CLSHeme - 02 - Anemias

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  1. Rule of 3 (w/ normals).  Violations?
    • RBC: 4.6-6.0x10^6/uL
    • RBC x3 = Hgb: 14-18g/dL
    • Hgb x3 = Hct: 40-54%
    • *NOTE - women slightly lower
    • Violations - microcytic & elevated, macrocytic & decreased, hemolyzing (Ab coat), clumping (cold agg, abn plasma proteins)
  2. MCV, MCH, MCHC - equation, explanation, units
    • MCV: chart?chf=bg,s,00000000&cht=tx&chl=%5Cfrac%7BHct%5Ctimes10%7D%7BRBC%7D&chs=114x68 volume.  (fL)
    • MCH: chart?chf=bg,s,00000000&cht=tx&chl=%5Cfrac%7BHgb%5Ctimes10%7D%7BRBC%7D&chs=118x74 mass hgb per cell. (pg)
    • MCHC: chart?chf=bg,s,00000000&cht=tx&chl=%5Cfrac%7BHgb%5Ctimes100%7D%7BHct%7D&chs=134x74 [ ] hgb per cell volume. (%)
  3. What is inside reticulocytes?  Why measure?  Corrected count? RPI? maturation time?
    • RNA/ribosome remnants
    • Evaluation of erythrocytic activity in marrow
    • Corrected count: %retic x Hct/.45 (.45 = normal hct)
    • RPI: corrected retic / maturation time (from table)
    • *maturation time increases as hct decreases (increased epo causes earlier release)
  4. Ferritin, hemosiderin, transferrin, heme
    • Ferritin: iron storage (easily accessable)
    • Hemosiderin: iron storage (not easily accessable, RE macrophages)
    • transferrin: transport (TIBC)
    • heme: primary utilization of iron
  5. Normal ranges: serum iron, ferritin, TIBC
    • serum iron: 50-170 ug/dL(male slightly higher)
    • Ferritin: 12-30ng/mL
    • TIBC: 250-450 ug/dL
  6. Hypochromic anemias (4) w/ basic details
    • Iron def: microcytic/hypochromic, increased TIBC, decreased iron stores, tferrin saturation
    • Chronic disease: microcytic/hypochromic, impaired iron metabolism, diserythropoiesis, TREAT DISEASE FIRST
    • Sideroblastic: microcytic/hypochromic, impaired iron utilization resulting in sideroblasts, dimporphic cell pop
    • sideroblasts - iron collected in mitochondria of erytrhocytes
    • Hereditary (X-linked, autosomal porphyrias) and acquired (lead poisoning, drugs)
    • Hemochromatosis: iron overload, hereditary or acquired via tfusion/anemia
  7. Steps of iron deficiency
    • Store iron depletion: marrow, ferritin
    • Transport iron depletion: ferritin, serum iron
    • Functional iron depletion: anemia, diserythropoiesis
  8. Macrocytic anemias (2) w basic details
    • B12/Folate (megaloblastic): pancytopenia, hyperseg, cabot rings, macro-ovalocytes
    • Lack of B12/Folate decreases avail nucleotides inhibiting cellular division
    • hyperceullular marrow, decreased retic (cells don't enter periph)
    • pernicious anemia (IF), autoimmune, dietary, etc
    • non-megaloblastic: altered maturation time of RBC precursors, many varying causes
  9. aplastic anemia and pure red cell aplasia AKAs and details
    • aplastic: <25% cellularity in marrow (all lines)
    • refractory, 95% acquired - idiopathic
    • FANCONI'S ANEMIA - rare congenital form (DS DNA can't be repaired)
    • PRCA: normocytic anemia (lack retic) without any other lines affected
    • congenital as Diamond-Blackfan syndrome
  10. Missing proteins + fragility/basic info - spherocytes, elliptocytes, stomatocytosis, xerocytosis
    • sphero: spectrin, ankyrin
    • increased fragility, splenomeg/mild jaundace
    • Ellipto: spectrin, protein 4.1
    • normal fragility, no major symptoms
    • Stomato: Na permeability increased
    • Xero: K+ permeability increased
  11. Paroxysmal nocturnal hemoglobinura - basic info (incl tests)
    • Complement binds to cell surface causing lysis
    • Blood in urine after sleep
    • Mild/severe anemia (chronic hemolysis)
