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Idiopathic Thrombocytopenic Purpura (ITP)
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Idiopathic Thrombocytopenic Purpura (ITP)
In this case study, Carolyn is a perfectly healthy female besides she recently developed of red dots over his skin and gums. He has a prolonged clotting screen and is diagnosed with idiopathic thrombocytopenic purpura (ITP).
According to Silverman and Dyne (2015), ITP is also known as primary immune thrombocytopenic purpura and autoimmune thrombocytopenic purpura. They defined ITP as an isolated type thrombocytopenia in which the bone marrow is normal and all other causes of thrombocytopenia are adequately excluded. It occurs acute in pediatric population while it presents as a chronic disease in adults.
According to Stasi and Newland (2011), up to 40% of adults develop no clinical manifestation at all and on most cases, the only presentation is localized hemorrhage in skin or mucosal surfaces. This is usually harmless. In a rare instance, it can be associated with severe uncontrolled bleeding event like intracranial hemorrhage (ICH). Purpura is a Latin word which means ‘purple’. Even though scientists talked about platelets in their various experiments but none of them gave a better description until 1865 when Max Schultze published an accurate description and referred platelets as ‘dust of the blood’.
George and Arnold (2015) described a few causes of ITP and suggested that it can be drug-induced, congenital, chronic liver disease, suppressed bone marrow, hypersplenism, pregnancy induced, human immunodeficiency virus (HIV), hepatitis C virus (HCV), chronic lymphocytic leukemia (CLL) and systemic lupus erythematosus (SLE).
Mohan (2010) stated that pathogenesis of ITP depends upon the duration of illness; acute and chronic.
For acute ITP, it is postulated that it is a self-limiting disorder and frequently affects the pediatric population only followed by a viral illness like infectious mononucleosis or HIV infection. The onset is sudden with severe attack of thrombocytopenia resolving within a week to 6 months. There is formation of viral antigen containing immune complexes which leads to formation of antibodies against them. These antibodies cross-reacts with platelets leading to their destruction.
Chronic ITP is a disease of adulthood and particularly affects women in their reproductive years. Its onset is insidious which may persist for several years. Its etiology is idiopathic but it has been postulated that the same immune complexes are the culprit behind immunologic destruction of platelets. Such an association can be seen in SLE, AIDS or autoimmune thyroiditis. The exact pathogenesis is elucidated by formation of anti-platelet antibodies which are IgG humoral antibodies formed in the spleen. These antibodies target the platelet glycoproteins Gp IIb-IIIa and Gp Ib-IX complexes. Sensitized platelets are then destroyed in the spleen and are phagocytosed by cells of reticulo-endothelial system.
According to Papadakis and McPhee (2015), patient of ITP may present with mucocutaneous bleeding which is variable depending on the platelet count of the patient. The symptoms are mild and barely noticeable besides appearance of bruises over skin. Patients may also experience nosebleeds, bleeding from the gums or gingiva. The authors also stated that other types of bleeding like intracranial hemorrhage does not occur till the platelet count is less than 20,000 – 30,000/mcL.
On laboratory investigations, patients have isolated thrombocytopenia with mild anemia secondary to bleeding. Serologic exclusion of HCV, HIV and Hepatitis B virus should be done. Patients older than 60 years who have cytopenia should have their bone marrow examined. ITP is a diagnosis of exclusion and absence of these findings in an asymptomatic patient with unexplained isolated thrombocytopenia renders the diagnosis of ITP.
According to Silverman and Dyne (2015), in the US the incident rates for ITP in adults is nearly 66 cases/ 1,000,000 per annum with 50% of the cases affecting children. In chronic type of ITP, the female to male ratio is 2.6:1 with 72% of more than 10 years old patient being females. In acute type ITP, the male to female ration is almost equal. The mortality rate for ITP in from hemorrhage is predicted to be 1% in children and 5% in adults.
According to British Society for Hematology guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and pregnancy (2003), platelet counts exceeding 30 x 109 / L require no treatment unless they are undergoing a procedure.
First line therapy should include oral corticosteroids and intravenous immunoglobulins (IVIg). Splenectomy should be considered as second line therapy and not as the first line therapy. In refractory chronic ITP, treatment options includes high dose steroids, high dose IVIg, intravenous anti-D, vinca alkaloids, danazol, immunosuppressives like azathioprine and cyclophosphamide, combination chemotherapy and dapsone. Failed cases of above mentioned cases should be administered interferon-α, anti-CD 20, campath 1H, mycophenolate motefil, protein A columns, chlorodeoxyadenosine, colchicine, liposomal doxorubicin, peripheral blood stem cell transplantation and plasmapheresis.
George, J., & Arnold, D. (2015, March 13). Immune Thrombocytopenia (ITP) in Adults. Retrieved April 17, 2015, from http://www.uptodate.com/contents/immune-thrombocytopenia-itp-in-adults-clinical-manifestations-and-diagnosis?source=search_result&search=idiopathic thrombocytopenia&selectedTitle=1~150
Guidelines for the investigation and management of idiopathic thrombocytopenic purpura in adults, children and in pregnancy (2003), British Journal of Haematology, 120: 574–596. doi: 10.1046/j.1365-2141.2003.04131.x
Mohan, H. (2010). CHAPTER 13; Disorders of Platelets, Bleeding Disorders and Basic Transfusion Medicine. In Textbook of Pathology (6th ed., p. 332). New Delhi: Jaypee Brothers Medical (PVT).
Papadakis, M., & McPhee, S. (2015). Chapter 14; Disorders of Hemostasis, Thrombosis. In Current Medical Diagnosis & Treatment; 2015 (54th ed., p. 538). New York: McGraw-Hill Education/Medical.
Silverman, M., & Dyne, P. (2015). Idiopathic Thrombocytopenic Purpura. In Medscape. WebMD Health Professional Network.
Stasi, R. and Newland, A. C. (2011), ITP: a historical perspective. British Journal of Haematology, 153: 437–450. doi: 10.1111/j.1365-2141.2010.08562.x
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