Hemolytic Anemia 1

Question 1

lifespan of RBC

Answer 1

120d

Question 2

sites of extra medullary hematopoiesis

Answer 2

spleen
liver
lymph nodes

Question 3

hemoglobinopathy

Answer 3

genetic mutation for abnormal globins

Question 4

thalssemia

Answer 4

mutation decreasing globin synthesis

Question 5

hemolysis

Answer 5

RBC destruction resulting in release of Hb

Question 6

signs of hemolytic anemia

Answer 6

elevated erythropoietin
increased erythropoiesis
increased Hb catabolites (bilirubin)

Question 7

intravascular hemolysis

Answer 7

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

Question 8

consequences of intravascular hemolysis

Answer 8

anemia
hemoglo binemia
hemoglobinuria
hemosiderinuria
jaundice

Question 9

anemia

Answer 9

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

Question 10

hemoglobinemia

Answer 10

free Hb in blood

Question 11

hemoglobinuria

Answer 11

free Hb in urine

Question 12

hemosiderinuria

Answer 12

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

Question 13

best marker for intravascular hemolysis

Answer 13

hemosiderinuria

Question 14

jaundice

Answer 14

yellowing of skin from elevated bilirubin

Question 15

extravascular hemolysis

Answer 15

less severe, more common

less deformable RBCs that can’t get through spleen and get stuck

Question 16

consequences of extravascular hemolysis

Answer 16

anemia
splenomegaly
jaundice

Question 17

cell counts/ indices that indicate hemolysis

Answer 17

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

Question 18

haptoglobin

Answer 18

binds free Hb

reduced in hemolysis

Question 19

osmotic fragility test

Answer 19

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

Question 20

osmotic fragility test in hemolysis

Answer 20

decreased in membrane defects

Question 21

conditions with membrane defects

Answer 21

hereditary spherocytosis

hereditary elliptocytosis

Question 22

protein electrophoresis

Answer 22

separating RBC membrane proteins by size with predictable migration pattern

Question 23

protein electrophoresis in hemolysis

Answer 23

different band patterns in membrane defects

Question 24

sickling test

Answer 24

used for sickle cell disease

mix blood with oxygen consuming agent and watch for sickling

Question 25

Hemoglobin electrophoresis

Answer 25

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

Question 26

hereditary spherocytosis genetics

Answer 26

autosomal dominant heterogeneous northern europeans same mutations within familiese

Question 27

etiology of hereditary spherocytosis

Answer 27

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

Question 28

pathophysiology of hereditary spherocytosis

Answer 28

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

Question 29

diagnostic findings associated with membrane defects

Answer 29

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

Question 30

complications of membrane defect

Answer 30

abc get trapped in the spleen | 10-20d life

Question 31

treatment for membrane defects

Answer 31

splectomy

Question 32

hereditary elliptocytosis genetics

Answer 32

``` autosomal dominant equatorial africa (resistance to malaria) ```

Question 33

etiology of hereditary elliptocytosis

Answer 33

alpha spectrin mutation

Question 34

G6PD deficiency genetics

Answer 34

x linked recessive G6PDa- blacks G6PD mediterranean- middle east

Question 35

pathophysiology of G6PD deficiency

Answer 35

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

Question 36

diagnostics for G6PD deficiency

Answer 36

Heinz bodies | bite cells

Question 37

heinz bodies

Answer 37

denatured Hg clumps inside RBC

Question 38

bite cells

Answer 38

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

Question 39

consequences of G6PD deficiency

Answer 39

acute hemolysis when exposed to oxidative compounds

Question 40

oxidative compounds

Answer 40

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

Question 41

treatment for g6PD deficiency

Answer 41

avoid oxidative stressses

Question 42

sickle cell disease genetics

Answer 42

8% of blacks | homozygous

Question 43

sickle cell disease etiology

Answer 43

point mutation in beta global gene that switches glutamate for valine

Question 44

sickle cell disease pathogenesis

Answer 44

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

Question 45

sickle cell disease diagnosis

Answer 45

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

Question 46

sickle cell disease acute complications

Answer 46

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

Question 47

sickle cell disease chronic complications

Answer 47

anemia cholelithiasis aplastic crisis

Question 48

sickle cell treatment

Answer 48

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

Question 49

complications of hemolytic disorders

Answer 49

bone marrow expansion iron overload with chronic transfusion gallstones aplastic crisis

Question 50

aplastic crisis

Answer 50

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

Question 51

infectious causes of hemolysis

Answer 51

parvovirus b19 babes microti falciparum malaria clostridium welchii