Aplastic anemia

↓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 biopsyhypocellular ,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 <first erytheroid,mild:granulocye,meg:low),banzen).2-9% hepatitis

Sever irritation to drugs(anti microbial:salvarsan,choloram,sulfonamids,stereptomycin),antityroid(carbimazol,),anti ach(phenyl butazin),anti histamin,cymitidin,DDT,gold,bismot,hair color…..

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 anemiaAutosomal 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