Table 7

WAS-associated sequelae

Donor group and age, y, at HSCTScoreSplenectomyType of sequelae
MSD*    
    0.8 Severe hearing deficiency 
    1.8 Severe hearing deficiency 
    1.3 Chronic sinusitis/mastoiditis; severe growth retardation 
    2.3 Bilateral equinus, spasticity, learning difficulties 
    7.0 Bronchiectasis, chronic restrictive pulmonary disease 
    2.5 Scleroderma, Sjögren's syndrome 
    8.5 Extensive vitiligo 
    10.8 Bronchiectasis 
URD    
    3.0 Severe hearing deficiency 
    5.5 Anxiety, stereotype, school adaptation difficulties 
    8.3 Myocardial hypertrophy, retinopathy 
    17.7 Surgery for aortic root dilatation (vasculitis prior to HSCT) 
    7.6 − Extensive chronic GVHD 
    2.8 − Extensive chronic GVHD 
MMRD    
    2.0 Severe neurologic sequelae following pneumococcal meningitis 
    1.8 Tendon retractions requiring surgery 
    2.4 − Severe neurologic sequelae following pneumococcal meningitis 
    17.0 Kidney transplant from mother 
    4.5 Epilepsy, cognitive deficiency 
    5.0 Severe anorexia due to chronic digestive GVHD 
Donor group and age, y, at HSCTScoreSplenectomyType of sequelae
MSD*    
    0.8 Severe hearing deficiency 
    1.8 Severe hearing deficiency 
    1.3 Chronic sinusitis/mastoiditis; severe growth retardation 
    2.3 Bilateral equinus, spasticity, learning difficulties 
    7.0 Bronchiectasis, chronic restrictive pulmonary disease 
    2.5 Scleroderma, Sjögren's syndrome 
    8.5 Extensive vitiligo 
    10.8 Bronchiectasis 
URD    
    3.0 Severe hearing deficiency 
    5.5 Anxiety, stereotype, school adaptation difficulties 
    8.3 Myocardial hypertrophy, retinopathy 
    17.7 Surgery for aortic root dilatation (vasculitis prior to HSCT) 
    7.6 − Extensive chronic GVHD 
    2.8 − Extensive chronic GVHD 
MMRD    
    2.0 Severe neurologic sequelae following pneumococcal meningitis 
    1.8 Tendon retractions requiring surgery 
    2.4 − Severe neurologic sequelae following pneumococcal meningitis 
    17.0 Kidney transplant from mother 
    4.5 Epilepsy, cognitive deficiency 
    5.0 Severe anorexia due to chronic digestive GVHD 

+ indicates yes; and −, no.

*

Of the 45 patients in this donor group, 8 had sequelae.

Of the 32 patients in this donor group, 6 had sequelae.

Of the 19 patients in this donor group, 6 had sequelae.

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