0521779407-20 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:22
Thalassemia 1417➣Diagnosis: serum ferritin, left ventricular ejection fraction (LVEF)
and myocardial T2* magnetic resonance (MR) measurements,
liver biopsy
➣Toxicities: cardiac, liver, endocrine (hypogonadism, hypothy-
roidism, diabetes, hypoparathyroidism)
➣Treatment: Desferrioxamine, given as continuous subcutaneous
or intravenous infusion (monitor for local skin reaction)
ICL670, given orally (monitor skin rash, renal effects)
iron chelation may be associated with an increased risk of
Yersinia enterocoliticainfection, auditory and visual losses
■Infection (particularly hepatitis B, C and HIV)
■Allosensitization
■Transfusion reaction (acute or delayed)Splenectomy
■Indications: increased or excessive transfusional requirements
➣risk of splenic rupture hypersplenism
■Complications: increased risk of infection with encapsulated bac-
teria (immunize patient against pneumococcus,Haemophilus
influenzae, and meningococcus prior to splenectomy, prophylactic
penicillin)Bone Marrow Transplantation
■Only curative therapy.
■Guidelines regarding this treatment still under investigation.follow-up
■Patients on chronic transfusions/iron chelation
➣Monthly: CBC
➣Every 3 months: serum ferritin, glucose, creatinine, iron, TIBC,
alkaline phosphatase, AST, ALT, LDH
➣Yearly: echocardiogram and EKG
Viral serologies (hepatitis B and C, HIV)
Endocrine evaluation
Bone age (in children)
Ophthalmology exam
Audiology exam
Cardiac T2* MR, LVEF measurements
Liver biopsy if iron overload
■Patients with mild-moderate beta thalassemia intermedia may
develop complications with age (bone deformity, osteoporosis, leg