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Flashcards in Hemolytic anemia Deck (24)
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1

Sickle cell anemia-Eti

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

2

Sickle cell anemia- Sx

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,

3

Sickle cell anemia- Dx

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

4

Sickle cell anemia- Tx

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

5

Sickle cell trait- Eti

Heterozygous hemoglobin- type AS

6

Sickle cell trait- Sx

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

7

Sickle cell trait- Dx

Normal CBC & blood smear. Electrophoresis shows Heme S

8

Sickle cell trait- Tx

Genetic counseling

9

Paroxysmal nocturnal hemoglobinuria- Eti

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

10

Paroxysmal nocturnal hemoglobinuria- Sx

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

11

Paroxysmal nocturnal hemoglobinuria- Dx

Flow cytometry

12

Paroxysmal nocturnal hemoglobinuria- Tx

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

13

Hereditary spherocytosis- Eti

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

14

Hereditary spherocytosis- Sx

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

15

Hereditary spherocytosis- Dx

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

16

Hereditary spherocytosis- Tx

Folic acid and splenectomy

17

G6PD Deficiency- Eti

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

18

G6PD Deficiency- Sx

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

19

G6PD Deficiency- Dx

Heinz bodies, G6PD enzyme assay low

20

G6PD Deficiency- Tx

Avoid oxidant drugs

21

Autoimmune hemolytic anemia- Eti

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

22

Autoimmune hemolytic anemia- Sx

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

23

Autoimmune hemolytic anemia- Dx

Positive coombs test,

24

Autoimmune hemolytic anemia-Tx

Prednisone