Hemolytic anemia Flashcards
3 general levels of diagnosing hemolytic anemia
Clinical suspicion
Screening lab tests
Precise identification of cause
5% or more increase in reticulocytes suggests what
Hemolysis of RBCs
Presence of blister cells and Heinz bodies suggests what
G6PD deficiency
Presence of Howell-Jolly bodies is seen in what two anemias
Sickle cell
Severe hemolytic anemia
Increased indirect bilirubin and LDH is seen in what category of anemias
Hemolytic anemias
Anemia that primarily involves phagocytosis by macrophages in the RE system
Extravascular hemolytic anemia
3 causes of intravascular hemolytic anemia
Mechanical injury
Complement fixation
Exogenous toxin
Inherited mutations affecting membrane proteins in RBCs, particularly spectrin and ankyrin
Hereditary spherocytosis
Inheritance of hereditary spherocytosis
Autosomal dominant
Possible complication in children with hereditary spherocytosis
Aplastic crisis after viral infection
Inheritance of G6PD deficiency
X-linked recessive
Reduced ability of RBC to protect against oxidative injury leading to hemolysis
G6PD deficiency
Heinz bodies and bite cells are seen in what anemia
G6PD deficiency
Inheritance of pyruvate kinase deficiency
Autosomal recessive
Mechanism of reduced clinical severity of pyruvate kinase deficiency
2,3-BPG is increased causing a right-shift of the O2 dissociation curve, increasing the release of O2 in the tissue
Echinocytes on smear is associated with what hemolytic anemia
Pyruvate kinase deficiency
Confirmatory test for pyruvate kinase deficiency
RBC enzyme assay
Sickle cell anemia mutation
Point mutation in 6th beta-globin gene from glutamate to valine results in HbS
93% A, 2% F and 5% A2 on Hb electrophoresis
Beta-thalassemia minor
90% F and 10% A2 on Hb electrophoresis
Beta-thalassemia major
Number of affected alpha-globin genes in alpha-thalassemia minor
2
Number of affected alpha-globin genes in HbH disease
3
Due to acquired mutations in PIG-A, an intermediary of GPI, on the X-chromosome
Paroxysmal nocturnal hemoglobinuria
