Thalassemia

 Ethnic: Mediterranean, African-American, middle east and Asian

 -thalassemia
o major (homozygote) (Cooley’s anemia) severe, need treatment
o intermediate (hetro)
o minor (hetro)
o manifest after 1st year of life, when Hb F disappears.
 -thalassemia
o 4 genes for the -chain synthesis
o 1 gene deletion silent carrier.
o 2 genes deletion mild microcytic anemia.
o 3 genes deletion severe hemolytic anemia.
o 4 genes deletion hydrps fetalis
  GI absorption of iron and with multi-Tx  iron overload
hemochromatosis .
 treatment :
o Tx and folate in mild form
o Multi Tx and iron chelating agent in the severe form
 o Splenectomy
o BM Tx
 Anesthesia consideration:
A prominent maxilla due to extra-medullary hemopiosis  difficult airway
B PE, pulmonary HTN, and restrictive lung disease.
CV CHF, Arrhythmia due to iron toxicity , Pericarditis and pericardia effusion.
CNS spinal cord compression due to  the size of the vertebral bodies
GI-Endo iron overload damage to
 Pancreas DM
 Thyroid and parathyroids hypo
 Hepatic failure
Hema anemia and it’s severity , coagulopathy
MSK cortical thinning  easy # (position)