Hemolytic Anemia

Question 1

What’s the mutation\Ab in TTP?

Answer 1

ADMAST 13

Question 2

What’s the role of ADMAST 13?

Answer 2

Cleaving VwF

Question 3

Name types of hemolytic anemia from most extrinsic to most intrinsic:

Answer 3

• AHA, MAHA, mechanical shearing, medications
• Spherocytosis, eliptocytosis
• PKD, G6PD
• Quantitative\Qualitative Hb-pathies

Question 4

How to differentiate between HUS & TTP:

Answer 4

Both (low Hb & platelet)

- HUS: post E-coli + diarrhea + renal failure.

Question 5

Differentiate between dark urine and tea colored urine in Hb-pathies:

Answer 5

• Dark: Hb urea (intravascular)

- Tea: Urobilinogen (extravascular in spleen)

Question 6

Clinical presentation in hemolytic anemia:

Answer 6

• jaundice & pallor
• Fatigue & tachycardia
• Splenomegaly & hepatomegaly

Question 7

What is the state of reticulocyte count in hemolytic anemia?

Answer 7

High

Question 8

What is the basic screening blood work-up for Hb-pathies:

Answer 8

• CBC (w\picture + Reticulocyte)
• LDH
• Urinalysis
• LFT (Total\ direct bilirubin)
• DCT
• EF
• osmotic fragility test

Question 9

What to do if you see target cell or sickle cell in blood smear?

Answer 9

Electrophoresis

Question 10

What test would be most supportive of your diagnosis if you suspect spherocytosis?

Answer 10

Osmotic fragility

Question 11

What are the types of AHA?

Answer 11

Warm, cold and paroxysmal cold

Question 12

Differentiate between warm, cold and paroxysmal cold AHA:

• AB
• temp of agglutination
• site of hemolysis
• presence of hemoglobinuria

Answer 12

• Warm: IgG, >36 extravascular, no.
• Cold: igM, intravascular, yes.
• PCH: IgG against P-antigen, extravascular

Question 13

Which of the following is a life-threatening condition

Warm, cold, paroxysmal cold AHA

Answer 13

Warm

Question 14

What is the clinical feature of warm AHA?

Answer 14

Sudden (pallor - jaundice - dark urine) + spleenomegaly

Question 15

What are the findings in warm AHA?

• CBC, blood film, Reticulocyte
• LDH & heptaglobin
• DCT
• Urinalysis
• bilirubin

Answer 15

• low Hb, normal indices, high reticulocyte, agglutinations & go to spleen for destruction
• high & low “in all hemolytic anemias”
• positive
• normal
• high indirect

Question 16

Main treatment of

• warm
• cold
• paroxysmal cold AHA
• hereditary spherocytosis

Answer 16

• Steroid
• supportive & plasmapheresis
• supportive + steroid
• folic acid

Question 17

What are other option to treat warm AHA:

Answer 17

IVIG, immunosuppression, anti-CD20 “retixumab”, splenectomy.

Question 18

What are the findings in warm AHA?

• CBC, blood film, Reticulocyte
• LDH & heptaglobin
• DCT
• Urinalysis

Answer 18

• low Hb, normal indices, high retic.
• High & low
• positive
• hemoglobinurea (tea colored)

Question 19

What do we do as a supportive care for cold AHA?

Answer 19

Warm patients

Question 20

In which group of population do we see PCH more often?

Answer 20

In children median age of 5 years.

Question 21

Name 2ndary causes of MAHA:

Answer 21

1- DIC
2- Drug induced
3- Autoimmune vasculopathy

Question 22

How does hemolysis occur in MAHA

Answer 22

RBCs are damaged when passing through obstructed\narrowed lumen

Question 23

Most severe type of defective cell membrane hemolytic anemia

Answer 23

Pyropoiklocytosis

Question 24

The following key words can give you hint about the diagnosis:

symptoms of hemolysis with viral infections & has history of neonatal jaundice.

Answer 24

Heriditary spherocytosis

Question 25

What is the CBC and film findings in hereditary spherocytosis?

Answer 25

High MCHC and spherocytes

Question 26

Is heriditary spherocytosis extravascular or intravascular hemolysis

Answer 26

Extra; thus splenomegaly

Question 27

What are the genes\their corresponding proteins affected in hereditary spherocytosis

Answer 27

- Ankyrin (band3) | - Spectrin (4.2)

Question 28

What is the shape of RBC in heriditary spherocytosis?

Answer 28

Round - small - less deformable.

Question 29

Differentiate between the inheritance of heriditary spherocytosis & elliptocytosis:

Answer 29

Both Autosomal dominant. - spherocytosis: more severe if recessive - elliptocytosis: more severe if dominant

Question 30

Other differentials for spherocytes in blood film:

Answer 30

Lead poisioning and ABO incompatiability (few cells)

Question 31

What is the screening test and the confirmatory test used to diagnose heriditary spherocytosis?

Answer 31

Screening: osmotic fragility test Confirmatory: Eosin-5-malemide “dye staining by flow cytometery”

Question 32

LFT findings in heriditary spherocytosis:

Answer 32

Increased total bilirubin - if gallstone: increased direct billirubin.

Question 33

Horizontal vs vertical defects will cause which type of cell membrane hemolytic anemias:

Answer 33

- horizontal: Elliptocytosis | - vertical: Spherocytosis

Question 34

Most commonly Heriditary eliptocytosis is inherited as:

Answer 34

Autosomal dominant

Question 35

If you were given a case of elliptocytosis, and you want to determine the severity, what CBC indices will reflect that

Answer 35

MCV

Question 36

Mutations in pyropoiklocytosis is in which gene

Answer 36

Spectrin

Question 37

Usually patients with pyropoiklocytosis have family members with:

Answer 37

heridetray eliptocytosis

Question 38

What is the mainstay treatment of hereditary pyropoiklocytosis during infancy?

Answer 38

Intermittent transfusion to avoid splenic crisis

Question 39

What is the role of splenectomy in heriditary pyropoiklocytosis?

Answer 39

Very significant, increase in Hb, however not totally eliminated

Question 40

What determines the severity of G6PD?

Answer 40

1-Quantity | 2- type of G6PD

Question 41

What is the mode of inheritance in G6pd

Answer 41

X-linked

Question 42

Color of urine in G6pd hemolysis is expected to be

Answer 42

Tea colored urine

Question 43

Name agents that can induce hemolysis in g6pd

Answer 43

Fava beans, sulfa medications, bacterim

Question 44

The only intravascular hemolysis mentioned in this lecture is

Answer 44

Cold AHA

Question 45

How to treat pyruvate kinase deficency?

Answer 45

Splenictomy, folic acid, transfusion