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. 2018 Jun 4;9:1103. doi: 10.3389/fimmu.2018.01103

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.