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Hemolytic anemia due to red blood cell enzyme and membrane PDF

47 Pages·2014·2.89 MB·English
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Preview Hemolytic anemia due to red blood cell enzyme and membrane

Hemolytic anemia due to red blood cell enzyme and mmeemmbbrraannee ddiissoorrddeerrss BBeellaannggeennvveerrkkllaarriinngg In overeenstemming met de regels van de Inspectie van de Gezondheidszorg (IGZ) Naam: REG Schutgens OOrrggaanniissaattiiee:: UUMMCC UUttrreecchhtt (cid:59) Ik heb geen 'potentiële' belangenverstrengeling Type van verstrengeling / financieel belang Naam van commercieel bedrijf Ontvangst van subsidie(s)/research ondersteuning: Ontvangst van honoraria of adviseursfee: Lid van een commercieel gesponsord ‘speakersbureau’: FFiinnaanncciiëëllee bbeellaannggeenn iinn eeeenn bbeeddrriijjff ((aaaannddeelleenn ooff ooppttiieess)):: Andere ondersteuning (gelieve te specificeren): Wetenschappelijke adviesraad: BBeellaannggeennvveerrkkllaarriinngg In overeenstemming met de regels van de Inspectie van de Gezondheidszorg (IGZ) Naam: HA van Wijk OOrrggaanniissaattiiee:: UUMMCC UUttrreecchhtt (cid:59) Ik heb geen 'potentiële' belangenverstrengeling Type van verstrengeling / financieel belang Naam van commercieel bedrijf Ontvangst van subsidie(s)/research ondersteuning: Ontvangst van honoraria of adviseursfee: Lid van een commercieel gesponsord ‘speakersbureau’: FFiinnaanncciiëëllee bbeellaannggeenn iinn eeeenn bbeeddrriijjff ((aaaannddeelleenn ooff ooppttiieess)):: Andere ondersteuning (gelieve te specificeren): Wetenschappelijke adviesraad: Classification hemolytic anemia Hereditary Acquired Hemoglobinopathy Immune mediated Membrane disorders Micro-angiopathic Enzyme deficiency PNH Extravascular Intravascular Macropphagges DIC Liver/spleen AHTR Intrinsic Extrinsic Hemoglobin structure Antibody Membrane Infection/toxin Metabolism Diagnostic strategy 1. Diagnose or exclude immune mediated hemolysis – Auto-immune hemolytic anemia (AIHA) – AAllllo-iimmune hhemollyttiic anemiia – Drug-induced 22. HHeerreeddiittaarryy oorr aaccqquuiirreedd?? – Family history – TTiimmee ooff oonnsseett – Concommitant symptoms Hereditary hemolytic anemias • Intracorpuscular defect of the red bblloodd cellll •• PPrreemmaattuurree rreemmoovvaall ffrroomm tthhee circulation by the spleen and monocyte-macrophage system • Extravascular hemolysis Hereditary hemolytic anemias General clinical features • Highly variable degree of anemia (palor, fatique) • Presentation at variable age • CChhroniic hhemollysiis vs periioddiic hhemollyttiic episodes • Exacerbation of hemolysis during infection/stress • SSpplleennoommeeggaally • Neonatal jaundice / intermittent jaundice • Gall stones • AApllasttiic criises • Iron overload • Leg ulcers • Failure to thrive • Generally good response to splenectomy Case 1 • Male, 1966 • Admitted 9-1984 with headache, tiredness and abdominal pain. • SSplleen enllargedd andd paiinffull • Laboratory: – HHbb 66.22 mmmmooll//LL – Reticulocytes 280 – Bilirubine 81 ((direkt 16)) – LDH 790 – Haptoglobine 0.2 – Directe antiglobine test (Coombs): negatief • Family history: several members with splenomegaly

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Hereditary hemolytic anemias. • Intracorpuscular defect of the red bl d ll ood cell. • Premature removal from the. • Premature removal from the circulation by the
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