Hemolytic anemia

Question 1

Sickle cell anemia-Eti

Answer 1

Hereditary recessive disorder in blacks-8% carriers. Sickling of RBCs due to aa substitution on beta hemoglobin. Onset first year of life

Question 2

Sickle cell anemia- Sx

Answer 2

Jaundice, gallstones, splenomegaly, poor healing ulcers over tibia. Acute pain in bones and chest with low grade fever. Repeated vaso-occlusion affects: Priapism & strokes, heart (cardiomegaly, murmurs,) lungs, ischemic necrosis of bone,

Question 3

Sickle cell anemia- Dx

Answer 3

Elevated bilirubin, irreversibly sickles cells 5-50% of RBCs, Howell-Jolly bodies & target cells. Elevated WBC. Hemoglobin electrophoresis- homozygous S

Question 4

Sickle cell anemia- Tx

Answer 4

Meds: Folic acid, hydroxyurea. Procedures- genetic testing, supportive care

Question 5

Sickle cell trait- Eti

Answer 5

Heterozygous hemoglobin- type AS

Question 6

Sickle cell trait- Sx

Answer 6

Asymptomatic, actue vaso-occlusion under extreme conditions, urine concentration

Question 7

Sickle cell trait- Dx

Answer 7

Normal CBC & blood smear. Electrophoresis shows Heme S

Question 8

Sickle cell trait- Tx

Answer 8

Genetic counseling

Question 9

Paroxysmal nocturnal hemoglobinuria- Eti

Answer 9

Acquired clonal hematopoietic stem cell disorder causing abnormal sensitivity of RBC to lysis by complement.

Question 10

Paroxysmal nocturnal hemoglobinuria- Sx

Answer 10

Hemoglobinuria (reddish brown) in AM, anemia, susceptibility to thrombosis

Question 11

Paroxysmal nocturnal hemoglobinuria- Dx

Answer 11

Flow cytometry

Question 12

Paroxysmal nocturnal hemoglobinuria- Tx

Answer 12

Severe- stem cell transplant, Eculizumab to reduce hemolysis, Iron replacement for deficiency

Question 13

Hereditary spherocytosis- Eti

Answer 13

Autosomal dominant- disorder of RBC membrane causing hemolysis by splenic macrophages

Question 14

Hereditary spherocytosis- Sx

Answer 14

Dx in childhood, palpable spleen, jaundice, gallstones, cholecystitis, severe aplastic anemia if folate deficienct

Question 15

Hereditary spherocytosis- Dx

Answer 15

Elevated MCHC- hyperchromic RBC, reticulocytosis, Coombs negative, spherocytes

Question 16

Hereditary spherocytosis- Tx

Answer 16

Folic acid and splenectomy

Question 17

G6PD Deficiency- Eti

Answer 17

Hereditary enzyme defect- Heinz bodies cause RBC membrane damage. Mediterranean

Question 18

G6PD Deficiency- Sx

Answer 18

Healthy until exposure to certain drugs that oxidize- dapsone, methylene blue. Hemolytic episode often self limited.

Question 19

G6PD Deficiency- Dx

Answer 19

Heinz bodies, G6PD enzyme assay low

Question 20

G6PD Deficiency- Tx

Answer 20

Avoid oxidant drugs

Question 21

Autoimmune hemolytic anemia- Eti

Answer 21

IgG autoantibody. Acquired disorder. Idiopathic in 50% cases- seen with SLE, CLL & lymphoma. Rapid onset and life threatening anemia.

Question 22

Autoimmune hemolytic anemia- Sx

Answer 22

Fatigue, dyspnea, angina pectoris, heart failure symptoms, jaundice, splenomegaly.

Question 23

Autoimmune hemolytic anemia- Dx

Answer 23

Positive coombs test,

Question 24

Autoimmune hemolytic anemia-Tx

Answer 24

Prednisone