Sunday, December 8, 2019

Immune Thrombocytopenic Purpura (THROM-bo-si-toe-PE-one-ah) or ITP is

Question: Discuss about the Immune Thrombocytopenic Purpura. Answer: Immune Thrombocytopenic Purpura Figure 1 Immune Thrombocytopenic Purpura (Lo Deane, 2014) Definition Immune Thrombocytopenic Purpura (THROM-bo-si-toe-PE-one-ah) or ITP is defined as low platelet count that results into a bleeding disorder whereby clotting of the blood cannot take place. When platelets are not enough to facilitate the healing of small cuts and abrasions under the skin, one experiences external bleeding(Bennett, Neunert, Imbach, Grace, VeselyKuhne, 2016) When they cannot facilitate the sealing of injuries on the walls of blood vessels one experiences internal bleeding. People with ITP have purple bruises known as purpura which appear on the skin or the mucous membranes found in places such as the mouth. The bleeding disorder may also cause purple or red pin-pointed dots on the skin called petechiae (Kashiwagi Tomiyama, 2015). There are two types of ITP namely acute and chronic ITP. The former is characterized by a sudden trigger of viral or bacterial infections and symptoms disappear in 6 months without future recurrence. Chronic ITP is recurrent and can occur at any age with symptoms lasting for a minimum of 6 months (Lo, Deane, 2014). However, with medical care, the increase of platelet to a reasonable level is important; this will enable the patient to live a normal life. However, children suffering from delicate ITP do not need treatment as the condition will resolve spontaneously. ITP has no cure; however, an induced remission can be used to rectify the condition. Etiology and Incidence In adults, the bodys immune system produces antibodies that attack and damage the blood platelets and also inhibits the production of platelets by the bone marrow. In some childhood cases, the bleeding disorder is linked to bacterial and viral infections such as hepatitis C which may set off the autoimmune reaction that causes ITP (Hassan et al. 2014). Virus infections such as chickenpox and measles in children could trigger ITP. Acute ITP is the most common form of ITP especially in children of ages between two and six years. Chronic ITP is more common among adults than children although it does not happen in adolescents.80% cases of ITP are estimated to be idiopathic or primary while the other 20% is secondary, meaning that they are as a result of other disorders such as hepatitis C (Haq, Darwish, Arias, Stein, Shabbir, Bussel, 2014).The incidence of ITP is considered to be 50-100 diagnosed cases in a million people annually, half of them being children. About 70% of the childhood cases end up in remission in 6months with or without treatment. Chronic ITP among adults has a 20-40% chance of recurrence. A third of the chronic cases remit upon follow-up observations while another third ends up with mild ITP (Zhang, Guo, Haq, West, Bussel Zehnder, 2014). Among adults, the male to female ratio ranges from 1:1.2 to 1.7 with the median age of adults diagnosed with ITP being 56-60. The ratio of male to female adult ITP cases seems to widen as their ages advance.ITP cases among children are almost equal in both genders (Iyenga Maheshwari Jain, 2016). Pathophysiology According to the Harrington-Hollingsworth experiment, the immune system produces antibodies of the immunoglobulin G (Ig G) that target and coats the platelet membrane glycoproteins (Foord, Hoang, Hsieh Nugent, 2015) The autoantibodies also destroy the platelets precursor cells called megakaryocytes which slightly reduces the platelets count. Recent studies reveal that ineffective production of the glycoprotein hormone thrombopoietin which stimulates the production of platelets is a factor that reduces the number of platelets (Salve, Gupta, 2014). The auto-antibody production is an abnormal T cell mechanism in ITP which can be adjusted using drugs such as rituximab which targets the B cells (Nishimoto Kuwana, 2013). Clinical Manifestations Related to Pathophysiology Additionally, when there is a low blood platelet count of 20,000 per l, the purpura and petechiae bruises and spots spontaneously appear on the skin (Grainger, Maggs Elizabeth, 2016). In extreme cases, there is excessive menstrual flow in women and bleeding of the gums and nostrils. At a severe low count of less than 10,000 per l, blood masses may form in the mouth and other mucous membranes (Malpass, Lavigne, Pope, Oesterheld, 2015). The bleeding from minor cuts and abrasions is longer than usual due to the inability of the blood to clot. An extremely low number of platelets of less than 5,000 per l warrant critical complications such as bleeding in the skull or brain and another internal bleeding which can be caused by a blunt internal