Hemolytic anemia-Hereditary Spherocytosis Flashcards

1
Q

What happens to haptaglobin during hemolytic anemia?

A

Decrease which will lead to hemoglobinuria

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2
Q

Causes of hemolytic anemia?(intrinsic)

A

Intrinsic

Hemoglobinopathies:
Thalassemia (a&b)
SCD

RBC membrane defects:
Hereditary spherocytosis

Enzymopathy:
G6PD

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3
Q

Lab of hemolytic

A

^ retics
^ rdw
^ indirect billrubin
^ LDH
^ urobilinogen

Decrease haptaglobin

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4
Q

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

A

AD

Protien membrane : spectrin , ankryin will lose biconcave

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5
Q

What are the symptoms of HS?

A

Either asymptomatic

Or

Severe hemolytic
Jaundice
Pallor
Growth failure
Spleenomegaly
Gall stones

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6
Q

What are the special findings in HS?

A

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

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7
Q

What is the tt of HSP?

A

Supportive

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8
Q

When is splenectomy indicated in HS?

A

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

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9
Q

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?

A

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

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