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↓production 2 of 3 sign.granulocyte<500/ul,plt,20K/ul.corected retic<1% Sever aplastic anemia gra<200/ul
Hematologic findings Normo-macrocytic anemia Aniso-poikilo(oval-macrocyte) RDW Leukopenia,thrombocytopenia ▲fe,co,folate normal BM:fat tissue,fibrosis,↓cellularity,
BM biopsy hypocellular,increased fat spaces
signs Sever pancytopenia or chronic phase Bleeding (40%),infection,no splenomegaly,lymphodenophaty
etyology -Heriditary -non beriditary Idiopatic in 70% Drugs(anti neoplastic busulfan,oratan,) & chemical agent 11-20%(irradiation
"name": "etyology -Heriditary -non beriditary Idiopatic in 70% Drugs(anti neoplastic busulfan,oratan,) & chemical agent 11-20%(irradiation
Mechanism of dx Direct(immunologic,drugs,irradiatin,viruses Activation tcyt
prognosis Degree of pancytopenia Sex age
<50y BMT Anti lym-globolin,coricosteroids antibiotic
chol Dose dependent(reversable):↓retic,↓erytheroid cells Dose independent(3-5m after last )
Aplastic anemia related other disease Infection(HIV,B19,hepatitis) PNH Pregnancy immunologicDx(RA,lopous,MS,tyroid immune dx)
hereditary Heriditary defect in BM -fancony anemia Autosomal Ressesive,pancytopenia,sever in 8y,normo-macrocytic ▲HBF,ag I,NM hypo cellular,hypo pigmentasion,short,hypogonadism,deformit in finger,radius,).microcephaly,deformity (heart,kidny)
Chromosal breaking & rearranging Mitomycin C,deeppoxy butan, Defect in FANCA in70% In chromosal stability Survival 19y15% neoplasia -amegakaryocytic thrombocytopenia defect in c-MPL Dyskeratosis maternal,shoakhman- diamonad(pancras defect,nutropenia) syndrom shift to AA
-pure red cell aplasia non lasting aplastic crisis B19 Erytheroblastopenia in infants:1-3y.viruses Diamond-blackfan.rare,in 1y,▲HbF,↓BFU- E,CFU-E▲apoptosis 75%cortico steroids,
Sidroblasic anemia 20-60% e-precursore sidroblasts Ring sidroblast Defect in heme synthesis Erytheroid hyper plasia Normochrom-hypocromia ▲sfe,↓TIBC,▲saturation degree
Hereditary SA X-linked(↓δALAS-2 ↓link to B6 or ▲degradiation enzymto mitoconderial proase,autosomal dominant,ressesive) Mitoconderialdeletion(pyerson syndrom (pancras defect,BM cells vaculization,ring sidroblast
Acquired SA Idiophatic(15% sidroblast ring) Secondary:isonisid.sicloserin.pirazin amid Pb(ALA synthase,ALA dehydratase,hem syntetase) Ala,coproporfirin increase.chloram with inhibition protein synthase.Zn.Cu Penicilin amin Zn chelator Ethanol reversable SA B6 deficiency with malnutrition (neuropaty,skin irritation) B6 deficiency
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