Table 1.
Clinical Features | Frequency (%) |
---|---|
Hematological features | |
MDS | 70–84 [15,16] |
AML | 14–19 [15,16] |
ALL | 1.3 [15] |
AA | 2.5 [15] |
JMML | 1.3 [15] |
Immunodeficit | |
Monocytopenia | 49–78 [15,16] |
B lymphopenia | 86–100 [15,16] |
NK lymphopenia | 7.8–82 [15,16] |
Neutropenia | 47 [16] |
Infections | |
Severe viral infections | 70 [16] |
Disseminated NTM infections | 53 [16] |
Severe bacterial infections | 49–56 [15,16] |
Severe fungal infections | 16% [16] |
Persistent EBV viremia | 11% [16] |
Warts | |
HPV-related | 35–40 [15,16] |
Oncologic | 3.8 [15] |
Lymphedema | 11–15 [15,16] |
Pulmonary features | |
PAPs | 3.8–18 [15,16] |
Recurrent bacterial infections | 56 [15] |
Pulmonary hypertension | <20% [16] |
Thrombotic complications | 9–25 [15,16] |
Deafness | 1.3 [15] |
Autoimmune features | 11 [15] |
Urinary tract malformation | 5 [15] |
Obsetrian complications | 6.3–33 [15,16] |
Hypothyroidism | 1.3–14 [15,16] |