{"id":5272,"date":"2021-05-13T19:35:38","date_gmt":"2021-05-13T17:35:38","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/anemias\/hemolytic-anemias\/aplastic-anemia\/"},"modified":"2021-05-24T17:22:26","modified_gmt":"2021-05-24T15:22:26","slug":"aplastic-anemia","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/anemias\/aplastic-anemia\/","title":{"rendered":"Aplastic anemia"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Aplastic anemia is anemia caused by bone marrow failure. It is characterized by bone marrow hypoplasia and pancytopenia.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Typically appears in young (20) or older adults (>60).<\/li><\/ul>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Classification<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>Severe (SAA)<ul><li>Absolute reticulocyte count &lt;50G\/L<\/li><li>Platelet count &lt;20G\/L<\/li><li>Absolute neutrophil count &lt;0.5G\/L<\/li><\/ul><\/li><li>Very severe (vSAA)<ul><li>Severe + absolute neutrophil count &lt;0.2G\/L<\/li><\/ul><\/li><li>Moderate<ul><li>Values above severe (not fulfilling the criteria)<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Etiology<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Aplastic anemia can be primary (idiopathic or congenital) or secondary.<\/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 title2\">Idiopathic aplastic anemia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<p class=\"wp-block-paragraph\">Most common type (up to 2\/3 of cases). Autoimmune processes are typically evident (CD8+ T-cells), and is most often treated by cyclosporin and antilymphocyte globulin.<\/p>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Congenital &#8211; Fanconi anemia (FA)<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<p class=\"wp-block-paragraph\">An autosomal recessive disorder associated with growth retardation, CNS, renal, skeletal, and skin abnormalities. <\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>It is associated with defects in genes coding for proteins that protect cells against genetic damage. Patients with FA show numerous chromosomal breakage due to such defects.<\/li><li>It typically presents around early childhood, with some patients developing AML.<\/li><li>Treatment is androgen therapy and stem cell transplantation.<\/li><\/ul>\n\n\n\n<p class=\"wp-block-paragraph\">Other congenital causes include conditions with ribosomal biosynthesis defects such as:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Dyskeratosis congenita<\/li><li>Diamond\u2013Blackfan anemia<\/li><li>Schwachman-Diamond syndrome<\/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 title2\">Secondary aplastic anemia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">Can be caused by a variety of noxia that damage the hemopoietic cells of the bone marrow:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Chemotherapy (methotrexate and alkylating agents)<\/li><li>Drugs (chloramphenicol)<\/li><li>Chemicals (benzene)<\/li><li>Viral hepatitis<\/li><li>Irradiation<\/li><li>Viral infections (EBV)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Clinical presentation<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<ul class=\"wp-block-list\"><li>Easy bruising and bleeding gums<\/li><li>Menorrhagia<\/li><li>Increased susceptibility to infections<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_7\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Diagnosis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<ul class=\"wp-block-list\"><li>Total blood count (showing severe anemia, leukopenia, and thrombocytopenia)<\/li><li>Reticulocyte count (extremely low)<\/li><li>Blood smear (showing no cellular abnormality)<\/li><li>Flow cytometry (for CD55 and CD59 to exclude proxysmal nocturnal hemoglobinuria)<\/li><li>Bone marrow biopsy (showing hypocellularity)<\/li><li>Cytogenetic and molecular analysis (to detect congenital aplastic anemia and exclude myelodysplasia)<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_8\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_8\">\n\n\n<p class=\"wp-block-paragraph\">If the causative agent is known, it must be removed. To treat acutely severe cases of aplastic anemia, blood transfusion low in leukocytes (to prevent alloimmunity) is given along with iron chelators, antibiotics, and antifungal agents.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Long-term therapy must be adjusted to the patient and the severity of the disease:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>In young patients, SCT<\/li><li>Anti-thymocyte globulin (ATG) + cyclosporin + eltrombopag, sometimes known as &#8220;triple IST&#8221;<\/li><li>Alemtuzumab (anti CD52 mAb)<\/li><li>Androgens (in FA)<\/li><li>G-CSF (granulocyte colony-stimulating factor) and eltrombopag<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Classification<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Etiology<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Idiopathic aplastic anemia<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Congenital &#8211; Fanconi anemia (FA)<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Secondary aplastic anemia<\/h4><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical presentation<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h3><\/div>","protected":false},"excerpt":{"rendered":"<p>Aplastic anemia is anemia caused by bone marrow failure. It is characterized by bone marrow hypoplasia and pancytopenia. Typically appears in young (20) or older adults (>60). Classification Severe (SAA) Absolute reticulocyte count &lt;50G\/L Platelet count &lt;20G\/L Absolute neutrophil count &lt;0.5G\/L Very severe (vSAA) Severe + absolute neutrophil count &lt;0.2G\/L Moderate Values above severe (not [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5221,"menu_order":2,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5272","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>Aplastic anemia &#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\/aplastic-anemia\/\" \/>\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\\\/anemias\\\/aplastic-anemia\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/anemias\\\/aplastic-anemia\\\/\",\"name\":\"Aplastic anemia &#8211; 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