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Summary of All Anemia

Summary of All Anemia



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Published by benlarsena
summary_of_all anemia
summary_of_all anemia

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Published by: benlarsena on Feb 13, 2009
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Hemolytic Anemias
TypeType ohemolysisMutation/ CausePathogenesisClinical picture and findingsSickle cellanemia
Extravascular hemolysisPoint mutation in beta-globin chain-glureplaced by val- S HbCan be homozygous- all chains havemutation or heterozygous- abt half chains have mutationOn deoxygenation-polymerizaton and reversiblesickling, continued exposure-ireversible – mononuclear phagocytosis & microvascular obstructions.Particularly predisposed with salmonellaosteomyelitisHomozygous: severe anemia, HCT 18-30%,reticulocytosis, hyper-bilirubinemia, crisisHetero: asymptomatic until exposure to severehypoxiaHypospleenism in adults, spleenomegaly inchildren- infections.
intravascular hemolysisα- thalasemia: 4 genes on ch 16deletions- 1- silent carries, 4- hydropsfetalis, 3- diseaseExcessive β- & γ- chains- form stable tetramers(Hb H, Hb bart) low damage, ineffective in O2deliveryβ- thalasemia: 2 genes on ch 11 point mutations on certain regions of gene: in promoter & unsplicing regionof intron- β+ (reduced β-chains)In exon & splicing region of intron- β0(absence of β-chains)Decrease in β-chains – decreased HbMore imp: increase in free α-chains that aggregate,form inclusion in RBC (reduce plasticity of RBC, becomes more susceptible to mononuclear  phagocytosis) destruction also of blasts – ineffective erythropoisis - iron overloadMajor:Microcytic hypochromic + poikilocytosisFailure of normal developmentSkeletal deformities, reticulocytosis.Iron overload – leads to cardiac failure (neediron chelators)Minor Mild microcytic hypochromic Normal life expectancy
Intra- & extra-vascular hemolysisG6PD gene on ch X forms G6PDenzyme that regenerates GSH after itsoxidationMutation causes more rapid decay of enzyme (A
variant)Exposure to drugs, toxins or infections increases H peroxide so increases oxidation of GSH to GSSG,GSH regeneration is impaired so – accum. of H peroxide which denatures Hb. This ppts causingIVhemolysis and also EVhemolysis in spleen No symptoms unless exposure oxidative injury.In males severe oxidant injuryIn females asymptomaticHeinz bodies (precipitated Hb) and bite cells(phagocytosis by splenic phagocytes)
intravascular hemolysisAcquired membrane defect secondaryto a mutation that involves myeloidstem cells. Mutation in X-linked PIGA(synthesis of intramembranousglycoprotein anchor – PIG)PIGA deficient BM cells are present in normalindividuals, when there is immune-mediateddestruction or suppression of BM cells byrecognition of sp. PIG-tailed antigens the PIGAdeficient cells do not express the targets and soescape the attack and replace BMPIG-tailed proteins (3 that prevent activation of complement on normal RBC’s) cannot beexpressed so RBC sensitive to lytic activity of complement, also not expressed on granulocytesand platelets – susceptibility to infections andIVthrombosis
Extravascular hemolysisWarm: IgG or (rarely) IgA active at 37deg. 60% idiopathic and 40 %underlying disease (SLE) or drugs (a-methyl dopa, penicillin)Opsonization by IgG and subsequent phagocytosis by splenic macrophages. Attempts of phagocytosisleads to injured bits of CM – decreased SA:V ratio – spheroidal cells – sequestration+ve direct/ indirect coombs test for bothChronic mild anemia with moderatespleenomegalyCold: IgM binds to CM below 30 deg.(in distal parts)Complement most active at 37 deg. so noIVhemolysis, when cells go to warmer regionsIgM is not well bound but leaves behind C3bopsonin causing phagocytosis by kupffer cells(EV)Acute during recovery from Mycoplasma pneumonia and infectious mononucleosis or chronic resulting in transient mild anemia withoften Raynaud phenomenon
Resulting fromtrauma
Intravascular hemolysisValve prostheses and microangiopathic(caused by DIC, malignant HT, SLE or disseminated cancer)Valves cause abnormal pressure gradient andturbulent blood flow, and RBCs are squeezed thrunarrowed vessels, results in mechanical damage in bothBurr, hemlet and triangle cells
Intravascular hemolysisPlasmodium vivax, malariea, ovale &falciparum (most fatal causing cerebralmalaria and blackwater fever)After infecting hepatocytes, merozites infecterythrocytes forming trophozites (characteristic toeach specie) which divides giving rise to newmerozites that destroy RBCs upon escapeSpikes of shaking, chills and fever at intervalswhich coincide with merozite release from RBC.Brown discoloration of spleen, liver, lymphnodes & BM, massive spleenomegaly(hyperplasia of mononuclear phagocytes)

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You did really good job. I was so confused until I saw this summery. TX I was wondering Is there any way I can download this page? It would REALLY HELP.
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