IM anemia

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TerryZ
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251247
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IM anemia
Updated:
2013-12-06 19:12:46
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internal medicine IM anemia microcytic normocytic macrocytic hemolytic sickle thalassemia
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IM
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Internal medicine - anemia
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  1. Anemia - facts
    • Compens: /CO, /Extract, R-shift, /vol
    • Xfuse: Hgb<7, pt needs /O2 capacity
  2. Anemia - S/Sx
    • HA, fatig, \conc, D, N, abd discomf,        
    • palor, HoTN, tachy,
    • underly e.g. jaundice, stool blood
  3. Anemia - Dx components
    • Hgb/HCT: 1 unit PRBC} /Hgb 1, /HCT 3
    •               Hgb 7 or 8 OK if good card fn
    • Retic ndx: ?eff erythropoiesis >2%
    •    raw matls: Fe, B12, folate
    •    x marrow dz: aplastic anemia
    •    EPO: adequate
    •    Retic nix: survival
    • Blood smear: .
    • RBC ndx: esp MCV
  4. Anemia - Dx approach
    • Retic ndx <2 (normal)
    •   MCV<80 (microcytic)
    •      IDA, chron dz, thalass, ring siderobl
    •   MCV>100 (macrocytic)
    •      \B12,folate, liver dz, stim erythropo
    •   normocytic
    •      aplast,BMfibr,tumor,C-infl/malig,RF
    • Retic ndx > 2
    •   suspect blood loss, hemolysis
  5. Transfusion pearls
    • PRBC: no plt/clot-F; w/NS 90-120m; CBC
    • FFP: clot-F} //PT,PTT; coagulop; \clot-F
    • Cryoprecip: Factor VIII, fibrinogen
    •   for hemophilia A, \fibr (DIC) & vWD
    • Plt xfusn: 1u -> /10K plt cnt
    • Whole blood: massive blood loss
    • Ratio: platelets:FFP:PRBC=1:1:1, warm
  6. IDA - cause
    • C blood loss: menstrual, GI
    • Diet defic: infant/todl, adolesc, preg
  7. IDA - S/Sx
    • Pallor
    • Fatigue, general weakness
    • DOE
    • Orthostat lightheadedness
    • HoTN (if acute)
    • tachy
  8. IDA - Dx
    • \ferritin: most reliable
    • /TIBC, transferrin
    • \TIBC saturation
    • \Fe
    • /RDW
    • periph smear: μcytic HoChromic
    • marrow bx: AuStd, only if ?blood loss
    • ?GI bleed: guiaic, colonoscopy
  9. IDA - Tx
    • Oral Fe: ferrous SO4;SE const,N,dyspep
    • Parenteral: Fe dextran IM/IV
    • xfuse: severe or cardPulm dz
  10. Thalassemia - def
    • Inherited d/o
    • Inadequate prod'n α or β-globin chain
  11. β-thalassemia - facts
    • Etiol: /α-chains damage RBC membrane
    • Occ: Medit, ME, India
    • Types: T major, minor, intermedia
  12. Thalassemia major
    • AKA: Cooley's
    • Def: homozygous β-chain thalassemia
    • Course: dx 6-12 mo; xfuse dependent
    • S/Sx: sev μc/Ho,/HSM,/marrow space,
    •         \growth,death{CHF, crew-cutXR
    • Dx: Hgb EPh}/Hgb F&A2; target cells
    • Tx: freq PRBC xfusions
  13. Thalassemia minor
    • Def: heterozygous β-chain
    • Sx: asympt, mild HoChrom anemia
    • Dx: Hgb electrophoresis
    • Tx: Unnecessary
  14. Thalassemia intermedia
    • Etiol: involves both β-globin genes
    • Severity: intermediate
    • Tx: usually not xfusion dependent
  15. α-thalassemia - facts
    • \α-chains
    • β-globins form tetramers (abnl)
    • Types: carriers,α-T trait,Hgb H dz,all 4
  16. α-thalassemia - silent carrier
    • Def: mut/del one α-locus
    • S/Sx: asympt; nml Hgb,HCT;
