Pathology notes

Apr 16th, 2015
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HEMOLYTIC ANEMIASintro; These are anemia that are associated withaccelerated destruction of RBCs. Destruction may be due tointrinsic(intracorpurscular)defects orextrinsic[extracorpurcular]defects. Characterized by;-1.^rbc destruction2.erythrocytosis3.iron retention Can be ;intravascular or extra vascular Leads to hemoglobinemia, hemoglobunuria,hemosiderinuria, hyperbilirubinemia-jaundice Extra vascular hemolysis[most common]occurs within phagocytes of the spleen andliver. Not associated with hemoglobinemia orhemoglobunuria. In chronic hemolytic anemia, change in ironmetabolism leads to inc iron absorption fromthe gut therefore causing systemichemosiderosisExamples; Hereditary spherocytosis An autosomal dominant disorder caused byinherited mutations that affect rbc membraneskeletal leading to loss of membrane andeventual conversion of rbc to spherocytes,which are phagocytosed and removed byspleen. the cell is les deformable hence vulnerable tosplenic sequestration.Pathogenesis; HS mutations weakens the vertical interactionbetween membrane skeletal and the intrinsicmembrane proteins i.e. ankyrin,band 3 andspectrin. RBC acquire weakened membrane stabilityand decreased SA;VOL ratio. Due to their deformability they aresequestrated in spleen cords and destroyed.Morphology; On smear, RBC lack the central zone of pallor. There are peripheral blood reticulocytes Splenomegaly is comm

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