Table 3.3.

Standard care and screening guidelines for thalassemia intermedia and major

Assessment	Ages	Frequency	Comments
Bone mineral density	≥10	Yearly	DEXA scan or quantitative CT
Tanner stage	10–20	Yearly	Perform yearly starting at age 10 and continuing until breast or gonadal Tanner Stage 5 or age 20.
Liver iron content	All	Yearly	MRI method (R2, T2*) or liver biopsy
Cardiac T2*	≥ 10	Yearly (see comment)	To be performed when available for patients with biochemical evidence of iron overload or age 10 years or older
Cardiac studies	≥ 10	Yearly	Echocardiography and/or cardiac function by MRI; indicate the need to assess for pulmonary hypertension when ordering an echocardiogram
CBC plus differential	All	Yearly (minimum)	If transfused, preceding each transfusion
Blood chemistries	All	Yearly	BUN, creatinine, calcium, magnesium, phosphorus, and zinc
LFTs	All	Yearly	ALT, AST, total bilirubin, albumin
Ferritin	All	Yearly (minimum)	If transfused, preceding each transfusion
HIV	All	Yearly	Only for transfused participants starting at the first transfusion
Hepatitis testing	All	Yearly	Only for transfused participants starting at the first transfusion. HBV serology. HCV serology and PCR
Plasma ascorbate	All	Yearly	12 h fast
Serum glucose	≥10	Yearly	12 h fast
Endocrine panel I	≥6	Yearly	TSH, free T4, parathyroid hormone, 25-hydroxy vitamin D, and 1,25 dihydroxy vitamin D levels
Endocrine panel II	≥10	Yearly	Testosterone (males only), FSH and LH (males and females), and estradiol (females only)
Ophthalmology and Audiology testing	Children and Adults	Yearly	All patients undergoing chelation. Auditory testing necessary for those on desferroxamine