Hemolytic Anemia

Question 1

What are common signs in hemolytic anemia?

Answer 1

Jaundice, pallor, Chills, Fever, glossitis, ulcers, aplastic crisis. Kidney Failure can happen bc you can’t clear Hb fast enough and it is toxic to the kidneys.

Question 2

What is the formula for MCV?

Answer 2

(Hematocrit divided by RBC count) X 10fL

Question 3

What is the formula for MCH?

Answer 3

(HGB divided by RBC count) X 10 PG

Question 4

What is the formula for MCHC?

Answer 4

(HGB divided by Hematocrit) X 100%

Question 5

Steps to evaluate Hemolysis (8)

Answer 5

1) Peripheral Smear
2) Retic count
3) Bilirubin
4) LDH
5) Plasma Hemoglobin
6) Urine Hemoglobin
7) Urine Hemosiderin
8) Haptoglobin

Question 6

What do the presence of microspherocytes indicate?

Answer 6

Loss of membrane

Question 7

What are Schistocytes? How can you get them?

Answer 7

Fragmented RBCs

Any disease that leads to fibrous deposition on vessel walls

Question 8

Alloimmunity is a risk of transfusion in Hemolytic Anemia. What type of cells would be present if this were to happen?

Answer 8

Bite cells

Question 9

Schistocytes can be caused by what?

Answer 9

DIC (fibrin strands damage RBCs) and mechanical valves (like the example in class)

Question 10

What causes TTP? (Thrombotic Thrombocytopenia Purpura)

Answer 10

A defect of certain Matrix Metallo-proteinases (MMPs)

Question 11

True or False

Dark plasma is better than lighter plasma

Answer 11

False- the darker the plasma is the worse

Question 12

What test do you use for Autoimmune Hemolytic Anemias?

Answer 12

DAT- Direct Coombs test

Question 13

Why are some anemias missed on DAT tests?

Answer 13

The human body is more sensitive to IgG than the test so about 5% are missed

Question 14

What do you see in regards to histology of cold acting antibodies?

Answer 14

They show massive agglutination

Question 15

What is March Hemoglobinuria?

Answer 15

Breaking of RBCs by extreme exertion on feet, example is foot soldiers. Blood seen in urine.

Question 16

What do you do when you see a brown recluse spider or a black widow spider?

Answer 16

Get a spider cookie and wait til it takes the bait then hit it with your spider smashing shoe. If all else fails, run.

Question 17

What is a normal RBC lifespan?

Answer 17

120 days

Question 18

When RBCs are broken down, what is released?

Answer 18

Iron (recirculated via transferrin)
Protoporphyrin (broken down into bilirubin which is converted to other shit)
Globin (broken down into amino acids used for protein synth)

Question 19

What are haptoglobins?

Answer 19

Proteins present in normal plasma that bind hemoglobins and then the complex can be removed by the RE system

Question 20

What is an aplastic crisis? Often follows?

Answer 20

Abnormal decrease in retic count and sudden increase in anemia

Parvovirus infection

Question 21

In his seasonal hemolysis due to cold agglutinin syndrome slide, What is inversely proportional to environmental temperature?

Answer 21

LDH

Question 22

In cold autoimmune hemolytic anemia, what is the Ig type? In warm?

Answer 22

IgM

IgG

Question 23

Can you get necrotizing hemolytic anemia from a brown recluse spider bite?

Answer 23

You betcha

Question 24

In hemolytic anemia what are haptoglobin levels? Why?

Answer 24

Low, bc they are binding up excess Hg (Hg increases as RBC are lysed) and removed by RE system

Question 25

What's going on in hereditary spherocytosis?

Answer 25

Defects in membrane proteins (spectrin, ankyrin, protein 4.2) leads to LOSS of membrane and formation of spherocytes (spherical RBCs) that can't pass through splenic microcirculation leading to splenomegaly and anemia

Question 26

What's going on in G6PD deficiency?

Answer 26

Can't produce NADPH so can't reduce glutathione so can't protect against oxidative stress-->acute hemolytic anemia

Question 27

What kind of cells do you see in G6PD def?

Answer 27

Bite cells due to formation of Heinz bodies and subsequent removal of them by the spleen

Question 28

What is the inheritance pattern of G6PD def?

Answer 28

X linked

Question 29

In hemolytic anemia what are haptoglobin levels? Why?

Answer 29

Low, bc they are binding up excess Hg (Hg increases as RBC are lysed) and removed by RE system

Question 30

What's going on in hereditary spherocytosis?

Answer 30

Defects in membrane proteins (spectrin, ankyrin, protein 4.2) leads to LOSS of membrane and formation of spherocytes (spherical RBCs) that can't pass through splenic microcirculation leading to splenomegaly and anemia

Question 31

What's going on in G6PD deficiency?

Answer 31

Can't produce NADPH so can't reduce glutathione so can't protect against oxidative stress-->acute hemolytic anemia

Question 32

What kind of cells do you see in G6PD def?

Answer 32

Bite cells due to formation of Heinz bodies and subsequent removal of them by the spleen

Question 33

What is the inheritance pattern of G6PD def?

Answer 33

X linked

Question 34

What's going on in pryruvate kinase deficiency?

Answer 34

Decreases ATP formation leads to rigid RBCs and lysis (due to swelling of cell bc no na/k pump)

Question 35

What happens to the oxygen dissociation curve in pryruvate kinase deficiency? Why?

Answer 35

Right shift due to increased intracellular 2,3-DPG

Question 36

What are autoimmune hemolytic anemias? What test is used in dx? Two types...?

Answer 36

Ab production by body against own RBCs; direct Coombs; warm (Ab reacts at 37 Celsius) and cold (Ab reacts at 4 Celsius)

Question 37

In warm AIHA, what kind of hemolysis do you see? In cold?

Answer 37

Extra vascular (phagocytosis by macrophages and removal by RES in spleen) and intravascular (complement lysis) Intravascular only (complement MAC only)

Question 38

What's going on in paroxysmal nocturnal hemoglobinuria?

Answer 38

Acquired clonal disorder of marrow stem cells (defective pigA gene)-->defective GPI anchor-->lack of DAF and CD59-->complement (intravascular) lysis

Question 39

How do you dx PNH?

Answer 39

Flow cytometry

Question 40

What is the tx for PNH? MOA?

Answer 40

Ecluzimab | Mab against complement c5