Hemolytic Anemia

Question 1

What is are Heinz Bodies?
Where do you find them?

Answer 1

Denatured Hb (supravital)
G6PD

Question 2

What are Howe-Jolly Bodies?
Where do you find them?

Answer 2

Nuclear Remnants
Splenectomy, Ineffective hematopeiesis

Question 3

What is Basophillic Stipling?
Where do you find them?

Answer 3

Residual RNA/Polysomes
Thalassemia, Lead, Enzymopathies, MDS

Question 4

What are Pappenheimer Bodies?
Where do you find them?

Answer 4

Iron inclusions
Sideroblastic anemia, Asplenia

Question 5

What are HbH inclusions?
Where do you find them?

Answer 5

Beta Tetramers (supravital)
Alpha Thalassemia (3 gene del)

Question 6

What are the AD mutations for Hereditary Spherocytosis

Answer 6

Vertical Mutations
Ankyrin - ANK1
Band3 - SLC4A1
Beta Spectrin - SPTB

Question 7

What are the AR mutations for Hereditary Spherocytosis

Answer 7

Vertical Mutations
Alpha Spectrin - SPTA1
Protein 4.2 - EPB42

Question 8

What are the mutationsin Hereditary Eliptocytosis

Answer 8

Horizontal Mutations
Spectrin - SPTA,SPTB
Band 3
Protein 4.2

Question 9

What is the mutation in Hereditary Pyropoikilocytosis (HPP)

Answer 9

AR
Spectrin - SPTA1

Question 10

Lab Findings for
Hydrocytosis

Answer 10

Increased MCV
Decreased MCHC
Na+ gain
RhaG gene

Question 11

Lab findings for Xerocytosis

Answer 11

Increased MCV & MCHC
Decreased Osmotic Fragility (opp HS)
K+ Loss
AD: PIEZO1, KCCN4
Very high thrombosis risk after splenectomy (avoid)

Question 12

Left Shifted Oxygen
Dissociation Curve

Answer 12

Oxygen “Latches on”
Everything “Lower”
CO2, Acid, 2,3DPG, Temp

Question 13

Right Shifted Oxygen
Dissociation Curve

Answer 13

Oxygen is Relinquished”
Everything “Rises”
CO2, Acid, 2,3DPG, Temp

Question 14

Unstable Hb

Answer 14

Koln - Most freq, left shift curve
Zurich - Incr CO affinity, hemolytic
Poole - unstablegamma chain
Indianapolis - too unstableto find in PB, acts like Thal Major, AD inheritance

Question 15

Most common type of
G6PD

Answer 15

Class 3
Activity 10-60%
t1/2 13 days
G6PD A - (African american)
Kaiping

Question 16

Which class of G6PD can have decreased WBC fuction

Answer 16

Class 1

Question 17

Pyruvate Kinase Mutation

Answer 17

PKLR

Question 18

PKD - O2 Dissociation Curve

Answer 18

Right Shift - Incr 2,3 DPG

Question 19

Glucose Phosphate Isomerase Deficiency

Answer 19

AR
Neuro Symptoms
Bland Smear

Question 20

Pyrimidine 5’ Nucleaotidase Deficiency

Answer 20

Neuro Symptoms
Basophilic Stipling
Dx - Incr pyrimidine in RBC, Molec Testing

Question 21

RBC Enzymopathies that cause
Myopathy

Answer 21

Phosphofructokinase
Aldolase
Phosphoglycerate Kinase (X linked)

Question 22

RBC Enzymopathies that cause
Neuro Symptoms

Answer 22

Glucose Phosphate Isomerase Def
Pyrimidine 5’ Nucleotidase Def
Triosephosphate Isomerase Def
Phosphoglycerate Kinase Def (X linked)
Adenylate Kinase Def

Question 23

Pathophys of PNH

Answer 23

Loss of CD55/59
PIGA mutations

Question 24

Toxins that target phospholipases

Answer 24

Clostridium
Brown Recluse/Snake Venom

Question 25

Where do you find DonathLandsteiner ab

Answer 25

Paroxysmal Cold Hemaglobinuria