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37 Cards in this Set

  • Front
  • Back
Hereditary spherocytosis
autosomal dominant trait
abnormal RBC membrane due to a protein and lipid defect. Reduced surface area, impaired flexibility and increased Na permeability
Increased MCHC
Increased osmotic fragility
Hereditary elliptocytosis
autosomal dominant trait
defect inteh membrane cytoskeleton
greater than 25% ellipotocytes
extravascular hemolysis
jaundice
increased extravascular hemolysis
indirect conjugated bilirubin
extravascular hemolysis
shape problem not hgb problem
pyruvate kinase deficiency
rare
autosomal recessive
enzyme deficiency of the EM pathway
burr cells
reduces ATP production needed for NA/K pump
glucose 6 phosphate dehydrogenase deficiency
inherited
doesnt appear without a oxidant stressor
x linked recessive
red cell enzyme deficiency causing hemolysis
intravascular hemolysis
heinz bodies/bite cells
Hemoglobin S (sickle cell anemia)
inherited
substitution of valine for glutamic acid in the beta chains of the Hgb molecule
homozygous (SS) sickle cell disease
heterozygous (AS) sickle cell trait
intra- and extravascular hemolysis
Targets
HGB SC
inherited
2 hgb varients
targets
pocketbook cells
mild anemia if at all
HGB C
targets
hgb c crystals
mchc increased
usually no symptoms
can become micro/hypo because body keeps taking cells out of circulation and producing more then run out of iron
paroxsmal nocturnal hemoglobinuria
rare
acquired
intrinsic
blood pH drops at night and binds complement cells lyae in the morning
extra- and intravascular hemolysis
biochemical and membrane abnormality
low hgb and platelet
hams acified serum test or the 'sugar H2O' test
heinz bodies
denaturation and precipitation of unstable hgb
pitted golf ball
supravital staining
microangiopathic hemolytic anemias
a disease of small blood vessels (changes within the small vessels cause circulating RBCs to be hemolyzed/fragmented)
DIC
occurs in patches
inside vessels
clotting
fibrin clots form inside vessels and RBCs are fragmented as they push through the the clots
TTP
rare
extensive platelet clumping occurs within blood vessels leading to decresed plts and schisto
causes renal failure
HUS
infants and kids
intravascular hemolysis
renal failure
p. falciparum
rare
may be more than one ring
causes hemolysis the fastest
maurere dots may be present
crescent gamecytes (bannana shaped)
p. vivax
rare
four maturation stages
rings
trophozoited
schizonts
gametocytes
schuffners granules or stippling
isoantibodies/alloantibodies
specific antigen from an individual of the same species
autoantibodies
reacts with self antigens
DAT
differentiates immune hemolytic anemias from non immune hemolytic anemias
detects the presence of antibodies that are attached to the Ags on RBC membrane
immune hemolytic anemia
Abs that attach to Ags on RBC membranes
fanconis
inherited aplastic anemia
idiopathic aplastic anemia
cause unknown 70% of cases
secondary aplastic anemia
acquired 25% of cases
medication, radiation
pure red cell aplasia
rare
N/N
inherited or acquired
normal leukocytes and platlet counts adn a marked decrease in marrow erythroblasts
megaloblastic macrocytic anemia
abnormal development of cell nuclei (DNA synthesis)a as cells mature in the bone marrow
marco/oval
cytoplasm more immature
nuclei large chromatin never becomes clumped
nucleoli are retained
hypersegmented neutrophils
vitamin B12 deficiency
meat and dairy
3-6 years worth in storage
requires for a single critical reaction during normal DNA synthesis
become deficient by defective production of intrinsic factor, nutritional deficiency, competition for dietary B12(bacteria), malabsorption
hypersegmented neutrophils
folate deficiency
leafy greens, liver, kidney, fruit, dairy
3 months worth in storage
required for three reactions that lead do DNA synthesis(involving amino acid synthesis- purines and pyrimidines)
folate needs B12 to work
become deficient by short supply, impaired absorption, increased need, impaired utilization(alcohol), excessive loss
hypersegmented neutrophils
pernicious anemia
lack of intrinsic factor
ineffective erythropoiesis
increased erythrocyte precursors in the bone marrow but decreased release into the peripheral blood.
decreased reticulocytes
ineffective granulopoiesis
abnormal formation of leukocytes
ineffective thrombosis
abnormal formation of plt with decreased plt life span
iron deficient anemia
body iron stores are inadequate to preserve homeostasis
caused by increased demand, inadequate intake, chronic blood loss
anemia of the chronic disease
destructing faster than the bone marrow can produce
caused by a chronic disease, infection, inflammation, malignancy
iron is trapped in the macrophages
unable to compensate
sideroblastic anemia
accumulation of excess iron from increased iron storage in macrophages in various tissue
hereditary =rare
acquired - most common lead poisoning
lead injures the RBC membrane and blocks at least three enzymes in the heme synthesis pathway (slows down the breakdown of RNA= basophilic stippling)
thal
inherited
defect in the production of alpha and beta globin chains
alpha thal
defect in the synthesis of alpha chain
silent carrier 1 gene deletion
alpha thal minor- 2 gene deletion
HbH disease- 3 gene deletion
Barts hydrops fetalis 4 gene deletion