Red Blood Cell Disorders 1 Flashcards

1
Q

average life span of the RBC:

A

120 days

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2
Q

what is the difference between MCH (mean cell hemoglobin) and MCHC (mean cell hemoglobin concentration)?

A

MCH: average content of hemoglobin PER RBC
MCHC: average concentration of Hb in a given volume of packed RBC’s

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3
Q

what is the significance of RDW?

A

RDW = Red cell Distribution Width

it is a coefficient of variation of RBC volume

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4
Q

normal size of a RBC should be nearly the same size as _____

A

lymphocytes

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5
Q

what diseases can cause microcytic hypo chromic anemias

A
  • iron deficiency
  • lead poisoning
  • anemia of chronic disease
  • thalassemia
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6
Q

what disease can cause normochromic normocytic anemia?

A
  • acute blood loss
  • hemolytic anemia
  • anemia of chronic disease
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7
Q

what disease can cause normocytic macrocytic anemia?

A
  • folate deficiency

- B12 deficiency

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8
Q

lead poisoning can result in what type of anemia?

A
  • microcytic hypochromic anemia
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9
Q

what are two broad categories of causes of anemia?

A
  • accelerated RBC loss or destruction

- impaired RBC production

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10
Q

compare the anemia seen in acute vs chronic blood loss

A

acute: risk of hypovolemia and associated with compensatory rise of erythropoietin levelsl normochormic normocytic anemia
chronic: no risk of hypovolemia; associated with iron deficiency → microcytic hypochromic anemia

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11
Q

what kind of anemia is associated with acute hemorrhage

A

normochromic normocytic

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12
Q

what kind of anemia is associated with chronic blood loss?

A

microcytic hypochromic

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13
Q

what are 3 mechanisms of intravascular hemolysis

A
  • mechanical injury to RBC’s such as defective valves, thrombi or heat
  • complement fixation of antibody coated RBC’s
  • infections (intracellular parasites such as malaria or production of toxins such as clostridia)
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14
Q

examples of extravascular hemolysis

A
  • due to inc phagocytic destruction of RBC’s primarily in the spleen
  • RBC’s less deformable: spherocytosis, sickle cell
  • RBC”s are rendered foreign via antibody coating
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15
Q

______ hemolysis is associated with splenomegaly

A

extravascular

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16
Q
discuss the following plasma serum levels in hemolytic anemia: 
unconjugated bilirubin
LDH 
haptoglobin 
free hemoglobin level
A

↑ unconjugated bilirubin
↑ LDH
↓ or absent haptoglobin (binds free Hb so when most of them bind the Hb, the levels of haptoglobin will ↓)
↑ free hemoglobin level

17
Q

which type of hemolysis: intravascular. or extravascular has more bilirubin in the serum

A

extravascular. with intravascular, some of the bilirubin can be lost in the urine

18
Q

hemoglobinuria is seen in intra/extra vascular hemolysis

A

intravascular hemolysis due to the absence of haptoglobin

19
Q

compare the levels of haptoglobin between intra and extravascular hemolysis

A

intravascular: absent haptoglobin
extravascular: ↓ haptoglobin

20
Q

what an examples of intrinsic RBC defects leading to hemolytic anemia?

A
  • membrane defect: hereditary spherocytosis
  • enzyme defect: G6PD
  • hemoglobin defect: sickle cell and thalassemia
  • paroxysmal noturnal hemoglobinuria
21
Q

what mutated proteins are associated with hereditary spherocytosis?

A
  • ankyrin
  • band 3
  • spectrin
  • band 4.2
22
Q

intercurrent infections of ____________ with hereditary spherocytosis can induce aplastic or hemolytic crisis

A

parvovirus B19

23
Q

↑ or ↓ MCHC in patients with hereditary spherocytosis

A

24
Q

________ are indicative of splenectomy

A

Howell - Jolly bodies

25
Q

what are two tests you can do to diagnose hereditary spherocytosis

A
  • osmotic fragility test: spherocytes lyse prematurely when exposed to hypotonic salt solutions
  • Coombs test: negative test is diagnostic of hereditary spherocytosis
26
Q

_______ form of glutathione is required to convert hydrogen peroxide to water via the enzyme __________

A

reduced; glutathione peroxidase

27
Q

in order to get more reduced glutathione, it requires the enzyme ________ and ______

A

glutathione reductase and NADPH

28
Q

“bite cells” are seen in ______

A

G6PD

29
Q

describe the steps to the formation of Heinz bodies in ______

A

G6PD;

oxidants will oxidate the SH groups on globing chains of Hb → precipitation of donation globins on RBC membrane

30
Q

when should a G6PD enzyme assay be done?

A
  • three months after the hemolytic episode has resolved

dont do the assay during the acute hemolytic episode because it will produce false negative results