Hematology 3 - Hemolytic Anemia, G6PD Deficiency, Sickle Cell Anemia

Question 1

Define hemolysis

Answer 1

Life of RBC shortened below 100 days

Question 2

2 types of Hemolytic Anemia?

Answer 2

1. Intrinsic- defect intrinsic to RBC

2. Extrinsic-external factors causing anemia

Question 3

What types of things cause intravascular hemolysis?

Answer 3

Mechanical heart valves, transfusion rxn, toxins (snake venom), infx, oxidant stress, complement mediated hemolysis

Question 4

What types of things cause extravascular hemolysis?

Answer 4

Membranopathy, enzymopathy, hemoglobinopathy (ex thalassemia) immune mediated

Question 5

Schistocytes represent hemolysis where?

Answer 5

Intravascular

Question 6

Shereocytes, spur cells, bite cells represent hemolysis where?

Answer 6

Extravascular

Question 7

The “-opathies” are which Hemolytic Anemia class?

Answer 7

Intrinsic

Question 8

Immune, burns, valve hemolysis are which Hemolytic Anemia class?

Answer 8

Extrinsic

Question 9

Hemolytic Anemia signs and symptoms?

Answer 9

Jaundice, yellow discoloration, Scleral icterus, abdominal pain, dark urine, thrombus, petechiae and purpura. In addition to normal anemia Sx.

Question 10

Hemolytic Anemia PE findings?

Answer 10

Normal anemia and jaundice, Scleral icterus, dark/discolored urine, splenomegaly, petechiae, purpura, abd tender, eccymosis

Question 11

What is the cause of dark/discolored urine in Hemolytic Anemia?

Answer 11

Hyperbilirubinemia

Question 12

What happens to RBCs in Hemolytic Anemia?

Answer 12

Breakdown

Question 13

What are the 2 parts of heme?

Answer 13

Iron

Porphyrin ring

Question 14

Where does Bilirubin come from?

Answer 14

Breakdown of heme portion of RBC (porphyrin ring)

Question 15

What is hallmark lab finding for Hemolytic Anemia?

Answer 15

Elevated reticulocyte count with stable or falling Hgb, OR seeing abnormal cells on smears (shictocytes and spherocytes)

Question 16

What binds free hemoglobin?

Answer 16

Hepatocytes

Question 17

Hemolytic Anemia lab studies

Answer 17

Elevated reticulocytosis w/stable or falling Hgb
↓Haptoglobin
Urine hemosiderin – positive
↑ Indirect bilirubin – elevated
↑↑ Serum LD
↑ Methemalbuminemia – plasma methemalbumin
•↑ Hemoglobinemia – free plasma hemoglobin or free urine hemoglobin

Question 18

Positive Urine hemosiderin more common where?

Answer 18

Intravascular

Question 19

↑ Indirect bilirubin more common where?

Answer 19

Extravascular

Question 20

Direct Coombs looks at what?

Answer 20

Tests for RBCs coated with antibodies or complement

Question 21

Indirect Coombs looks at what?

Answer 21

Tests for antibodies against RBCs in the serum

Question 22

What does Heinz Body represent?

Answer 22

Denatured hemoglobin. Unstable hemoglobin

variants, G6PD, enzyme defect

Question 23

What is the common hemolytic anemia due to cell membrane defect?

Answer 23

Hereditary Spherocytosis

Question 24

What causes Hereditary Spherocytosis?

Answer 24

DNA mutation causing membranopathy

Question 25

What shapes be seen on peripheral smear in Hereditary Spherocytosis?

Answer 25

Spherocytes

Question 26

Hereditary Spherocytosis Direct Coombs test positive or negative?

Answer 26

Negative. Not an antibody issue.

Question 27

How is the diagnosis of Hereditary Spherocytosis often made?

Answer 27

Clinical grounds

Question 28

Tx for Hereditary Spherocytosis?

Answer 28

Supportive. Folic acid supplements. Transfuse as needed.

Question 29

When to consider splenectomy for Hereditary Spherocytosis?

Answer 29

If severe symptoms