Anemia Due to Hemolysis Flashcards

1
Q

Hemolysis is defined as __________.

A

decreased RBC survival or an increase in RBC turnover beyond the normal range

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2
Q

RBCs can survive in blood for _________.

A

120 days (+/- 20 days)

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3
Q

Marrow production can increase by ______.

A

6-8 fold

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4
Q

List five cellular processes associated with normal turnover.

A
  • decreased enzyme activity
  • changes in calcium balance
  • oxidative injury
  • changes in membrane surface constituents
  • antibodies attaching to membrane surface
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5
Q

What lab values are typically elevated in hemolytic anemia?

A
  • reticulocyte count
  • serum/urine hemoglobin
  • bilirubin (unconjugated)
  • methemalbumin
  • LDH, SGOT (“housekeeping enzymes”)
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6
Q

What is the most common molecular abnormality in hereditary spherocytosis?

A

Spectrin deficiency

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7
Q

Hereditary spherocytosis presents with _______.

A

anemia, intermittent jaundice, splenomegaly, and responsiveness to splenectomy

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8
Q

Other than spectrin deficiency, spherocytosis can also be caused by _________.

A

ankyrin and band 3 defects

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9
Q

Familial spherocytosis is transmitted in a _________.

A

dominant (75%) and recessive (25%) manner

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10
Q

Conjugated hyperbilirubinemia results from ________.

A

liver damage

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11
Q

Aplastic crisis is _________.

A

the temporary cessation of RBC production (which is markedly worse in patients with sickle cell)

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12
Q

Genetically, glucose-6-phosphate dehydrogenase deficiency is _______.

A

an X-linked recessive disorder

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13
Q

G6PD is involved in the ______ pathway, which produces NADPH that then leads to ________.

A

pentose; glutathione production–important for production against oxidative damage

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14
Q

In autoimmune hemolytic anemia, ______ causes intravascular hemolysis and _____ causes extravascular hemolysis.

A

IgM; IgG

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15
Q

Splenic hemolysis is referred to as _______.

A

extravascular

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16
Q

In extravascular hemolysis, macrophages ________.

A

swallow RBCs in the spleen, storing iron in ferritin and releasing protophorphyrin as bilirubin

17
Q

In the liver, bilirubin released by macrophages is _________.

A

metabolized in the phase II pathway (by addition of a glucuronic acid

18
Q

Describe what happens in the absence of glutathione (as found in G6PD deficiency).

A

Inability to protect against oxidation results in denatured hemoglobin that binds to the membrane, damaging the cell.

19
Q

Coombs tests examine for _______.

A

hemolytic anemia