High-grade
B-cell lymphomas with MYC, BCL2, and BCL6 rearrangements (triple hit
lymphoma) are uncommon. We studied the clinicopathologic features of 40 patients with triple hit
lymphoma and compared them to 157 patients with MYC/BCL2 double hit
lymphoma and 13 patients with MYC/BCL6 double hit
lymphoma. The triple hit
lymphoma group included 25 men and 15 women with a median age of 61 years (range, 34-85). Nine patients had a history of
B-cell lymphoma. Histologically, 23 (58%) cases were
diffuse large B-cell lymphoma and 17 cases had features of
B-cell lymphoma, unclassifiable, with features intermediate between
diffuse large B-cell lymphoma and
Burkitt lymphoma. Most cases of triple hit
lymphoma were positive for CD10 (100%), BCL2 (95%), BCL6 (82%), MYC (74%), and 71% with MYC and BCL2 coexpression. P53 was overexpressed in 29% of triple hit
lymphoma cases. The clinicopathological features of triple hit
lymphoma patients were similar to patients with MYC/BCL2 and MYC/BCL6 double hit
lymphoma, except that triple hit
lymphoma cases were more often CD10 positive compared with MYC/BCL6 double hit
lymphoma (p < 0.05).
Induction chemotherapy used was similar for patients with triple hit
lymphoma and double hit
lymphoma and overall survival in triple hit
lymphoma patients was 17.6 months, similar to the overall survival of patients with double hit
lymphoma (p = 0.67). Patients with triple hit
lymphoma showing P53 overexpression had significantly worse overall survival compared with those without P53 overexpression (p = 0.04). On the other hand, double expressor status and prior history of
B-cell lymphoma did not correlate with overall survival. In conclusion, most patients with triple hit
lymphoma have an aggressive
clinical course and poor prognosis and these
tumors have a germinal center B-cell immunophenotype, similar to patients with double hit
lymphomas. P53 expression is a poor prognostic factor in patients with triple hit
lymphoma.