hemolytic anemias (extrinsic) chapter 21 objectives + chapter questions

intrinsic defects leading to increased erythrocyte destruction

hereditary spherocytosis disrupts ___ interactions

vertical

clinical findings for hereditary spherocytosis

increased osmotic fragility, increased MCHC, increased RDW

hereditary elliptocytosis disrupts ___ interactions

horizontal

hereditary elliptocytosis is a(n)

immune-hemolytic anemia

paroxysmal nocturnal hemoglobinuria is caused by the lack of which GPI proteins?

CD55 & CD59

G6PD deficiency can be triggered by

oxidative stress, ingestion of fava beans

what does G6PD normally do?

detoxifies H2O2, protects hemoglobin

what morphology is seen in G6PD deficiency

heinz bodies

pyruvate kinase is caused by

a genetic mutation

what does pyruvate kinase normally do?

forms ATP

pyruvate kinase RBC morphology

burr cells

G6PD deficiency & pyruvate kinase are both

enzymopathies

which is NOT an indicator of hemolysis

decreased hemoglobin

in HS, a characteristic abnormality in the CBC results is

increased MCHC

the altered shape of the spherocyte in HS is due to

a mutated RBC membrane protein affecting vertical protein interactions

which one of the following sets of results is consistent with HS?

increased osmotic fragility, negative DAT result

the RBCs in HE are abnormally shaped & have unstable cell membranes as a result of

defects in horizontal membrane protein interactions

the peripheral blood film for patients with mild hereditary elliptocytosis (HE) is characterized by

elliptical RBCs

acanthocytes are found in association with

abetalipoproteinemia

the most common manifestation of G6PD deficiency is

acute hemolytic anemia caused by drug exposure or infections

a patient experiences an episode of acute intravascular hemolysis after taking primaquine for the first time. the physician syspects that the patent may have G6PD deficiency & orders an RBC G6PD assay 3 days after the hemolytic episode began. how will this affect the test result?

false increase in enzyme activity due to retics

the most common defect or deficiency in the anaerobic glycolytic pathway that causes chronic HNSHA

pyruvate kinase deficiency

which of the following lab tests would be best to confirm PNH?

flow cytometry