Table 5a.

Beta-Thalassemia Major: Recommended Surveillance

System/ConcernEvaluationFrequency
Hematologic CBCEvery 3-4 wks & w/illness
General Physical exam by physician familiar w/affected person & manifestations of β-thalassemia majorMonthly
Growth Assess growth & developmentEvery 3 mos throughout childhood
Cardiac Cardiologist visitAnnually starting in childhood
Myocardial MRI to assess iron overloadEvery 1-2 yrs starting either in childhood or when liver iron concentration is 1st ↑
Liver
  • Serum ALT
  • Serum ferritin 1
Every 3 mos
Liver MRI to assess iron overloadAnnually starting in childhood
  • Liver ultrasound
  • Serum alpha-fetoprotein for early detection of hepatocarcinoma
Annually in adults w/hepatitis C &/or iron overload
Endocrine Laboratory eval of thyroid, endocrine pancreas, parathyroid, adrenal, & pituitary function per endocrinologistAnnually starting in childhood
Eyes Ophthalmology examAnnually in those treated w/iron chelation
Hearing Audiology exam
Side effects of specific iron chelation therapies
  • Serum creatinine, creatinine clearance, &/or plasma cystatin C levels
  • Urine protein
In persons on deferasirox: prior to therapy, weekly in 1st mo after initiation or modification of therapy, & monthly thereafter
Laboratory assessment of hepatic functionIn persons on deferasirox: before initiation of treatment, every 2 wks during 1st mo, & monthly thereafter
Neutrophil countIn persons on deferiprone: weekly & in those w/signs/symptoms of infection
Venous thrombosis Assess for leg swelling or pain, unexplained dyspnea, pleuritic chest painAt each visit
Cholelithiasis Assess for postprandial abdominal pain
Orthopedic Bone densitometry to assess for osteoporosisEvery 1-5 yrs in adults

ALT = alanine transaminase; CBC = complete blood count

1.

Serum ferritin is not always reliable for evaluating iron burden because it is influenced by other factors, the most important being the extent of liver damage.

From: Beta-Thalassemia

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