{"id":5611,"date":"2021-05-21T01:56:00","date_gmt":"2021-05-20T23:56:00","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/thrombocytopenia-and-platelet-disorders\/platelet-disorders\/"},"modified":"2021-05-31T15:56:53","modified_gmt":"2021-05-31T13:56:53","slug":"platelet-disorders","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/thrombocytopenia-and-platelet-disorders\/platelet-disorders\/","title":{"rendered":"Platelet disorders"},"content":{"rendered":"<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Etiology<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>Primary (acquired)<ul><li><strong>Defects of platelet adhesion<\/strong><ul><li><em><strong>Bernard\u2013Soulier syndrome: <\/strong><\/em>An autosomal recessive disorder caused by mutations in the <strong>GPIb gene<\/strong>. <strong>The platelets are larger than normal.<\/strong> There is defective binding to vWF and exposed subendothelial connective tissues leading to failed aggregation.<\/li><\/ul><\/li><li><strong>Defects of platelet aggregation<\/strong><ul><li><strong>Thrombasthenia (Glanzmann\u2019s disease)<\/strong>: An autosomal recessive disorder in which <strong>GPIIb\/IIIa<\/strong> receptors are defective, leading to failure of primary platelet aggregation.<\/li><\/ul><\/li><\/ul><ul><li><strong>Storage pool diseases (defects of platelet secretion)<\/strong><ul><li><strong>Alpha granule deficiency: <\/strong>A mutation in a <strong>growth factor independent gene (<em>GFI1B<\/em>)<\/strong> leads to the deficiency of <strong><u>alpha granules<\/u><\/strong>, a condition known as  <strong><u><em>\u201c<\/em>grey platelet disease<em>\u201d<\/em><\/u><\/strong>. Alpha granules contain Factor V, VWF, and fibrinogen.<\/li><li><strong>Dense granule deficiency<\/strong><ul><li>isolated dense granule deficiency<\/li><\/ul><ul><li>Hermansky-Pudlak sy.<\/li><li>Chediak-Higashi sy.<\/li><li>Wiskott-Aldrich sy.<\/li><\/ul><\/li><\/ul><\/li><li><strong>&#8220;Aspirin-like&#8221; diseases<\/strong><ul><li>COX deficiency<\/li><li>TX synthase deficiency<\/li><li>TXA2 receptor deficiency<\/li><\/ul><\/li><\/ul><\/li><li>Secondary<ul><li><strong>Antiplatelet drugs<\/strong><ul><li><strong>Aspirin: <\/strong>most common. Inhibition of cyclo\u2010oxygenase with impaired thromboxane A2 synthesis.<\/li><li><strong>Dipyridamole: <\/strong>inhibits platelet aggregation by blocking the reuptake of adenosine and is usually used as an adjunct to aspirin.<\/li><li><strong>Clopidogrel and prasugrel <\/strong>inhibit ADP receptors.<\/li><li>Antidepressants and antibiotics<\/li><\/ul><\/li><li><strong>Malignancies<\/strong> and other diseases<ul><li>Platelet functions can be defective in diseases such as polycythemia vera, paroxysmal nocturnal hemoglobinuria.<\/li><\/ul><\/li><\/ul><ul><li><strong>Hyperglobulinaemia<\/strong> (paraprotein): associated with multiple myeloma may cause interference with platelet adherence, release aggregation.<\/li><li><strong>Uremia<\/strong>, liver cirrhosis,<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Patients show skin and mucosal haemorrhage despite a normal platelet count and normal levels of vWF.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>CBC<\/li><li>Bleeding time, PFA-100<\/li><li>Platelet aggregation \/secretion<\/li><li>Peripheral smear<\/li><li>Bone marrow test<\/li><li>Renal\/ liver function tests<\/li><li>Serum electrophoresis<\/li><li>Flow cytometry<\/li><li>Mepacrin dye<\/li><li>Electron microscopic evaluation<\/li><\/ul>\n\n\n\n<figure class=\"wp-block-image size-large\"><img loading=\"lazy\" decoding=\"async\" width=\"480\" height=\"289\" src=\"https:\/\/meddists.com\/learn\/wp-content\/uploads\/2021\/05\/image-2.png\" alt=\"\" class=\"wp-image-5761\" srcset=\"https:\/\/meddists.com\/learn\/wp-content\/uploads\/2021\/05\/image-2.png 480w, https:\/\/meddists.com\/learn\/wp-content\/uploads\/2021\/05\/image-2-300x181.png 300w\" sizes=\"auto, (max-width: 480px) 100vw, 480px\" \/><\/figure>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Prevention: avoid aspirin and NSAID<\/li><li>DDAVP (Octostim)<\/li><li>Platelet transfusion<\/li><li>rFVIIa<\/li><li>Fibrinolysis inhibitors<\/li><li>Oral contraceptives<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Etiology<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical presentation<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Etiology Primary (acquired) Defects of platelet adhesion Bernard\u2013Soulier syndrome: An autosomal recessive disorder caused by mutations in the GPIb gene. The platelets are larger than normal. There is defective binding to vWF and exposed subendothelial connective tissues leading to failed aggregation. Defects of platelet aggregation Thrombasthenia (Glanzmann\u2019s disease): An autosomal recessive disorder in which GPIIb\/IIIa [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5575,"menu_order":1,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5611","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Platelet disorders &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/thrombocytopenia-and-platelet-disorders\/platelet-disorders\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/thrombocytopenia-and-platelet-disorders\\\/platelet-disorders\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/thrombocytopenia-and-platelet-disorders\\\/platelet-disorders\\\/\",\"name\":\"Platelet disorders &#8211; 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