Table 1.

Comparisons of IEI-related complications in transplanted and matched non-transplanted patients with CID

Complications at baselineAll patients (n = 212)No alloSCT (n = 153)alloSCT (n = 59)P value
Infections     
Bacterial infection 101 (48%) 60 (39%) 41 (69%) <.001  
Viral infection  32 (15%) 7 (5%) 25 (42%) <.001  
Parasitic infection 8 (4%) 2 (1%) 6 (10%) .007  
Invasive aspergillosis 11 (5%) 5 (3%) 6 (10%) .042  
Autoimmune cytopenia     
Autoimmune neutropenia 27 (14%) 19 (12%) 8 (18%) .327 
Autoimmune hemolytic anemia 47 (23%) 42 (27%) 5 (10%) .011  
Autoimmune thrombocytopenia 35 (17%) 28 (18%) 7 (12%) .258 
Colitis 43 (20%) 29 (19%) 14 (24%) .438 
Granuloma 28 (13%) 21 (14%) 7 (12%) .72 
Liver involvement  27 (13%) 19 (12%) 8 (14%) .823 
Interstitial pulmonary involvement§  25 (12%) 23 (15%) 2 (3%) .017  
Hemophagocytic syndrome 14 (7%) 8 (5%) 6 (10%) .194 
Vasculitis 6 (3%) 3 (2%) 3 (5%) .351 
Malignancy     
Lymphoid proliferation 70 (33%) 48 (31%) 22 (37%) .412 
Myeloid malignancy 2 (1%) 1 (1%) 1 (2%) .48 
Solid cancer 11 (5%) 8 (5%) 3 (5%) 
Complications at baselineAll patients (n = 212)No alloSCT (n = 153)alloSCT (n = 59)P value
Infections     
Bacterial infection 101 (48%) 60 (39%) 41 (69%) <.001  
Viral infection  32 (15%) 7 (5%) 25 (42%) <.001  
Parasitic infection 8 (4%) 2 (1%) 6 (10%) .007  
Invasive aspergillosis 11 (5%) 5 (3%) 6 (10%) .042  
Autoimmune cytopenia     
Autoimmune neutropenia 27 (14%) 19 (12%) 8 (18%) .327 
Autoimmune hemolytic anemia 47 (23%) 42 (27%) 5 (10%) .011  
Autoimmune thrombocytopenia 35 (17%) 28 (18%) 7 (12%) .258 
Colitis 43 (20%) 29 (19%) 14 (24%) .438 
Granuloma 28 (13%) 21 (14%) 7 (12%) .72 
Liver involvement  27 (13%) 19 (12%) 8 (14%) .823 
Interstitial pulmonary involvement§  25 (12%) 23 (15%) 2 (3%) .017  
Hemophagocytic syndrome 14 (7%) 8 (5%) 6 (10%) .194 
Vasculitis 6 (3%) 3 (2%) 3 (5%) .351 
Malignancy     
Lymphoid proliferation 70 (33%) 48 (31%) 22 (37%) .412 
Myeloid malignancy 2 (1%) 1 (1%) 1 (2%) .48 
Solid cancer 11 (5%) 8 (5%) 3 (5%) 

EBV, Epstein-Barr virus.

Significant at P < .05.

Viral infections included extensive warts, EBV-associated ulcers, complicated VZV infection, HPV-CIN, EBV viremia, and varicella without complication, herpesvirus, and HPV-CIN in nontransplanted patients.

Liver involvement included nodular regenerative hyperplasia, sclerosing cholangitis, and 1 patient with hepatitis T cell infiltration and another with EBV hepatitis.

§

Lung involvement included 9 of 23 nontransplanted patients with CTLA-4/LRBA deficiency.

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