thrombocytopenic purpura (ITP) can be defined as “an immune-mediated acquired disease characterized by a transient or persistent decrease in the platelet count”. Medical treatment is usually not needed but, in some cases, intravenous immunoglobulin G (IVIG), corticosteroids, and anti-D immunoglobulins are used. Splenectomy can be an option for chronic cases with no response to pharmacological treatments. The aim of this study was to describe the clinical characteristics and outcomes of pediatric patients with ITP in King Abdulaziz Medical City (KAMC) and King Abdullah Specialist Children’s Hospital (KASCH) in a 10-year period.
Methods
The study was conducted at KAMC and KASCH. The number of recorded cases was 95, which included all ITP patients aged 1 to 14 from both genders who presented to KAMC previously and KASCH currently from January 1, 2007, to December 31, 2017. The data analysis and entry were performed using the Statistical Package for the Social Sciences (SPSS) version 25 (IBM Corp., Armonk, NY).
Results
Among 95 pediatric patients with ITP, 51 (53.7%) were males and 44 (46.3%) were females, with a median age of 4.00 ± 3.977. Among them, 84 (92.7%) had purpura, 38 (46.3%) had epistaxis, 43 (39%) had petechia, 17 (24.3%) had fever, and 20 (19.5%) had gum bleeding. Out of 95 patients, 91 (95.8%) were given treatment. Out of those 91 patients who were given treatment, IVIG was used in 84 (92.3%), steroids were used in 44 (48.4%), 14 patients received platelet transfusion (15.4%), rituximab was used in 7 (7.7%), and splenectomy was done in 5 (5.5%); 32 (33.7%) cases were considered chronic (more than one year), and 63 (66.3%) were considered acute. Among chronic patients, only one death was recorded, while in acute, no deaths were recorded.
Conclusion
In conclusion, ITP is an autoimmune disease that decreases platelet count. The results showed a significant difference in treatment compared to the literature but similar results in other aspects.
... In the final review of this symposium, Singh et al. address frequently asked questions in ITP [12]. Given the common occurrence of ITP, the debate on the benefits and risks of the multitude of therapies available for ITP vs. conservative management [13][14][15] is familiar to all of us. Eltrombopag and romiplostim are preferred options to treat children with persistent or chronic ITP in high-income countries, but there are no guidelines on the duration of therapy [12]. ...
... Steroid therapy comprised of; in cases of acute ITP when there was severe bleeding treatment start with Methylprednisolone 10 mglkg and increasing dose up to 30 mglkg can be used [13]. In case of mild to moderate bleeding patients received oral prednisolone 2mglkg,cases not responding to steroid and with active bleeding,they were given IV Immunoglobulin,few cases received platelets transfusion,the Steroid Non-responders were excluded from The study. ...
Childhood Idiopathic thrombocytopenic purpura (ITP) is one of the most commons autoimmune bleeding disorders characterized by isolated, immune-mediated low platelet count, the T-follicular helper (Tfh) cells are a subset of effector CD4 (+) T cells, that plays a pivotal role in maintaining self-tolerance, deregulation of Tfh activities has a key role in immune process taking place in ITP in which the production of platelet autoantibodies might be caused by cytokine network dysregulation. The objective of our study was to analyze the relationship of Tfh cells CD4&CD25 and C-X-C ligand 13 (CXCL13) expressions before and after steroid thereby in pediatric ITP. Material and method: the study included 45 pediatric patients with acute ITP and 20 healthy controls; we used flowcytometry to assess percentages of CD4 and CD25 cells as markers of regulatory T cells, also, the serum level of interleukin- CXCL13 was measured by ELISA at diagnosis and after 4 weeks receiving corticosteroid. Results: the expression of CD4 & CD25 markers were significantly reduced in ITP cases, who also showed an elevated CXCL13 level in comparison to controls, however, the level of CXCL13 declined after treatment, the CXCL13 optimum cut off point for predicting ITP response to therapy was determined to be 90 pg/ml with AUC 0.976, Sensitivity 88.89% and Specificity 100% P-value < 0.00. Conclusion: Serum CXCL-13 levels could be used as a significant predictor of response to therapy in ITP patients, CD4&CD25 expression has a role in the pathogenesis of childhood acute ITP principally linked to the level of platelet count drop. Keywords: Immune thrombocytopenia (ITP), Flowcytometry, T follicular helper cells, CXCL-13 chemokine, Pediatric, Autoimmune disease
... Parameters that has been suggested to denote excessive bleeding include, clots greater than 1 inch, bleeding resulting in iron defi- ciency anaemia, and frequency of pad change more than every 3 hours. Whereas severe acute bleeding is defined as bleeding that requires more than 1 pad or tampon per hour or symptoms of hypovolemia [1]. Menorrhagia occurring during adolescence is usually presumed to be associated with anovulation. ...
