Table 1.

Clinical definition required to confirm identified complications.

Complications	Definition
Extramedullary hematopoiesis (EMH)	Physical or radiologic evidence of extramedullary hematopoietic foci with or without symptoms
Pulmonary hypertension (PHT)	Systolic pulmonary artery pressure > 35 mmHg, which corresponds to a tricuspid regurgitant velocity on Doppler echocardiography of > 2.8 m/s plus exertional dyspnea without evidence of left heart disease
Thrombosis	Compression ultrasonography, contrast venography, or angiography evidence of thrombus
Cardiomyopathy	Echocardiographic, electrodiagnostic, or radiologic evidence of pathological change of myocardium such as hypertrophy, dilatation, or restriction
Cholelithiasis	Radiologic evidence of gallbladder stones
Abnormal liver function	ALT > 50 U/L
Pseudoxanthoma elasticum (PXE)	Histopathologic evidence of pathological change in elastic fibers to inelastic tissue
Leg ulcers	Ischemic or necrotic skin lesion on the lower extremity by general visual inspection
Osteoporosis (OP)	Bone densitometry T score < 2.5 SD
Abnormal plasma glucose	Fasting plasma glucose > 110 mg/dL at least one time
Hypothyroidism	TSH > 4.7 U/L and a free T4 > 0.8 ng/dL
Hypogonadism	Females: > 13 years, not yet Tanner B2 (i.e., prepubertal breast development) or > 14 years requiring estrogen replacement therapy or > 15 years with primary amenorrhea; males: > 14 years, not yet Tanner G2 (i.e., prepubertal genital development) or on androgen replacement therapy or > 17 years, not yet Tanner G4 (i.e., midpubertal genital development)
Iron overload	Maximum ferritin level >800 ng/mL with or without radiologic or histopathologic evidence

Adapted from [8].