HbH Disease (3) Flashcards
1
Q
What is produced in high amounts to compensate for low HbA / HbA2
A
HbH + excess B globin
2
Q
Blood Film
A
Target Cells
H bodies
Hypochromia
3
Q
Tests done for confirmation
A
Immunochromatographic strip test (anti-HbH AB)
DNA test to see whats deleted (HbF no present)
4
Q
Clinical Presentation
A
Hepatosplenomegoly
Ineffective haemopoeisis
5
Q
Treatment
A
genearlly not required
AB to prevent infection (strepto pneumoniae)
Regular Folic acid if pregnant
Splenectomy / BT
