Composite Lymphoma as Co-occurrence of Advanced Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Carrying Trisomy 12 and t(14;18) and Peripheral T-cell Lymphoma

Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

“…The majority of the patients (22/35) had a history of CLL/SLL known for 2 to 14 years (median: 5 y) and a smaller subset of patients (13/35) were diagnosed simultaneously or within a short-time interval (< 1 y) with CLL/SLL and PTCL. Most cases (21/38) consisted of composite lesions comprising both PTCL and CLL/SLL in the same biopsy, [13][14][15][16][18][19][20][21][22][24][25][26]28,29,[32][33][34] whereas in the other cases (17/38) the PTCL tissue did not contain the CLL/SLL component. 12,[17][18][19]23,26,27,30,31 The majority of cases (22/38) were qualified as PTCL or PTCL, NOS, often with a cytotoxic phenotype (in 12 cases), 12/38 cases were ALCLs (6 ALK-positive, 5 ALK-negative, and 1 ALK status unknown), and there were 1 case each of aggressive EBV-negative natural killer-cell leukemia, EBV-positve nasal natural killer/T-cell lymphoma, T-large granular lymphocyte leukemia, and nodal lymphoma with a TFH cell phenotype.…”

“…The occurrence of cutaneous or systemic T-cell neoplasms in CLL/SLL patients has been documented in several case reports and case series (Table 1). 12–39 Mechanistically, lymphomagenesis has been linked to the accumulation of oligoclonal or monoclonal T-cell populations with abnormal phenotypes or disrupted functional properties commonly observed in CLL/SLL patients 40,41. Among the 38 systemic T-cell lymphomas reported in CLL/SLL patients so far (Table 1), the majority were diagnosed as peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS),15,16,18,19,21,23,25,26 anaplastic lymphoma kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL), or ALK-negative ALCL 14,17–20,24…”

“…12–39 Mechanistically, lymphomagenesis has been linked to the accumulation of oligoclonal or monoclonal T-cell populations with abnormal phenotypes or disrupted functional properties commonly observed in CLL/SLL patients 40,41. Among the 38 systemic T-cell lymphomas reported in CLL/SLL patients so far (Table 1), the majority were diagnosed as peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS),15,16,18,19,21,23,25,26 anaplastic lymphoma kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL), or ALK-negative ALCL 14,17–20,24…”