Table 2.
Lymphoma subtypes in primary immunodeficiencies associated with high susceptibility to develop EBV-driven lymphoproliferative diseases.
| Mutated gene number of patients/total | Hodgkin lymphoma (subtype) | B-cell NHL | T-cell lymphoma | ||
|---|---|---|---|---|---|
| DLBCL | Burkitt lymphoma | Not specified | |||
|
SH2D1A 25–30% |
30–40%: abdominal (40–50%), cervical (30–40%), spinal (10–20%) |
40–60%: abominal (50–60%), cervical (20%) |
20–30% | 1 patient (CNS) | |
|
ITK 9/13 |
6 patients (1 mixed cellularity) | 1 patient | 1 patient | 1 patient | |
|
MAGT1 5/11 |
2 patients | 1 patient | 1 patient | 1 patient | |
|
COR01A 4/9 |
4 patients | ||||
|
CD27 6/18 |
3 patients (2 scleronodular and 1 mixed cellularity) | 2 patients | 1 patient | ||
|
CTPS1 2/12 |
2 patients (CNS 2/2) |
||||
|
RASGRP1 4/6 |
2 patients (1 scleronodular and 1 mixed cellularity) | 2 patients | |||
|
CD70 4/6 |
4 patients (1 scleronodular and 1 mixed cellularity) | ||||
CNS, central nervous system; NHL, non-Hodgkin lymphoma; DLBCL, diffuse large B-cell lymphoma; EBV, Epstein–Barr virus.