{"id":5287,"date":"2021-05-14T20:16:38","date_gmt":"2021-05-14T18:16:38","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/neutropenia-agranulocytosis\/"},"modified":"2022-01-17T13:08:15","modified_gmt":"2022-01-17T11:08:15","slug":"neutropenia-agranulocytosis","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/neutropenia-agranulocytosis\/","title":{"rendered":"Neutropenia (agranulocytosis)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\">Neutropenia is defined as an abnormally low neutrophil count, below 1.5G\/L.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Divided into <strong>mild <\/strong>(1.0-1.5G\/L), <strong>moderate <\/strong>(0.5-1.0G\/L) and <strong>severe <\/strong>(&lt;0.5G\/L).<\/li><li>More common in females.<\/li><li>Although they are used interchangeably, <strong>agranulocytosis <\/strong>refers to a more severe condition in which all granulocytes (including eosinophils and basophils) are severely reduced.<\/li><li>Leukopenia refers to a decrease in all white blood cell counts.<\/li><li>The lower the value, the higher the chances are that the patient will develop recurrent infections.<\/li><li>It can come by itself or as a part of pancytopenia (where all blood cell counts are decreased) in cases such as HIV.<\/li><\/ul>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Risk factors<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>Older age<\/li><li>HLA-B38 and DR4<\/li><li>EBV<\/li><li>Drug usage<ul><li>Probenecid together with captopril<\/li><li>ACEi together with interferons<\/li><li>Levamisole<\/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\">Neutropenia can be primary (idiopathic or congenital) or acquired (drug-induced, immune, tumor-induced, infectious).<\/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 neutropenia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Chronic idiopathic neutropenia is a diagnosis of exclusion in case of prolonged unexplained neutropenia.<\/li><li>More common in females.<\/li><li>In some populations (West Africa), polymorphisms in the Duffy antigen chemokine receptor gene lead to the loss of its expression on the surface of neutrophils, causing substantially reduced neutrophil counts.<\/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 title2\">Congenital neutropenia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\"><li>Severe congenital neutropenia (Kostmann&#8217;s syndrome)<ul><li>AD mutations in ELA2 gene coding for neutrophil elastase.<\/li><li>Typically presents early on with severe, recurrent infections.<\/li><\/ul><\/li><li>Cyclic neutropenia<ul><li>3-4 week cycles of temporary neutropenia with elevated monocyte count.<\/li><li>Associated with ELA2 defects.<\/li><\/ul><\/li><li>Other congenital disorders can present with neutropenia, such as:<ul><li>Schwanmann-Diamond syndrome<\/li><li>Wiskott-Aldrich syndrome<\/li><li>Chediak-Higashi syndrome<\/li><\/ul><\/li><li>Typically treated with G-CSF<\/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\">Drug-induced neutropenia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<p class=\"wp-block-paragraph\">A variety of drugs can cause neutropenia, typically as an idiosyncratic reaction:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Mitamizole (analgesic)<\/li><li>Chloramphenicol and trimethoprim-sulfamethoxazole (antibiotics)<\/li><li>Phenytoin and carbamazepine (AEDs)<\/li><li>Clozapine<\/li><li>Rituximab<\/li><li>Gold<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_6\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Immune-associated neutropenia<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_6\">\n\n\n<ul class=\"wp-block-list\"><li>Autoimmune (antibodies produced against neutrophils)<\/li><li>SLE-induced<\/li><li>Felty&#8217;s syndrome (RA + splenomegaly + neutropenia)<\/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\">Clinical presentation<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_7\">\n\n\n<p class=\"wp-block-paragraph\">If the neutropenia is a part of pancytopenia, the clinical presentations of the other -penias are present (anemia and thrombocytopenia). Neutropenia-specific presentations include:<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Chronic, recurrent infections (typically as ulcers in the throat and mouth) appearing with fever (<strong>febrile neutropenia<\/strong>) or without<\/li><li>Septicemia<\/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\">Diagnosis<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_8\">\n\n\n<ul class=\"wp-block-list\"><li>Blood tests<\/li><li>Serological tests (ANAs)<\/li><li>Bone marrow examination<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_9\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Treatment<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_9\">\n\n\n<ul class=\"wp-block-list\"><li>For severe, acute neutropenia:<ul><li>Neutropenic diet (avoid raw milk, yogurt, fresh fruits, and vegetables, B12 supplementation)<\/li><li>Improved hygiene, use chlorhexidine mouth-wash<\/li><li>In some cases, patients are put in isolation.<\/li><\/ul><\/li><li>G-CSF<\/li><li>Corticoids or splenomegaly in autoimmune neutropenia<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_10\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Febrile neutropenia<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_10\">\n\n\n<p class=\"wp-block-paragraph\">Febrile neutropenia is defined as fever (&gt;38c) along with a neutrophil count of less than 1.5G\/L.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li>Most commonly with a background of chemotherapy and hematological cancers.<ul><li>The mucosal barriers of the patient become damaged, increasing the chance of infection.<\/li><\/ul><\/li><li>In most cases, the infectious agent cannot be identified, and the fever is classified as FUO<ul><li>Staphylococci and streptococci are the causuative agents in most cases that are identifiable<\/li><\/ul><\/li><li><strong>MASCC <\/strong>and <strong>CISNE <\/strong>are scoring systems used to help identify high-risk patients requiring hospitalization.<\/li><li>Diagnosis<ul><li>Blood culture<\/li><li>Urinalysis<\/li><li>Throat culture<\/li><li>Stool sample (if diarrhea is present)<\/li><\/ul><\/li><li>Treatment<ul><li>Emperic treatment using fluoroquinolone together with augmentin (amoxicillin+clavulanic acid)<\/li><li>In high-risk patients use cefepime or carbapenem as anti-pseudomonal treatment<\/li><li>In case of lack of response, consider vancomycin (MRSA), linezolid (VRSA), fluconazole (fungi)<\/li><\/ul><\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Risk factors<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Etiology<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Idiopathic neutropenia<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Congenital neutropenia<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Drug-induced neutropenia<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Immune-associated neutropenia<\/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><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Febrile neutropenia<\/h3><\/div>","protected":false},"excerpt":{"rendered":"<p>Neutropenia is defined as an abnormally low neutrophil count, below 1.5G\/L. Divided into mild (1.0-1.5G\/L), moderate (0.5-1.0G\/L) and severe (&lt;0.5G\/L). More common in females. Although they are used interchangeably, agranulocytosis refers to a more severe condition in which all granulocytes (including eosinophils and basophils) are severely reduced. Leukopenia refers to a decrease in all white [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":4472,"menu_order":3,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5287","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>Neutropenia (agranulocytosis) &#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\/neutropenia-agranulocytosis\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"3 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\\\/neutropenia-agranulocytosis\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/neutropenia-agranulocytosis\\\/\",\"name\":\"Neutropenia (agranulocytosis) &#8211; 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