    • Tx: none necessary
  17. α-thalassemia trait (minor)
    • Def: mut/del 2 α-loci
    • Occ: AA
    • S/Sx: mild μc/Ho
    • Tx: none necessary
  18. Hgb H dz
    • Def: mut/del 3 α-loci
    • S/Sx: hemolytic A, splenomeg, μc/Ho
    • Dx: Hgb electroph} Hgb H
    • Tx: Freq PRBC xfuse, splenectomy
  19. Sideroblastic anemia - facts
    • Def: abnl RBC Fe metab; hered/acquird
    • Drugs: chloramphenicol,INH,EtOH
    • Etc: Pb, collagenVD, myelodysplastic syn
  20. Sideroblastic anemia - S/Sx
    • /Fe & ferritin
    • oTIBC
    • o/TIBC sat
    • Ringed sideroblasts in bone marrow
  21. Sideroblastic anemia - Tx
    • Remove offending agents
    • ?Pyridoxine
  22. Microcytic anemia - iron studies
    • .           Serum   Serum
    • _          ferritin     iron     TIBC    RDW
    • IDA          L           L         H         H
    • ACD        N/H         L        L/N       N
    • Thalass    N/H       N/H       N       N/H
  23. Anemia of chronic dz - facts
    • Chronic infection (TB, abcess)
    • CA (lung, breast, Hodgkin's)
    • Inflam (RA, SLE)
    • trauma
    • rlse inflam cytokines -> \erythropoiesis
    • Similar appearance to IDA
  24. Anemia of chronic dz - Dx
    • \Fe
    • \TIBC
    • \transferin
    • /ferritin levels
    • nc/nC (μc/Ho)
  25. Anemia of chronic dz - Tx
    • Tx underlying process
    • x Iron
    • ??EPO (40u SQ weekly)
  26. Aplastic anemia - facts
    • Def: bone marrow fail->pancytopenia
    • Etio: Idio(MC), rad, med, viral, chem
    •   Med: chloramph,sulfonam,gold,mazie
    •   Vir: HPV,HCV,HBV,EBV,CMV,HZV,HIV
    •   Chem: benzene, insectides
  27. Aplastic anemia - S/Sx
    • Anemia: fatigue, dyspnea
    • TCP: petechia, easy bruise
    • /Inf: 2/2 neutopenia
    • xform: into acute leukemia
  28. Aplastic anemia - Dx
    • nc/nC
    • marrow bx: HoCellular, absent progen
  29. Aplastic anemia - Tx
    • bone marrow plant
    • PRBC/plts fusion if necessary
    • tx underlying causes
  30. Vit B12 deficiency - facts
    • B12: homocystein}methionine; methylmalonyl CoA}succcinyl CoA
    • Stores: Liver 3 yrs
    • Source: meat, fish
    • Bound2: intrinsic factor(gastric parietal)
  31. Vit B12 deficiency - causes
    • Impaired absorption
    • Pernicious anemia (\intrinsic factor)
    • Gastrectomy
    • Poor diet (vegetarian)
    • Alcoholism
    • Crohn's dz - ileal resection
    • Org'sms - fish tapeworm,bact overgrow
  32. Vit B12 deficiency - S/Sx
    • anemia
    • sore tongue
    • neurop: \LE posit'n,vibr; atax;
    •     UMN: /DTRs, spast, weak, Babinski
    • urin/fecal incontinence, impotence
    • dementia
  33. Vit B12 deficiency - Dx
    • PBS: megaloblastic anemia
    •         MCytic RBCs (MCV>100)
    •         HSeg neutrophils
    • \B12 (<100)
    • /methylmalonic a, homocysteine
    • Abs against intrinsic factor (pernicious)
    • Schilling test: reveals cause \B12
    •    IM Vit B to saturate binding sites
    •    PO RA vit B; measure urine/plasma
    •    Repeat w/IF. /B12= pernicious anem
  34. Vit B12 deficiency - Tx
    • Parenteral tx prefer: IM 1/mo
    • Un-tx = irrevers neuro dz
  35. Folate deficiency - facts
    • Stores: ltd (3 mo)
    • Source: green veggies (x overcooked)
    • Cause: \intake, EtOH, LT abx,/ demand,
    •         preg, hemol, Rex, pheny, dialysis
  36. Folate deficiency - S/Sx
    • \serum folic acid (can appear nl!)
