Hemolytic anemia-Hereditary Spherocytosis Flashcards
What happens to haptaglobin during hemolytic anemia?
Decrease which will lead to hemoglobinuria
Causes of hemolytic anemia?(intrinsic)
Intrinsic
Hemoglobinopathies:
Thalassemia (a&b)
SCD
RBC membrane defects:
Hereditary spherocytosis
Enzymopathy:
G6PD
Lab of hemolytic
^ retics
^ rdw
^ indirect billrubin
^ LDH
^ urobilinogen
Decrease haptaglobin
Hereditary spherocytosis
What type of inheritance is it?
And where is the defect?
AD
Protien membrane : spectrin , ankryin will lose biconcave
What are the symptoms of HS?
Either asymptomatic
Or
Severe hemolytic
Jaundice
Pallor
Growth failure
Spleenomegaly
Gall stones
What are the special findings in HS?
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
What is the tt of HSP?
Supportive
When is splenectomy indicated in HS?
Indicated:
In a child who is 5 yo, if he had severe for HS with chronic anemia / growth failure
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?
Partial splenectomy:
Cause he is 4 years
Presenting with GF , severe hemolytic