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Flashcards in Hemolytic Anemia 1 Deck (51):
1

lifespan of RBC

120d

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sites of extra medullary hematopoiesis

spleen
liver
lymph nodes

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hemoglobinopathy

genetic mutation for abnormal globins

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thalssemia

mutation decreasing globin synthesis

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hemolysis

RBC destruction resulting in release of Hb

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signs of hemolytic anemia

elevated erythropoietin
increased erythropoiesis
increased Hb catabolites (bilirubin)

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intravascular hemolysis

mechanical injury or toxic injury resulting in complement fixation from antibody/antigen reactions

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consequences of intravascular hemolysis

anemia
hemoglo binemia
hemoglobinuria
hemosiderinuria
jaundice

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anemia

reception in total RBC cells leading to tissue hypoxia and reduced oxygen caring capacity

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hemoglobinemia

free Hb in blood

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hemoglobinuria

free Hb in urine

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hemosiderinuria

hemosiderin binds iron from hemoglobin in urine and is excreted in urine

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best marker for intravascular hemolysis

hemosiderinuria

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jaundice

yellowing of skin from elevated bilirubin

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extravascular hemolysis

less severe, more common
less deformable RBCs that can't get through spleen and get stuck

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consequences of extravascular hemolysis

anemia
splenomegaly
jaundice

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cell counts/ indices that indicate hemolysis

increased reticulocyte count
reduced MCV
elevated MCHC
increased free Hb
decreased haptoglobin
increased LDH
increased free Hb

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haptoglobin

binds free Hb
reduced in hemolysis

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osmotic fragility test

expose abc to decreasing salt/increasing water concentration and watch when lysis occurs

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osmotic fragility test in hemolysis

decreased in membrane defects

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conditions with membrane defects

hereditary spherocytosis
hereditary elliptocytosis

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protein electrophoresis

separating RBC membrane proteins by size with predictable migration pattern

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protein electrophoresis in hemolysis

different band patterns in membrane defects

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sickling test

used for sickle cell disease
mix blood with oxygen consuming agent and watch for sickling

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Hemoglobin electrophoresis

different band pattern from Hb with a change in the protein (sickle cell)

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hereditary spherocytosis genetics

autosomal dominant
heterogeneous
northern europeans
same mutations within familiese

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etiology of hereditary spherocytosis

membrane defect from reading frame shifts of stop codons resulting in mutated ankyrin, spectrum, band 4.2

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pathophysiology of hereditary spherocytosis

fragile less deformable RBC
little pieces of membrane are lost --> forms a sphere

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diagnostic findings associated with membrane defects

low/normal MCV
elevated MCHC
more sensitive to osmotic lysis
missing bands in protein electrophoresis

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complications of membrane defect

abc get trapped in the spleen
10-20d life

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treatment for membrane defects

splectomy

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hereditary elliptocytosis genetics

autosomal dominant
equatorial africa (resistance to malaria)

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etiology of hereditary elliptocytosis

alpha spectrin mutation

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G6PD deficiency genetics

x linked recessive
G6PDa- blacks
G6PD mediterranean- middle east

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pathophysiology of G6PD deficiency

increased susceptibility to oxidative injury
missing G6PD needed to convert NADP to NADPH which reduces glutathione so it can remove H2O2

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diagnostics for G6PD deficiency

Heinz bodies
bite cells

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heinz bodies

denatured Hg clumps inside RBC

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bite cells

RBC with bites taken out by splenic macrophages
attempted removal of heinz bodies

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consequences of G6PD deficiency

acute hemolysis when exposed to oxidative compounds

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oxidative compounds

certain drugs (antimalarials, sulfonamides, nitrofurantoin)
fava beans
viral/bacterial infections

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treatment for g6PD deficiency

avoid oxidative stressses

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sickle cell disease genetics

8% of blacks
homozygous

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sickle cell disease etiology

point mutation in beta global gene that switches glutamate for valine

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sickle cell disease pathogenesis

deoxygenation triggers polymerization reaction that forms rigid polymers that damage the membrane
may be reversible with oxygenation

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sickle cell disease diagnosis

clinical presentation
family history
blood smear- drepanocytes, anisocytosis, poikilocytosis
sickling test
hemoglobin electrophoresis
dna testing

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sickle cell disease acute complications

acute microvascular occlusion and tissue damage
bone, lung, spleen- infarction, sequestration, brain, penis
susceptibility to encapsulated organisms

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sickle cell disease chronic complications

anemia
cholelithiasis
aplastic crisis

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sickle cell treatment

reduce HbS
blood transfusion
dehydration
increase HbF
reduce inflammation
maintain intracellular pH

49

complications of hemolytic disorders

bone marrow expansion
iron overload with chronic transfusion
gallstones
aplastic crisis

50

aplastic crisis

parvovirus infects erythroid precursor cells
low Hb and Hct
giant pronormoblasts in bone marrow

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infectious causes of hemolysis

parvovirus b19
babes microti
falciparum malaria
clostridium welchii