{"id":2945,"date":"2010-03-18T01:36:46","date_gmt":"2010-03-18T05:36:46","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=2945"},"modified":"2013-08-02T00:13:04","modified_gmt":"2013-08-02T04:13:04","slug":"elliptocytosis-hereditary","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/","title":{"rendered":"Elliptocytosis (Hereditary)"},"content":{"rendered":"

Pathophysiology<\/p>\n

1) family of six separate but related syndromes – common elliptocytosis, pyropoikilocytosis, spherocytic hereditary elliptocytosis, stomatocytic elliptocytosis (rare) and South Asian ovalocytosis (rare) 2) all are caused by defects in dimer interactions of the cytoskeleton protein spectrin, which causes fragmentation and disruption of the cytoskeletal lattice 3) acquired elliptocytosis can occur in myelodysplastic disorders<\/p>\n

Signs and Symptoms<\/p>\n

1) can range from being clinically silent and discovered only on exam of peripheral blood smear to a severe hemolytic anemia 2) any of the variants that are homozygous and hereditary pyropoikilocytosis have moderate to severe anemia 3) splenomegaly<\/p>\n

Characteristic Test Findings<\/p>\n

Laboratory – 1) mild to severe anemia 2) decreased haptoglobin levels 3) increased reticulocyte count 4) increased osmotic fragility test<\/p>\n

Histology\/Gross Pathology<\/p>\n

\"elliptocytosis\"<\/p>\n

1) common elliptocytosis – at least 15% of RBCs are elliptical on peripheral blood smear 2) pyropoikilocytosis – has red cell fragments, poikilocytes, or microspherocytes 3) spherocytic hereditary elliptocytosis – has ovalocytes and spherocytes<\/p>\n

