anemia Flashcards
megaloblastic anemia is caused from
defective DNA synthesis-B12/Folate deficiency
aka macrocytic anemia (large cells)
This binding protein ‘ ’ attaches to B-12 in gastric juice
R
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
TC II (transcobolamine II) and brings it to the bone marrow TC I and III grab the B12 but don't go anywhere
B12 is a coenzyme that methylates….
homocysteine to methionine
pernicious anemia
impaired uptake of Vit B12 from GI tract due to lack of intrinsic factor
pernicious anemia can present at age
2 when stores from utero are utilized
common clinical features of anemia
- glossitis
- angular stomatitis
- jaundice
- weight loss
when do neural tube defects occur?
within 10 days of conception
Serum Fe and ferritin levels are raised in
megaloblastic anemia bc Fe is used to make RBCs
Serum folate is elevated and RBC folate is decreased in…
B12 deficiency
use B12 with….
- total gastrectomy
- ileal resection
use folate with…
-pregnancy ASAP
hereditary spherocytosis is caused by a defect in
spectrin (RBC membrane protein) defect
leads to decreased HH
hereditary spherocytosis inheritance is…
dominant, northern european
clinical features of spherocytosis….
- aplastic crisis
- splenomegaly (removed after age 5)
- jaundice
What lab tests are used to dx spherocytosis?
osmotic fragility test “increased”
elliptocytosis is ( ) than spherocytosis
milder disorder
what is wrong with G6PD deficiency pts
impaired reduction of glutathione, inability to remove oxidants
x linked disease, follows malaria belt
these cells are seen in G6PD deficiency crisis
bite cell
blister cell
heinz bodies removed by spleen
pyruvate kinase deficiency shows this type of inheritance
autosomal recessive
Evan’s syndrome vs
aplastic anemia
not making RBCs or platelets
not making any blood cells
who requires increased Fe
- pregnancy
- infancy and adolescence
- menstruation
what are the types of Hg you have as an infant?
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%
If hg electrophoresis is normal then you have the
alpha trait, if it is low then you have beta
sickle cell
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
how to tx sickle cell
Tx-overload circulatory system with double maintenance fluid, give pks
Hg C disease
instead of glutamic acid it is leucine, target red cells that sickle
aplastic/hypoplastic anemia
loss of all blood cell type production
MCC aplastic anemia
Hep A
congenital aplastic anemia is AKA
fanconi, recessive inheritance
skeletal defects, absent thumbs/radii
occurs b/w ages 5-10
main presenting feature of ALL/AML
aplastic anemia
PE findings for a pt with aplastic anemia?
liver/spleen/nodes are not enlarged, the marrow however is no good
blood profile for an aplastic anemic pt
-MCV 95-110
-decreased retic count
-no abnormal cells on smear
-marrow replaced by fat
essentially just making a few cells
MCC of marrow failure
graft verse host rejection
red cell aplasia
-no red cell production
-low HH and almost no retic count
congenital-blackfan-diamond recessive inheritance
transient erythroblastopenia of childhood
- transient form of red cell aplasia
- HH 5/15, leave kid alone, seen 2-3 years of age
