Inherited hemolytic anemia: a possessive beginner's guide
Significant advances have been made in diagnosis and clinical management of inherited red cell membrane disorders that result in hemolytic anemia. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function account...
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| Veröffentlicht in: | Hematology 2018-11, Vol.2018 (1), p.377-381 |
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| description | Significant advances have been made in diagnosis and clinical management of inherited red cell membrane disorders that result in hemolytic anemia. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function accounts for hereditary xerocytosis (HX) and hereditary overhydrated stomatocytosis (OHS). The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE, and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Alterations in cell volume as a result of disordered membrane cation permeability account for reduced life span red cells in HX and OHS. Importantly, splenectomy is not beneficial in these 2 membrane transport disorders and is not recommended because it is ineffective and may lead to an increased risk of life-threatening thrombosis. Rational approaches are now available for the diagnosis and management of these inherited red cell disorders, and these will be discussed in this review. |
| doi_str_mv | 10.1182/asheducation-2018.1.377 |
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Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function accounts for hereditary xerocytosis (HX) and hereditary overhydrated stomatocytosis (OHS). The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE, and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Alterations in cell volume as a result of disordered membrane cation permeability account for reduced life span red cells in HX and OHS. Importantly, splenectomy is not beneficial in these 2 membrane transport disorders and is not recommended because it is ineffective and may lead to an increased risk of life-threatening thrombosis. Rational approaches are now available for the diagnosis and management of these inherited red cell disorders, and these will be discussed in this review.</description><identifier>ISSN: 1520-4391</identifier><identifier>EISSN: 1520-4383</identifier><identifier>DOI: 10.1182/asheducation-2018.1.377</identifier><identifier>PMID: 30504335</identifier><language>eng</language><publisher>United States: American Society of Hematology</publisher><subject>Anemia, Hemolytic, Congenital - genetics ; Anemia, Hemolytic, Congenital - metabolism ; Anemia, Hemolytic, Congenital - pathology ; Anemia, Hemolytic, Congenital - therapy ; Elliptocytosis, Hereditary - genetics ; Elliptocytosis, Hereditary - metabolism ; Elliptocytosis, Hereditary - pathology ; Elliptocytosis, Hereditary - therapy ; Erythrocyte Membrane - genetics ; Erythrocyte Membrane - metabolism ; Erythrocyte Membrane - pathology ; Hemolytic Anemia: A Cornucopia of Causes ; Humans ; Hydrops Fetalis - genetics ; Hydrops Fetalis - metabolism ; Hydrops Fetalis - pathology ; Hydrops Fetalis - therapy ; Risk Factors ; Spherocytosis, Hereditary - genetics ; Spherocytosis, Hereditary - metabolism ; Spherocytosis, Hereditary - pathology ; Spherocytosis, Hereditary - therapy ; Thrombosis - genetics ; Thrombosis - metabolism ; Thrombosis - pathology ; Thrombosis - therapy</subject><ispartof>Hematology, 2018-11, Vol.2018 (1), p.377-381</ispartof><rights>2018 by The American Society of Hematology. All rights reserved.</rights><rights>2018 by The American Society of Hematology. All rights reserved. 2018 American Society of Hematology</rights><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c483t-db168d000e05d9c0b583dd1b0e65a03fc756443cf962113a188b6aa78016ccf43</citedby><cites>FETCH-LOGICAL-c483t-db168d000e05d9c0b583dd1b0e65a03fc756443cf962113a188b6aa78016ccf43</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245988/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245988/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,727,780,784,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30504335$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mohandas, Narla</creatorcontrib><title>Inherited hemolytic anemia: a possessive beginner's guide</title><title>Hematology</title><addtitle>Hematology Am Soc Hematol Educ Program</addtitle><description>Significant advances have been made in diagnosis and clinical management of inherited red cell membrane disorders that result in hemolytic anemia. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function accounts for hereditary xerocytosis (HX) and hereditary overhydrated stomatocytosis (OHS). The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE, and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Alterations in cell volume as a result of disordered membrane cation permeability account for reduced life span red cells in HX and OHS. Importantly, splenectomy is not beneficial in these 2 membrane transport disorders and is not recommended because it is ineffective and may lead to an increased risk of life-threatening thrombosis. Rational approaches are now available for the diagnosis and management of these inherited red cell disorders, and these will be discussed in this review.