Associated Conditions<\/p>\n

1) protein 4.1 defects and glycophorin C deficiency 2) some resistance to malaria occurs 3) renal tubular acidosis<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology 1) family of six separate but related syndromes – common elliptocytosis, pyropoikilocytosis, spherocytic hereditary elliptocytosis, stomatocytic elliptocytosis (rare) and South Asian ovalocytosis (rare) 2) all are caused by defects in dimer interactions of the cytoskeleton protein spectrin, which causes fragmentation and disruption of the cytoskeletal lattice 3) acquired elliptocytosis can occur in myelodysplastic disorders […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[5156],"tags":[1443,1445,1441,1444,1442,491,490],"yoast_head":"\nElliptocytosis (Hereditary) - InsideSurgery Medical Information Blog<\/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:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Elliptocytosis (Hereditary) - InsideSurgery Medical Information Blog\" \/>\n<meta property=\"og:description\" content=\"Pathophysiology 1) family of six separate but related syndromes – common elliptocytosis, pyropoikilocytosis, spherocytic hereditary elliptocytosis, stomatocytic elliptocytosis (rare) and South Asian ovalocytosis (rare) 2) all are caused by defects in dimer interactions of the cytoskeleton protein spectrin, which causes fragmentation and disruption of the cytoskeletal lattice 3) acquired elliptocytosis can occur in myelodysplastic disorders […]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/\" \/>\n<meta property=\"og:site_name\" content=\"InsideSurgery Medical Information Blog\" \/>\n<meta property=\"article:published_time\" content=\"2010-03-18T05:36:46+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2013-08-02T04:13:04+00:00\" \/>\n<meta property=\"og:image\" content=\"http:\/\/insidesurgery.com\/wp-content\/uploads\/2010\/03\/elliptocytosis.jpg\" \/>\n<meta name=\"author\" content=\"Editor\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"Editor\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/\",\"url\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/\",\"name\":\"Elliptocytosis (Hereditary) - InsideSurgery Medical Information Blog\",\"isPartOf\":{\"@id\":\"https:\/\/insidesurgery.com\/#website\"},\"primaryImageOfPage\":{\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage\"},\"image\":{\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage\"},\"thumbnailUrl\":\"http:\/\/insidesurgery.com\/wp-content\/uploads\/2010\/03\/elliptocytosis.jpg\",\"datePublished\":\"2010-03-18T05:36:46+00:00\",\"dateModified\":\"2013-08-02T04:13:04+00:00\",\"author\":{\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\"},\"breadcrumb\":{\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#breadcrumb\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/\"]}]},{\"@type\":\"ImageObject\",\"inLanguage\":\"en-US\",\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage\",\"url\":\"\",\"contentUrl\":\"\"},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/insidesurgery.com\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Elliptocytosis (Hereditary)\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/insidesurgery.com\/#website\",\"url\":\"https:\/\/insidesurgery.com\/\",\"name\":\"InsideSurgery Medical Information Blog\",\"description\":\"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news\",\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/insidesurgery.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"en-US\"},{\"@type\":\"Person\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\",\"name\":\"Editor\",\"image\":{\"@type\":\"ImageObject\",\"inLanguage\":\"en-US\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/\",\"url\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"contentUrl\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"caption\":\"Editor\"}}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Elliptocytosis (Hereditary) - InsideSurgery Medical Information Blog","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/","og_locale":"en_US","og_type":"article","og_title":"Elliptocytosis (Hereditary) - InsideSurgery Medical Information Blog","og_description":"Pathophysiology 1) family of six separate but related syndromes – common elliptocytosis, pyropoikilocytosis, spherocytic hereditary elliptocytosis, stomatocytic elliptocytosis (rare) and South Asian ovalocytosis (rare) 2) all are caused by defects in dimer interactions of the cytoskeleton protein spectrin, which causes fragmentation and disruption of the cytoskeletal lattice 3) acquired elliptocytosis can occur in myelodysplastic disorders […]","og_url":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/","og_site_name":"InsideSurgery Medical Information Blog","article_published_time":"2010-03-18T05:36:46+00:00","article_modified_time":"2013-08-02T04:13:04+00:00","og_image":[{"url":"http:\/\/insidesurgery.com\/wp-content\/uploads\/2010\/03\/elliptocytosis.jpg"}],"author":"Editor","twitter_misc":{"Written by":"Editor","Est. reading time":"1 minute"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/","url":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/","name":"Elliptocytosis (Hereditary) - InsideSurgery Medical Information Blog","isPartOf":{"@id":"https:\/\/insidesurgery.com\/#website"},"primaryImageOfPage":{"@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage"},"image":{"@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage"},"thumbnailUrl":"http:\/\/insidesurgery.com\/wp-content\/uploads\/2010\/03\/elliptocytosis.jpg","datePublished":"2010-03-18T05:36:46+00:00","dateModified":"2013-08-02T04:13:04+00:00","author":{"@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594"},"breadcrumb":{"@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#breadcrumb"},"inLanguage":"en-US","potentialAction":[{"@type":"ReadAction","target":["https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/"]}]},{"@type":"ImageObject","inLanguage":"en-US","@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#primaryimage","url":"","contentUrl":""},{"@type":"BreadcrumbList","@id":"https:\/\/insidesurgery.com\/2010\/03\/elliptocytosis-hereditary\/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https:\/\/insidesurgery.com\/"},{"@type":"ListItem","position":2,"name":"Elliptocytosis (Hereditary)"}]},{"@type":"WebSite","@id":"https:\/\/insidesurgery.com\/#website","url":"https:\/\/insidesurgery.com\/","name":"InsideSurgery Medical Information Blog","description":"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news","potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/insidesurgery.com\/?s={search_term_string}"},"query-input":"required name=search_term_string"}],"inLanguage":"en-US"},{"@type":"Person","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594","name":"Editor","image":{"@type":"ImageObject","inLanguage":"en-US","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/","url":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","contentUrl":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","caption":"Editor"}}]}},"_links":{"self":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/2945"}],"collection":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/comments?post=2945"}],"version-history":[{"count":0,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/2945\/revisions"}],"wp:attachment":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/media?parent=2945"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/categories?post=2945"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/tags?post=2945"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}