    • RBC folic acid
    • \B12, /homocysteine, oMethymalonic a,
    • /MCV, anisocyt(size), poikilocyt(shape)
    • neutrophil hypersegm (5+ lobes)
    • HCell bone marrow (hyperplasia x 3)
  37. Folate deficiency - Tx
    • PO folic acid
    • (1-5mg/day)
  38. Hemolytic anemias - facts
    • D: premat destr RBC; marrow nl
    • P: erythropoiesis} /retic, can't keep up
    • C: A/C; acq/inher; intravasc/extravasc
  39. Hemolytic anemias by mechanism
    • Extrinsic
    •   Immune hemolysis
    •   Mechan hemolysis(pros valves, μangio)
    •   Ext: meds,burns,toxins,malaria,clostrid
    • Intrinsic
    •   Hgb abnl: sickle, Hgb C dz, thalassemia
    •   Membr: hered spherocytosis, PNH
    •   Enz def: G6PD, pyruvate kinase defic
  40. Hemolytic anemias - S/Sx
    • of anemia, underlying dz
    • bone crises in SCD
    • jaundice
    • dark urine (intravascular proc)
    • HSM, cholelith, LAP (chronic)
  41. Hemolytic anemias - Dx
    • Hgb/HCT: degree of hemolysis
    • /retic: /RBC production
    • Smear: }
    •   Schistocytes: trauma/mechanical
    •   Spherocytes: helmet; extravascular
    •   Sickled RBCs: SCA
    •   Heinz bodies: G6PD
    • Haptoglobin: low in hemolytic anemia
    • /LDH: RBCs destroyed
    • /Unconj bili: degradation of hgb
    • Direct Coombs test: + AI hemolyt anem
    • Osmotic fragility: .
  42. Hemolytic anemias - Tx
    • Underlying cause
    • xfuse PRBCs (severe, hemodyn compr)
    • folate supplement (depleted in hemol)
  43. Sickle cell anemia - facts
    • AR d/o: Hgb S replaces Hgb A
    • Distingu: electrophoresis
    • \O2: acid,hypox,ΔT,dehyd,inf}polymerize
    • Sickled: -> obstruction -> ischemia
    • Trait: 1/12 AA, screening
  44. Sickle cell anemia - prognosis
    • Surv: ::vaso-occl crises;
    • med: 35yo
    • less: -25 to 30y
  45. Sickle cell anemia - S/Sx
    • Jaund,pal,gallstn,CHF,aplast;VasoOcclus:
    •   P-ful crises: bone}sevP tib,hum,fem
    •   hand-foot syn: 4-6mo dorsa swell
    •   acute chst: pulmInfrct,CP,RD,infilt,HoO
    •   splenInfarct: -> autosplenectomy
    •   Jt avasc necr: hip & shoulder
    •   Priapism: .5-3h;hydral,nifed,stilbestrol
    •   CVA: cerebral throm (children)
    •   Opthm: infarct,hemorr,retinop,detach
    •   Ren pap necr w/hemat: ~P
    •   C leg ulcer: lateral maleoli
    •   Abd crisis: adult; like acute abd.
  46. Sickle cell anemia - Dx
    • Anemia: MC
    • Smear: Sickle-shaped RBCs
    • Predisp: Salmonella osteo{splenic malfn
  47. Sickle cell anemia - Tx
    • Advise: avoid ^alt; /fluids; tx infect'ns
    • Vaccine: S.pneumo,H.flu,N.menning
    • PCN: prophylactic 3m-6y
    • Folic a: supplem
    • Mg P crises: hydrat,morphine,warm,O2
    • Hydroxyurea: HgbF
    • Xfuse: ?clin bond;A-chest,priap,\card
  48. G6PD - facts
    • Gluc-6-phosphate dehydrogenase def
    • x-linked recessive d/o (M)
    • precip: sulfonamides, nitrofurantoin,
    •           primaquine,dimercaprol,fava,inf
  49. G6PD - types
    • A-varient: mild,10%AAM,old RBC hemol
    •    Trig: inf;primaquine,sulfonamide,TMP
    • Sev: Med, all RBCs, fava=hemA, Xfuse
  50. G6PD - S/Sx
    • Drug-induced: episod hemolytic anemia
    • PE: dark urine + jaundice
  51. G6PD - Dx
    • Smear: bite cells (-Heinz bodies)
    •            Heinz bodies: abnl Hgb precip
    • \NADPH form'n: on G6PD assay
    • G6PD lvl: diagnostic, may be nl, repeat
  52. G6PD - Tx
    • avoid precip drugs
    • maint hydration
    • RBC transfusion when necessary

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