Hemolytic anemia-Hereditary Spherocytosis

Question 1

What happens to haptaglobin during hemolytic anemia?

Answer 1

Decrease which will lead to hemoglobinuria

Question 2

Causes of hemolytic anemia?(intrinsic)

Answer 2

Intrinsic

Hemoglobinopathies:
Thalassemia (a&b)
SCD

RBC membrane defects:
Hereditary spherocytosis

Enzymopathy:
G6PD

Question 3

Lab of hemolytic

Answer 3

^ retics
^ rdw
^ indirect billrubin
^ LDH
^ urobilinogen

Decrease haptaglobin

Question 4

Hereditary spherocytosis
What type of inheritance is it?
And where is the defect?

Answer 4

AD

Protien membrane : spectrin , ankryin will lose biconcave

Question 5

What are the symptoms of HS?

Answer 5

Either asymptomatic

Or

Severe hemolytic
Jaundice
Pallor
Growth failure
Spleenomegaly
Gall stones

Question 6

What are the special findings in HS?

Answer 6

In CBC:
MCHC ***** ! ^
RDW
Mcv either normal or slightly decreased

Blood film:
Spherocytes : smaller than RBC’s , hyperchromic , no pallor
And other hemolytic +

Ema:
GOLD!
Decreased binding of fluoresence to membrane protiens in HS erythyrocytes

Osmotic +

At 0.68

Others:
Genetic
Acidfied glycrol lysis test

Question 7

What is the tt of HSP?

Answer 7

Supportive

Question 8

When is splenectomy indicated in HS?

Answer 8

Indicated:
In a child who is 5 yo, if he had severe for HS with chronic anemia / growth failure

Question 9

A 4 yo child came to the ER
With jaundice , spleenomegaly
He was noticed to have growth failure

What is your next step in management?

Answer 9

Partial splenectomy:
Cause he is 4 years
Presenting with GF , severe hemolytic