Menorrhagia is a one of the most common presenting complaints seen in modern gynecology. Common causes include those recently proposed by FIGO using the acronym PALM COEIN (polyp; adenomyosis; leiomyoma; malignancy and hyperplasia; coagulopathy; ovulatory dysfunction; endometrial; iatrogenic; and not yet classified). Very rarely menorrhagia is due to an inherited bleeding condition.
Familial congenital thrombocytopenia is also known to cause menorrhagia although case reports are very sparse and confirmed cases seldom seen in gynecology practice.
We present a rare case of severe intractable menorrhagia occurring with the first period in a 12 year old girl who has a positive family history of bleeding disorder. This patient was recently managed shortly after menarche in our unit and presented with a series of challenges in terms of diagnosis, management and therapy. There was no response to initial treatment with blood, blood products as well as antifibrinolytics. Hormonal therapy was tried eventually as bleeding continued despite these measures. The patient also
suffered a thrombotic stroke with right hemiparesis while she was still bleeding. This presented a therapeutic dilemma as we had to balance measures to control the vaginal bleeding which are pro-thrombotic and at the same time trying to preventing further strokes/evolvement of her stroke with anticoagulants and thrombolytics.
... Typical findings include pallor, lymphadenopathy, petechiae, purpurae, bony tenderness and hepatosplenomegaly. Differential diagnosis and approach to a child with bleeding and idiopathic thrombocytopenic purpura have been elaborated in recent reviews (1).Not uncommon manifestations include, pyrexia of unknown origin, limping, back ache, etc., in absence of organomegaly (2). Complete blood count may reveal variable findings, including anemia, thrombocytopenia, a normal, reduced or elevated white count, lymphocytic predominance and often (not necessary) blasts in the peripheral smear (3). ...
Newly diagnosed immune thrombocytopenia (ITP) is a relatively common disorder of childhood that does not require an exhaustive laboratory workup for diagnosis. A history and physical exam with a review of the peripheral smear are crucial for excluding secondary causes of thrombocytopenia. Several guidelines have been published to guide physicians in the management of ITP. However, the decision for treatment can be arduous. The management strategy should not be focussed on the platelet count but the severity of bleeding symptoms. Agents for treating acute ITP, including corticosteroids, immunoglobulin and anti-D immunoglobulin, do not seem to have a significant impact on the natural history of the disease. The majority of children with ITP do not need therapy and have a spontaneous resolution of the disease. Some children can develop chronic ITP that is not commonly life-threatening but can lead to impaired quality of life. Traditional therapies such as rituximab and splenectomy for chronic ITP are not without significant adverse effects. Thrombopoietin receptor agonists are newer agents for the treatment of chronic ITP and hold promise, however, their cost currently precludes use in most of the patients in low-middle-income countries. This review compares and contrasts the specific treatments available for the treatment of ITP to help the reader make a balanced choice. This review, based on a series of case examples, will help physicians in making decisions about choosing a practical management strategy for patients with newly diagnosed as well as chronic ITP.
