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heme II > section 17 - anemias pt 2 > Flashcards

section 17 - anemias pt 2 Flashcards

1
Q

define hemoglobinemia

A

excess hemoglobin in blood plasma

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2
Q

define hemoglobinuria

A

excess hemoglobin in urine

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3
Q

define hematuria

A

blood in urine

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4
Q

define hemosiderinuria

A

hemosiderin in urine

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5
Q

define the membrane defect in hereditary spherocytosis

A
  • vertical spektrin ankyrin defect
  • causes lipid destabilization and loss of membrane material with age
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6
Q

describe osmotic fragility with hereditary spherocytosis

A

increased

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7
Q

describe relative anemia (hydremia)

A
  • increase plasma vol but stable hct
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8
Q

list possible causes of hydremia

A
  • pregnancy
  • hyperproteinemia
  • mass IV or FFP infusion
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9
Q

describe classic presentation of hereditary spherocytosis

A
  • jaundice
  • splenomegaly
  • anemia
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10
Q

describe treatment possibilities of HS

A
  • if mod to severe hemolysis = splenectomy
  • splenectomy will resolve symptoms but defect persists
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11
Q

lab findings of spherocytosis

A
  • DAT (=)
  • folic acid: decrease (using more)
  • MCV: decrease
  • MCHC: increased
  • reticulocytes: increased in normal response
  • RDW: increased
  • osmotic fragility: increased
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12
Q

describe the principle of osmotic fragility

A

RBCs placed in hypotonic soln will draw in water and swell until lysis

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13
Q

at what point do normal rbc pops begin lysis

A

0.45% and complete at 0.35%

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14
Q

what is the osmotic fragility of target cells

A

decreased due to less hgb present opening up space for water

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15
Q

equation of osmotic fragility

A

% hemolysis = (OD of sample supernatant/OD of water tube)*100

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16
Q

at what point do spherocytes begin hemolysis in osmotic fragility testing

A

0.85%-0.55%

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17
Q

describe the main defect in hereditary elliptocytosis

A

defect in alpha or beta chain spectrin - horizontal interactions causing failure to return to disk shape

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18
Q

lab findings of hereditary elliptocytosis

A
  • inc elliptocytes
  • MCV norm-inc
  • MCHC norm
  • osmotic fragility norm
  • may be mildly heat sensitive
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19
Q

what is the RBC morphology of spherocytic HE

A

spoon shaped ovalocytes
- protective against malaria

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20
Q

describe the defect of hereditary pyropoikilocytosis

A

alpha or beta spectrin mutation and decreased synthesis of alpha spectrin leading to fragmentation

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21
Q

describe thermal instability of hereditary elliptocytosis

A
  • heated at 45 C they fragment
  • normal cells fragment at 49C
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22
Q

findings of hereditary pyropoik

A
  • facial bone abnormalities
  • MCV decrease
  • MCHC inc
  • inc spherocytes/elliptocytes
  • micro poik
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23
Q

describe defect of hereditary stomatocytosis

A
  • deficiency of stomatin and failure of sodium potassium pump
  • increase cellular water = cells swell
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24
Q

other lab findings of hereditary stomatocytosis

A
  • MCV inc
  • MCHC decrease
  • stomatocytes
  • osmotic fragility inc
  • 2,3-BPG decrease
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25
Q

describe defect of hereditary xerocytosis

A
  • cells lose potassium due to permeability defect causing dehydration
26
Q

lab findings of hereditary xerocytosis

A
  • MCV inc
  • MCHC inc
  • stomatocytes
  • target cells
  • puddle cells
  • 2,3-BPG decrease
  • osmotic fragility decrease
27
Q

describe the defect in acanthocytosis

A
  • abnormal membrane lipids reflect dist of plasma phospholipids
  • acanthocytes contain excess cholesterol
28
Q

describe possible causes of acanthocytosis

A
  • hereditary absent LDL
  • liver disease
  • malnutrition
  • certain blood groups
  • vitamin E deficiency
29
Q

generally describe anemia of liver disease

A
  • cholesterol and lipids are inc leading to alteration of RBC
  • round macrocytes
30
Q

generally describe anemia of alcoholism

A
  • ethanol has direct toxic effect on precursor cells, marrow cellularity, and rbc morphology
31
Q

what is the most common hereditary enzyme deficiency

A

G-6-PD (X linked)

