Table 2.

Common and other GVHD manifestations according to the 2005 NIH-CC present in children at the time of initial onset of cGVHD

NIH-CC cGVHD organ system and clinical manifestation*Frequency in all cGVHD cases (n = 51)Frequency in confirmed NIH-CC cGVHD cases (n = 43)
Skin (n = 32; 62.7%)   
 Dry skin 22 (43.1) 19 (44.2) 
 Erythema/maculopapular rash 17 (33.3) 15 (34.9) 
 Pruritus 13 (25.5) 12 (27.9) 
 Isolated hyperpigmentation 6 (11.8) 6 (14) 
 Isolated hypopigmentation 5 (9.8) 5 (9.8) 
 Ichthyosis 5 (9.8) 4 (9.3) 
 Keratosis pilaris 3 (5.9) 2 (4.7) 
GI tract (n = 20; 39.2%)   
 Anorexia 18 (35.3) 15 (34.9) 
 Weight loss/failure to thrive 15 (29.4) 13 (30.2) 
 Nausea/vomiting 10 (19.6) 7 (16.3) 
 Chronic diarrhea 6 (11.8) 5 (11.6) 
Liver (n = 14; 27.5%)   
 Increased total bilirubin, alkaline phosphatase, AST, and/or ALT >2× ULN 14 (27.5) 13 (30.2) 
Mouth (n = 16; 31.4%)   
 Erythema 11 (21.6) 10 (23.3) 
 Pain 8 (15.7) 7 (16.3) 
 Mucositis 5 (9.8) 5 (11.6) 
 Gingivitis 1 (2) 1 (2.3) 
Eyes (n = 11; 21.6%)   
 Itchy 9 (17.6) 9 (20.9) 
 Photophobia 3 (5.9) 3 (7) 
 Blepharitis 2 (4) 2 (4.7) 
 Excessive tearing 2 (4) 2 (4.7) 
 Difficulty opening eyes in morning 2 (4) 2 (4.7) 
 Periorbital hyperpigmentation 1 (2) 1 (2.3) 
Scalp (n = 6; 11.8%)   
 Thinning of scalp hair 6 (11.8) 6 (14) 
Musculoskeletal system (n = 1; 5.9%)   
 Muscle weakness 1 (2) 1 (2.3) 
Genital (n = 1; 5.9%)   
 Vaginal dryness 1 (2) 1 (2.3) 
Other   
 Pericardial/pleural effusions/ascites 4 (7.8) 2 (4.7) 
 Eosinophilia 3 (5.9) 3 (7) 
 Immune thrombocytopenia 1 (2) 
 Nephrotic syndrome 1 (2) 0 (0) 
 Cardiomyopathy 1 (2) 1 (2.3) 
 Neuropathy 1 (2) 1 (2.3) 
NIH-CC cGVHD organ system and clinical manifestation*Frequency in all cGVHD cases (n = 51)Frequency in confirmed NIH-CC cGVHD cases (n = 43)
Skin (n = 32; 62.7%)   
 Dry skin 22 (43.1) 19 (44.2) 
 Erythema/maculopapular rash 17 (33.3) 15 (34.9) 
 Pruritus 13 (25.5) 12 (27.9) 
 Isolated hyperpigmentation 6 (11.8) 6 (14) 
 Isolated hypopigmentation 5 (9.8) 5 (9.8) 
 Ichthyosis 5 (9.8) 4 (9.3) 
 Keratosis pilaris 3 (5.9) 2 (4.7) 
GI tract (n = 20; 39.2%)   
 Anorexia 18 (35.3) 15 (34.9) 
 Weight loss/failure to thrive 15 (29.4) 13 (30.2) 
 Nausea/vomiting 10 (19.6) 7 (16.3) 
 Chronic diarrhea 6 (11.8) 5 (11.6) 
Liver (n = 14; 27.5%)   
 Increased total bilirubin, alkaline phosphatase, AST, and/or ALT >2× ULN 14 (27.5) 13 (30.2) 
Mouth (n = 16; 31.4%)   
 Erythema 11 (21.6) 10 (23.3) 
 Pain 8 (15.7) 7 (16.3) 
 Mucositis 5 (9.8) 5 (11.6) 
 Gingivitis 1 (2) 1 (2.3) 
Eyes (n = 11; 21.6%)   
 Itchy 9 (17.6) 9 (20.9) 
 Photophobia 3 (5.9) 3 (7) 
 Blepharitis 2 (4) 2 (4.7) 
 Excessive tearing 2 (4) 2 (4.7) 
 Difficulty opening eyes in morning 2 (4) 2 (4.7) 
 Periorbital hyperpigmentation 1 (2) 1 (2.3) 
Scalp (n = 6; 11.8%)   
 Thinning of scalp hair 6 (11.8) 6 (14) 
Musculoskeletal system (n = 1; 5.9%)   
 Muscle weakness 1 (2) 1 (2.3) 
Genital (n = 1; 5.9%)   
 Vaginal dryness 1 (2) 1 (2.3) 
Other   
 Pericardial/pleural effusions/ascites 4 (7.8) 2 (4.7) 
 Eosinophilia 3 (5.9) 3 (7) 
 Immune thrombocytopenia 1 (2) 
 Nephrotic syndrome 1 (2) 0 (0) 
 Cardiomyopathy 1 (2) 1 (2.3) 
 Neuropathy 1 (2) 1 (2.3) 

All data are n (%).

ALT, alanine aminotransferase; AST, aspartate aminotransferase; GI, gastrointestinal; ULN, upper limit normal.

*

Percentage shown by each organ system reflects the proportion of individuals with cGVHD (including all patients with NIH-CC or non–NIH-CC cGVHD) with ≥1 common or other cGVHD manifestation in that organ system at the time of initial cGVHD diagnosis. Common and other cGVHD manifestations as per 2005 NIH-CC that were not reported to the study (0 patients) are not included.

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