    • Sugar water hemolysis test (tonicity)
    • Ham's acidified serum lysis test (inactivate pt serum and add to pt cells and ctl cells - hemolysis on only pt cells)
  12. Enzyme defect basic info - G6DP, Pyruvate kinase
    • G6DP: enzyme normally restores methemoglobin to oxyhemoglobin
    • acute attacks after oxidizing drugs, vigorous exercise, etc
    • Heinz bodies preesent
    • PK: Last step of glycolysis is stunted
    • Can be mild to severe requiring transfusions
  13. Hemoglobinopathies with basic info incl mutation - S, C, SC, D, E, F, G, unstable hgb variants
    • S: Valine sub for glutamic acid at pos 6 of hgb
    • two copies of mutation = 80%S, 20%F
    • sickled cells accumulate in organs and vasooccclusion
    • transfusion (F?) and marrow tplant
    • C: Lysine subs for glutamic acid at pos 6
    • mild hemolytic anemia in disease, no findings for trait
    • Many target cells, hgb C crystals
    • SC: combo, but less severe than SS
    • D: glycine for glutamic acid at pos 121
    • mild anemia, no treatment req
    • E: lysine for glutamic acid at pos 26
    • found in 30% of SE Asia pop
    • trait asymp, disease microcytic anemia w/ target
    • F: beta or delta chain is inactive, no disease
    • rarely have >20% F hgb
    • G: alpha variant - lysine for asparagine at pos 68
    • no treatment req
    • O-ARAB: lysine for glutamic acid at pos 121
    • very rare, but SEVERE if inheritited with S
    • UHV: homozygous condition incompatible with life
    • 25% patients are anemic, majority no impact
  14. Thalassemias - beta minor, beta major, alpha 1-4 mutations
    • bmin: asymptomatic, A2 increased, microcytic values
    • bmaj: severe anemia, extreme erythroid hyperplasia, hepatosplenomegaly
    • a1: silent carrier
    • a2: "minor", normal - mild, microcytic/hypochromic
    • a3: "major", moderate anemia with few transfusions, target cells, 25-40% Bart's or H (depending on age)
    • a4: "Bart's/Hydrops fetalis", lethal (no O2 carried).
  15. DAT
    • pt serum + test cells incubation
    • ^ incubation with anti-human IgG
    • agglutination is positive test.
  16. Autoimmune hemolytic anemias BASIC - warm, cold, paroxymal cold hemoglobinuria, drug-induced mechanisms (4)
    • warm: body temp (poor prognosis), iditopathic, pos DAT
    • cold aggl: idiopathic or assoc w/ lymproliferative disorder, pos DAT
    • can be secondary to infection (self limiting anti-I)
    • PCH: children after viral infection, anti-P reacts at cooler temps
    • Donath-Landsteiner test confirms (whole blood and test blood at 4 and 37, spin and compare for hemolysis)
    • DI: immune complex formation, drug adsorption to cell surface, membrane modification and abs of proteins, autoimmunity
  17. Anemia from systemic diseases - renal, liver, alcoholism, endocrine, malignancy, HIV, infancy, malaria
    • renal: decreased EPO
    • liver: lipids, clotting factors, proteins
    • alcohol: abn  hgb, chronic liver disease
    • endocrine: addison's (insufficiency), thyroid affects EPO and O2 demand, gonads stimulate erythroids
    • malignancy: marrow infiltration/fibrosis
    • HIV: reduced T cells, treatment inhibits DNA synthesis
    • infancy: decreased EPO, low EPO response
    • malaria: schizonts burst ruptruing red cells - multiple cycles

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victimsofadown
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325908
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CLSHeme - 02 - Anemias
Updated:
2016-11-21 15:35:54
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CLSHeme 02 Anemias
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