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Heme 1 > anemia > Flashcards

anemia Flashcards

1
Q

megaloblastic anemia is caused from

A

defective DNA synthesis-B12/Folate deficiency

aka macrocytic anemia (large cells)

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2
Q

This binding protein ‘ ’ attaches to B-12 in gastric juice

A

R

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3
Q

Intrinsic factor is made via parietal cells. IF-B12 complex attaches to receptors in distal ileum and releases into blood stream. This complex attaches to ( ) in the portal blood

A 
TC II (transcobolamine II) and brings it to the bone marrow
TC I and III grab the B12 but don't go anywhere
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4
Q

B12 is a coenzyme that methylates….

A

homocysteine to methionine

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5
Q

pernicious anemia

A

impaired uptake of Vit B12 from GI tract due to lack of intrinsic factor

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6
Q

pernicious anemia can present at age

A

2 when stores from utero are utilized

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7
Q

common clinical features of anemia

A
  • glossitis
  • angular stomatitis
  • jaundice
  • weight loss
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8
Q

when do neural tube defects occur?

A

within 10 days of conception

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9
Q

Serum Fe and ferritin levels are raised in

A

megaloblastic anemia bc Fe is used to make RBCs

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10
Q

Serum folate is elevated and RBC folate is decreased in…

A

B12 deficiency

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11
Q

use B12 with….

A
  • total gastrectomy

- ileal resection

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12
Q

use folate with…

A

-pregnancy ASAP

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13
Q

hereditary spherocytosis is caused by a defect in

A

spectrin (RBC membrane protein) defect

leads to decreased HH

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14
Q

hereditary spherocytosis inheritance is…

A

dominant, northern european

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15
Q

clinical features of spherocytosis….

A
  • aplastic crisis
  • splenomegaly (removed after age 5)
  • jaundice
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16
Q

What lab tests are used to dx spherocytosis?

A

osmotic fragility test “increased”

17
Q

elliptocytosis is ( ) than spherocytosis

A

milder disorder

18
Q

what is wrong with G6PD deficiency pts

A

impaired reduction of glutathione, inability to remove oxidants
x linked disease, follows malaria belt

19
Q

these cells are seen in G6PD deficiency crisis

A

bite cell
blister cell
heinz bodies removed by spleen

20
Q

pyruvate kinase deficiency shows this type of inheritance

A

autosomal recessive

21
Q

Evan’s syndrome vs

aplastic anemia

A

not making RBCs or platelets

not making any blood cells

22
Q

who requires increased Fe

A
  • pregnancy
  • infancy and adolescence
  • menstruation
23
Q

what are the types of Hg you have as an infant?

A

95% fetal Hg, rest is A1 and A2
Once you take your first breath you start making a1/a2 and stop making f
By 18 months of age a1 is 95%

24
Q

If hg electrophoresis is normal then you have the

A

alpha trait, if it is low then you have beta

25
Q

sickle cell

A

Beta chain, 6th position glutamic acid (normal,negative) is replaced with valine (neutral)
2 different types of cells are produced, normal RBCs and sickled……the normal one cannot bend and they break and get trapped in the capillaries leading to a painful crisis

26
Q

how to tx sickle cell

A

Tx-overload circulatory system with double maintenance fluid, give pks

27
Q

Hg C disease

A

instead of glutamic acid it is leucine, target red cells that sickle

28
Q

aplastic/hypoplastic anemia

A

loss of all blood cell type production

29
Q

MCC aplastic anemia

A

Hep A

30
Q

congenital aplastic anemia is AKA

A

fanconi, recessive inheritance
skeletal defects, absent thumbs/radii
occurs b/w ages 5-10

31
Q

main presenting feature of ALL/AML

A

aplastic anemia

32
Q

PE findings for a pt with aplastic anemia?

A

liver/spleen/nodes are not enlarged, the marrow however is no good

33
Q

blood profile for an aplastic anemic pt

A

-MCV 95-110
-decreased retic count
-no abnormal cells on smear
-marrow replaced by fat
essentially just making a few cells

34
Q

MCC of marrow failure

A

graft verse host rejection

35
Q

red cell aplasia

A

-no red cell production
-low HH and almost no retic count
congenital-blackfan-diamond recessive inheritance

36
Q

transient erythroblastopenia of childhood

A
  • transient form of red cell aplasia

- HH 5/15, leave kid alone, seen 2-3 years of age

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