Thalassemia Flashcards
Thalassemia is what type of anemia?
Genetic
What does thalassemia mean?
A group of diseases involved decreased/abnormal production of normal hemoglobin causing decrease rbc production
Thalassemia is due to what?
And leads to?
The absent of globulin protein
Which leads to abnormal hgb synthesis
When we are missing that globulin protein and leads to abnormal hgb synthesis, what occurs?
Hemolysis
Thalassemia mainly occurs in which ethnic groups?
Near Mediterranean Sea
Equatorial near regions of Asia, Middle East and Africa
Thalassemia is autosomal recessive genetic based, which means what?
You could get one or two thalassemic genes
One thalassemic gene means ?
It’s also more ?
Two thalassemic gene means ?
It’s also more?
1 = minor ; mild form of disease
2 = major ; severe form of disease
Thalassemia minor
Clinical manifestations (7)
Frequently asymptomatic
Moderate anemia
- microcytosis ( small )
- hypochromia ( pale cells )
Mild splenomegaly
Bronzed skin color
Bone marrow hyperplasia
Do we need to treat thalassemia minor? Why?
No because our body adapts to the reduction of hemoglobins
Thalassemia major
Clinical manifestations (7)
And when do symptoms develop?
At what age ^
Life threatening
Physical & mental growth deficits
Pale
Jaundiced
Splenomegaly ( large spleen )
Hepatmegaly ( large liver )
Cardiomyopathy ( large heart )
Age 2
For thalassemia major
Why is splenomegaly & hepatomegaly
And cardiomyopathy’s enlarged??
Splenomegaly & hepatomegaly enlarged due to trying to store that iron
Cardiomyopathy for the heart overworking
What is the pathophysiology for thalassemia Major?
What happens in a normal person?
So what happens ?
And you get what?
(4)
Your bone marrow over grows to try to compensate for the fact that you don’t have enough hemoglobin
Your red blood cell production is stimulated when you have decrease oxygen carrying capacity of your blood; then your body tells your bone marrow to step up and make more red blood cells; hemoglobin
So when you have thalassemia majored your bone marrow is doing this stepping up to make more hemoglobin but they make immature erythroid and they die
Which leads to chronic bone marrow hyperplasia
( overgrowth of the bones )
Thalassemia major
clinical manifestations (4)
Cardiac complications due to iron overload
Pulmonary disease
Hypertension
Thrombosis
Is thalassemia an iron deficiency anemia?
No
Thalassemia is a problem of?
Production of hemoglobin
So the reason why thalassemia major is not an iron problem is because have a problem with making hemoglobin. How does this relate to one another?
Iron is a building block to make hemoglobin, so we aren’t using that iron and we get iron overload and causes our cardiac complications ; like lung disease and hypertension
Is there any medications or diet therapy for thalassemia? For either major or minor?
No
How do you help treat thalassemia major ?
What are some nursing intervention? (5)
Blood transfusions with clearing agents that bind to iron to reduce iron overloading
Ascorbic acid( vitamin C ) with chelation in increase urine excretion of iron
Avoid ascorbic acid without chelation
Will increase iron absorption
Goal is Hgb of 10
Splenectomy
What is the only cure for thalassemia?
Hematopoietic stem cell transplantation ( HSCT )
We also want to be careful when doing a splenectomy on our Thalassemia major patients that?
They will have a higher Risk of infections because the spleen home to lymph tissues
( decrease immune function )
