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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)
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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)
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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
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Normal ranges: serum iron, ferritin, TIBC
- serum iron: 50-170 ug/dL(male slightly higher)
- Ferritin: 12-30ng/mL
- TIBC: 250-450 ug/dL
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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
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Steps of iron deficiency
- Store iron depletion: marrow, ferritin
- Transport iron depletion: ferritin, serum iron
- Functional iron depletion: anemia, diserythropoiesis
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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
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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
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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
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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)
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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
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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
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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).
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DAT
- pt serum + test cells incubation
- ^ incubation with anti-human IgG
- agglutination is positive test.
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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
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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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