Table 1

Clinical definitions required to confirm identified complications

ComplicationDefinition
EMH Radiologic evidence of extramedullary hematopoietic foci with or without symptoms 
PHT Systolic pulmonary artery pressure > 35 mm Hg, which corresponds to a tricuspid regurgitant velocity on Doppler echocardiography of > 2.8 m/s18  plus exertional dyspnea without evidence of left heart disease 
HF Modified Framingham criteria19  
Thrombosis Compression ultrasonography, contrast venography, or angiography evidence of thrombus 
Cholelithiasis Ultrasonographic evidence of gallbladder stones 
Abnormal liver function ALT > 50 U/L 
Leg ulcers Ischemic or necrotic skin lesion on the lower extremity by general visual inspection 
DM Fasting blood sugar ≥ 126 mg/dL, or 2-hour postprandial blood sugar ≥ 200 mg/dL, or symptoms of hyperglycemia and a casual (random) plasma glucose ≥ 200 mg/dL20  
Hypothyroidism TSH > 4.7 μU/L and a free T4 < 0.8 ng/dL21  
Osteoporosis Bone densitometry T score: 2.5 SD22  
Hypogonadism Females: > 13 y, not yet Tanner B2 (ie, prepubertal breast development) or > 14 y requiring estrogen replacement therapy or > 15 y with primary amenorrhea; males: > 14 y, not yet Tanner G2 (ie, prepubertal genital development) or on androgen replacement therapy or > 17 y, not yet Tanner G4 (ie, midpubertal genital development)23  
ComplicationDefinition
EMH Radiologic evidence of extramedullary hematopoietic foci with or without symptoms 
PHT Systolic pulmonary artery pressure > 35 mm Hg, which corresponds to a tricuspid regurgitant velocity on Doppler echocardiography of > 2.8 m/s18  plus exertional dyspnea without evidence of left heart disease 
HF Modified Framingham criteria19  
Thrombosis Compression ultrasonography, contrast venography, or angiography evidence of thrombus 
Cholelithiasis Ultrasonographic evidence of gallbladder stones 
Abnormal liver function ALT > 50 U/L 
Leg ulcers Ischemic or necrotic skin lesion on the lower extremity by general visual inspection 
DM Fasting blood sugar ≥ 126 mg/dL, or 2-hour postprandial blood sugar ≥ 200 mg/dL, or symptoms of hyperglycemia and a casual (random) plasma glucose ≥ 200 mg/dL20  
Hypothyroidism TSH > 4.7 μU/L and a free T4 < 0.8 ng/dL21  
Osteoporosis Bone densitometry T score: 2.5 SD22  
Hypogonadism Females: > 13 y, not yet Tanner B2 (ie, prepubertal breast development) or > 14 y requiring estrogen replacement therapy or > 15 y with primary amenorrhea; males: > 14 y, not yet Tanner G2 (ie, prepubertal genital development) or on androgen replacement therapy or > 17 y, not yet Tanner G4 (ie, midpubertal genital development)23  

ALT indicates alanine transaminase; DM, diabetes mellitus; TSH, thyroid stimulating hormone; and T4, thyroxine.

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