{"id":5259,"date":"2021-05-13T19:06:00","date_gmt":"2021-05-13T17:06:00","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/spherocytosis\/"},"modified":"2021-05-22T21:51:43","modified_gmt":"2021-05-22T19:51:43","slug":"spherocytosis","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/anemias\/hemolytic-anemias\/spherocytosis\/","title":{"rendered":"Spherocytosis"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\"><div class=\"intro\">Hereditary spherocytosis is an autosomal dominant defect of the RBC membrane leading to the formation of extremely vulnerable, defective RBCs known as spherocytes. These cells are prematurely destroyed in the spleen, leading to hemolytic anemia.<\/div><\/p>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Characteristics<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>Most common hereditary hemolytic anemia in Northern Europeans.<\/li><li>Parvovirus B19 can exacerbate the anemia by causing aplastic crisis.<\/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\">Pathophysiology<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">The defect is usually in ankyrin or spectrin, which are structural proteins, leading to weaker interactions between the cytoskeleton and the membrane proteins. <\/p>\n\n\n\n<p class=\"wp-block-paragraph\">This causes the RBCs to shed small parts of their cytoplasm in the form of vesicles and then the cells become spherical. These cells are destroyed in the spleen.<\/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\">Clinical presentation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Fluctuating jaundice<\/li><li>Splenomegaly<\/li><li>Pigment gallstones<\/li><\/ul>\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\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Reticulocyte count (5-20%)<\/li><li>Blood smear (demonstrating small, spherical red blood cells)<\/li><li>Flow cytometry (EMA or eosin-5-maleimide binding test showing decreased count)<\/li><li>Osmotic fragility test (placing the cells in saline causes them to rupture)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">Splenectomy in cases of recurrent jaundice and gallstones (+cholecystectomy).<\/p>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Characteristics<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Pathophysiology<\/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>Characteristics Most common hereditary hemolytic anemia in Northern Europeans. Parvovirus B19 can exacerbate the anemia by causing aplastic crisis. Pathophysiology The defect is usually in ankyrin or spectrin, which are structural proteins, leading to weaker interactions between the cytoskeleton and the membrane proteins. This causes the RBCs to shed small parts of their cytoplasm in [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5264,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5259","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>Spherocytosis &#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\/anemias\/hemolytic-anemias\/spherocytosis\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"1 minute\" \/>\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\\\/anemias\\\/hemolytic-anemias\\\/spherocytosis\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/anemias\\\/hemolytic-anemias\\\/spherocytosis\\\/\",\"name\":\"Spherocytosis &#8211; 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