</description><subject>Anemia, Hemolytic, Congenital - genetics</subject><subject>Anemia, Hemolytic, Congenital - metabolism</subject><subject>Anemia, Hemolytic, Congenital - pathology</subject><subject>Anemia, Hemolytic, Congenital - therapy</subject><subject>Elliptocytosis, Hereditary - genetics</subject><subject>Elliptocytosis, Hereditary - metabolism</subject><subject>Elliptocytosis, Hereditary - pathology</subject><subject>Elliptocytosis, Hereditary - therapy</subject><subject>Erythrocyte Membrane - genetics</subject><subject>Erythrocyte Membrane - metabolism</subject><subject>Erythrocyte Membrane - pathology</subject><subject>Hemolytic Anemia: A Cornucopia of Causes</subject><subject>Humans</subject><subject>Hydrops Fetalis - genetics</subject><subject>Hydrops Fetalis - metabolism</subject><subject>Hydrops Fetalis - pathology</subject><subject>Hydrops Fetalis - therapy</subject><subject>Risk Factors</subject><subject>Spherocytosis, Hereditary - genetics</subject><subject>Spherocytosis, Hereditary - metabolism</subject><subject>Spherocytosis, Hereditary - pathology</subject><subject>Spherocytosis, Hereditary - therapy</subject><subject>Thrombosis - genetics</subject><subject>Thrombosis - metabolism</subject><subject>Thrombosis - pathology</subject><subject>Thrombosis - therapy</subject><issn>1520-4391</issn><issn>1520-4383</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpVkE1PwzAMhiMEYmPwF6A3uHTYSdqmHJAQ4mPSJC5wjtLE3YL6MZp20v49RcAEJ1uy39d-H8YuEOaIil-bsCY3WNP7tok5oJrjXGTZAZtiwiGWQonDfZ_jhJ2E8A6AUnB-zCYCEpBCJFOWL5o1db4nF62pbqtd721kGqq9uYlMtGlDoBD8lqKCVr5pqLsM0Wrwjk7ZUWmqQGc_dcbeHh9e75_j5cvT4v5uGVupRB-7AlPlAIAgcbmFIlHCOSyA0sSAKG2WpFIKW-YpRxQGlSpSYzIFmFpbSjFjt9--m6GoyVlq-s5UetP52nQ73Rqv_08av9ardqtTLpNcqdHg6segaz8GCr2ufbBUVWPMdgiao8wBheQ4rmbfq7Ybg3dU7s8g6C_w-i94_QVeox7Bj8rzv1_udb-kxSdjJ4OV</recordid><startdate>20181130</startdate><enddate>20181130</enddate><creator>Mohandas, Narla</creator><general>American Society of Hematology</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20181130</creationdate><title>Inherited hemolytic anemia: a possessive beginner's guide</title><author>Mohandas, Narla</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c483t-db168d000e05d9c0b583dd1b0e65a03fc756443cf962113a188b6aa78016ccf43</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Anemia, Hemolytic, Congenital - genetics</topic><topic>Anemia, Hemolytic, Congenital - metabolism</topic><topic>Anemia, Hemolytic, Congenital - pathology</topic><topic>Anemia, Hemolytic, Congenital - therapy</topic><topic>Elliptocytosis, Hereditary - genetics</topic><topic>Elliptocytosis, Hereditary - metabolism</topic><topic>Elliptocytosis, Hereditary - pathology</topic><topic>Elliptocytosis, Hereditary - therapy</topic><topic>Erythrocyte Membrane - genetics</topic><topic>Erythrocyte Membrane - metabolism</topic><topic>Erythrocyte Membrane - pathology</topic><topic>Hemolytic Anemia: A Cornucopia of Causes</topic><topic>Humans</topic><topic>Hydrops Fetalis - genetics</topic><topic>Hydrops Fetalis - metabolism</topic><topic>Hydrops Fetalis - pathology</topic><topic>Hydrops Fetalis - therapy</topic><topic>Risk Factors</topic><topic>Spherocytosis, Hereditary - genetics</topic><topic>Spherocytosis, Hereditary - metabolism</topic><topic>Spherocytosis, Hereditary - pathology</topic><topic>Spherocytosis, Hereditary - therapy</topic><topic>Thrombosis - genetics</topic><topic>Thrombosis - metabolism</topic><topic>Thrombosis - pathology</topic><topic>Thrombosis - therapy</topic><toplevel>online_resources</toplevel><creatorcontrib>Mohandas, Narla</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mohandas, Narla</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Inherited hemolytic anemia: a possessive beginner's guide</atitle><jtitle>Hematology</jtitle><addtitle>Hematology Am Soc Hematol Educ Program</addtitle><date>2018-11-30</date><risdate>2018</risdate><volume>2018</volume><issue>1</issue><spage>377</spage><epage>381</epage><pages>377-381</pages><issn>1520-4391</issn><eissn>1520-4383</eissn><abstract>Significant advances have been made in diagnosis and clinical management of inherited red cell membrane disorders that result in hemolytic anemia. Membrane structural defects lead to hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), whereas altered membrane transport function accounts for hereditary xerocytosis (HX) and hereditary overhydrated stomatocytosis (OHS). The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in HS and HE, and splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span. Alterations in cell volume as a result of disordered membrane cation permeability account for reduced life span red cells in HX and OHS. Importantly, splenectomy is not beneficial in these 2 membrane transport disorders and is not recommended because it is ineffective and may lead to an increased risk of life-threatening thrombosis. Rational approaches are now available for the diagnosis and management of these inherited red cell disorders, and these will be discussed in this review.</abstract><cop>United States</cop><pub>American Society of Hematology</pub><pmid>30504335</pmid><doi>10.1182/asheducation-2018.1.377</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Anemia, Hemolytic, Congenital - genetics Anemia, Hemolytic, Congenital - metabolism Anemia, Hemolytic, Congenital - pathology Anemia, Hemolytic, Congenital - therapy Elliptocytosis, Hereditary - genetics Elliptocytosis, Hereditary - metabolism Elliptocytosis, Hereditary - pathology Elliptocytosis, Hereditary - therapy Erythrocyte Membrane - genetics Erythrocyte Membrane - metabolism Erythrocyte Membrane - pathology Hemolytic Anemia: A Cornucopia of Causes Humans Hydrops Fetalis - genetics Hydrops Fetalis - metabolism Hydrops Fetalis - pathology Hydrops Fetalis - therapy Risk Factors Spherocytosis, Hereditary - genetics Spherocytosis, Hereditary - metabolism Spherocytosis, Hereditary - pathology Spherocytosis, Hereditary - therapy Thrombosis - genetics Thrombosis - metabolism Thrombosis - pathology Thrombosis - therapy |
| title | Inherited hemolytic anemia: a possessive beginner's guide |
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