trauma (Altomare, Cetin, Wetten Wasser, 2016). Pharmacological treatment Surgery can be carried out to remove the spleen since the platelets bound by antibodies are used up by macrophages in the spleen. In the case of an emergency, platelets may be transfused to increase the short-term platelet count (Cines, Gernsheimer, Wasser, Godeau, Provan, Lyons Lopez, 2015). Immunosuppressive medications and corticosteroids may be administered to suppress the immune systems ability to produce the antibodies that hinder the circulation of platelets. Additionally, the thrombopoietin receptor agonists could also be administered in order to stimulate the production of platelets in the bone marrow (Ma, Chen, Wu, 2014). Conclusion An isolated number of platelets is the hallmark of ITP which manifests itself in excessive bleeding due to the insufficiency of the blood cells responsible for the clotting of blood. In most cases, ITP is not an extremely life-threatening disorder like other autoimmune diseases such as multiple sclerosis which are progressive disorders. This is because acute ITP does not necessarily need treatment and it disappears within a few weeks or months without future recurrence. The platelet count is expected to return to normal within 6-12 months. However, chronic ITP can last for several years and people suffering from it can terminate treatment and maintain a platelet count that is safe for their health. Treatment for ITP is only recommended where there is significant bleeding that puts a patient at the risk of morbidity or mortality due to severe hemorrhage. References Altomare, I., Cetin, K., Wetten, S., Wasser, J. S. (2016). The rate of bleeding-related episodes in adult patients with primary immune thrombocytopenia: a retrospective cohort study using a large administrative medical claims database in the US.Clinical Epidemiology,8, 231. Bennett, C., Neunert, C., Imbach, P., Grace, R., Vesely, S., Kuhne, T. (2016). Predictors of Remission in Pediatric Immune Thrombocytopenia: An Analysis of the Intercontinental ChildhoodStudyGroup Registry Ii Data.Pediatric Blood Cancer,63, S14. Cines, D. B., Gernsheimer, T., Wasser, J., Godeau, B., Provan, D., Lyons, R., Lopez, A. (2015). Integrated analysis of long-term safety in patients with chronic immune thrombocytopenia (ITP) treated with the thrombopoietin (TPO) receptor agonist romiplostim.International journal of hematology,102(3), 259-270. Grainger, J. D., Bolton-Maggs, P. H., Elizabeth, P. (2016). Response to First-Line Treatment in Childhood ITP. Foord, A., Hoang, J., Hsieh, L., Nugent, D. J. (2015). A New Look into Childhood Acute ITP: The Relationship between Thrombin Generation and Thrombocytopenia.Blood,126(23), 1058-1058. Iyenga, D. P., Maheshwari, D. U., Jain, D. A. (2016). Incidence, Pattern, and Characterization of Immune Thrombocytopenic Purpura.International Journal of Scientific Research,5(6). Haq, N., Darwish, S., Arias, V., Stein, J., Shabbir, E., Bussel, J. (2014, June). A SURVEY OF THE ETIOLOGY OF IMMUNE THROMBOCYTOPENIA (ITP). InHAEMATOLOGICA(Vol. 99, pp. 770-771). VIA GIUSEPPE BELLI 4, 27100 PAVIA, ITALY: FERRATA STORTI FOUNDATION. Hassan, S. S., Doobaree, U., Nandigam, R., Taylor, L., Bradford, C. M., Hoti, I., Pington, D. J. (2014). Understanding T cell involvement in human immune thrombocytopenia (ITP).British Journal of Haematology,165(1), 1-103. Kashiwagi, H., Tomiyama, Y. (2015). Recent advances in pathophysiology and treatment of immune thrombocytopenia.[Rinshoketsueki] The Japanese journal of clinical hematology,56(2), 177-184. Keim-Malpass, J., Lavigne, J. L., Pope, J., Oesterheld, J. (2015). Investigation of mean platelet volume as a predictor of acute versus chronic immune thrombocytopenic purpura in children: An exploratory analysis.Clinical Nursing Studies,3(3), p23. Lo, E., Deane, S. (2014). Diagnosis and classification of immune-mediated thrombocytopenia.Autoimmunity reviews,13(4), 577-583. Ma, J. Y., Chen, Z. P., Wu, R. H. (2014). The relationship between platelet specific antibodies and the onset, clinical manifestation, treatment and prognosis of ITP.Zhongguoshiyanxue ye Xue Zhi,22(6), 1771-1774. Nishimoto, T., Kuwana, M. (2013, January). CD4+ CD25+ Foxp3+ regulatory T cells in the pathophysiology of immune thrombocytopenia. InSeminars in hematology(Vol. 50, pp. S43-S49). WB Saunders. Salve, D. P., Gupta, D. N. (2014). Oral Manifestations of Idiopathic Thrombocytopenic Purpura: A Rare Case Report.International journal of scientific research,3(8). Zhang, H., Zhang, B., Guo, X., Haq, N., West, R. B., Bussel, J. B., Zehnder, J. L. (2014). Effects of ThrombopoietinMimetics on Patients with Chronic ITP: Perspectives from Blood Transcriptome Profiling Analysis.Blood,124(21), 2780-2780.

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