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

Chronic lymphocytic leukemia/small lymphoma (CLL/SLL) is an indolent small B-cell neoplasm that may transform into a clinically aggressive disease, namely Richter syndrome, usually as diffuse large lymphoma. Besides, CLL/SLL encompasses increased risk of developing other secondary cancers, including variety T-cell lymphomas, often the anaplastic large-cell type or with cytotoxic phenotype. Here, we report series patients composite lymphomas consisting and angioimmunoblastic (AITL), hitherto unrecognized association. The 3 (1 male/2 females, 68 to 83 y) presented high-grade-type symptoms. One patient was suspicious for in others AITL were concomitant de novo diagnoses. admixed same lymph nodes (3/3 cases) bone marrow (1/2 cases). In all cases, comprised prominent clear cells strong T follicular helper immunophenotype similar mutations TET2 DNMT3A alterations, IDH2 R172K/M, RHOA G17V. received chemotherapy. died early relapse. 2 remained complete remission AITL, 1 recurrent CLL, acute myeloid leukemia. These observations expand spectrum entities occur association CLL/SLL, adding rare variants neoplasms manifesting syndrome. Given disturbances homeostasis affect not only but also subsets, these contribute emergence derivation.