32
Q

what is G6PD responsible for

A
  • hexose monophosphate shunt and generation of NADPH to protect from oxidative injury
33
Q

what are the consequences of oxidative agent activity

A

denatured hgb (heinz bodies) and skeletal structural abnormalities

34
Q

how does G6PD result in hemolysis

A
  • due to heinz body formation and removal
  • limited to older cells due to lower amt of G6PD
  • amt retics affect rate of hemolysis
35
Q

what stain is used for identification of heinz bodies

A

supravital stains

36
Q

how does the G6PD screening test determine G6PD deficiency

A
  • G6PD deficiency shows no fluorescence under UV light
  • cells with G6PD will fluoresce
37
Q

what pathway is pyruvate kinase associated with

A

embden-meyerhof - ATP production

38
Q

how does PK screening show deficiency

A
  • PK deficiency shows fluorescence under UV light
39
Q

how can PK deficiency be treated

A

it can’t, only supportive therapy

40
Q

describe the defect in PNH

A

an acquired myeloproliferative disorder w/ defect in pluripotent stem cell causing absence of anchor protein

41
Q

in PNH how does a lack of anchor protein affect the cell

A

no production of CD55 or 59 allowing complement fixation on the cell membrane

42
Q

how does night time effect PNH

A

slow metabolic rate at night lowers plasma pH increasing complement activity and lysis

43
Q

what can PNH evolve into

A
  • aplastic anemia
  • IDA
  • 30% of patients have aplasia before PNH
44
Q

describe the hemolytic factor of MAHA

A

RBC pass through fibrin strands and fragment
- chronic course
- DIC is an acute MAHA

45
Q

what two disorders are associated with MAHA

A
  • thrombotic thrombocytopenic purpura (TTP)
  • hemolytic uremic syndrome (HUS)
46
Q

define TTP

A

abnormal clots with low platelets and bruising

47
Q

what is the mechanism causing TTP

A
  • small platelet aggregates and unusually large vonWilebrand factor platelets swarm around
48
Q

what causes long vonWillebrand factor

A

absent ADAMTS13
- responsible for cleavage of VW

49
Q

how does vonwilebrand usually work in the body

A
  • broken up pieces via ADAMTS13 are involved in adhesion of platelets to site of injury
50
Q

what are the two forms of TTP

A
  • acquired (more common): autoantibody to ADAMTS13
  • rare inherited ADAMTS13 deficiency
51
Q

describe HUS

A
  • resembles TTP
  • found in young children
  • causes acute renal failure
  • absence of neurologic symptoms
52
Q

how does HUS associated with MAHA relate to infections

A
  • after enteric infection toxins target renal capillary endothelium
  • initiate DIC and cancels fibrinolysis
  • platelet activation
  • platelet aggregation in kidney
53
Q

other causes of non-immune hemolytic anemia

A
  • malignant hypertension
  • DIC
  • malignant fibrin
  • chemotherapy
  • aortic heart valve (unnatural tissue damages RBCs as they pass)
  • march hemoglobinuria (physical damage)
54
Q

describe autoimmune hemolytic anemia and two main kinds

A

Ab against self
- warm reactive (most common): IgG reacts at body temp, DAT +
- cold reactive: IgM reacts at 4C, DAT =

55
Q

describe cold agglutinin disease syndrome

A
  • anti-I, secondary to infection
  • cold weather causes agglutination of RBC in skin capillaries
  • DAT + at 15-32 for complement
56
Q

describe paroxysmal cold hemoglobinuria (PCH)

A
  • common in kids
  • associated with viral disorder
  • IgG binds and fixes complement at low temp
  • hemolysis of fixed cells at higher temps
57
Q

what is the classid antibody of PCH

A

donath-landsteiner

58
Q

describe immune complex drug induced hemolytic anemia

A

drug and pt Ab form complex that attaches RBC and activates complement

59
Q

describe hapten drug induced hemolytic anemia

A

drug nonspecifically bound to RBC and pt develops anti-drug Ab that reacts with cell bound drug

60
Q

define alloimmune hemolytic anemia

A

antibody against foreign antigen - typically transfusion rxn or HDN

61
Q

describe the principle kleihauer betke test for fetal hgb detection

A

all hgb elute from rbcs in acid bath, hgb F elutes slower and can be distinguished from adult hgb once stained with hgb stain

heme II (11 decks)

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