Nodal T-follicular Helper Cell Lymphoma

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of rare disorders that result from clonal proliferation of mature post-thymic lymphocytes. These T-cell neoplasms account for approximately 10-15% of all lymphomas. The most common subtype of PTCL is classified as "not otherwise specified" (NOS) which accounts for 30-40%. PTCLs have been treated similarly with CHOP (Cyclophosphamide, Hydroxydaunorubicin, Vincristine and Prednisone), often with etoposide (CHOEP), followed by high-dose therapy and autologous stem cell transplantation (ASCT) in first remission. However, \<50% of the patients are cured with CHOP alone, and the progression-free survival rates at 5 years are as low as 20% for PTCLs. Meanwhile, for elderly patients who can't endure CHOPE and proceed ASCT, the long-term survival is even worse. Aclarubicin is an anthracycline which showed good safety profile in the treatment of both myeloid and lymphocytic leukemia. Previous studies have shown that aclarubicin only induces histone eviction without causing DNA damage, and it stands out in pre-clinical models and clinical studies, as it potently kills AML cells. Meanwhile, aclarubicin lacks cardiotoxicity, and can be safely administered even after the maximum cumulative dose of either doxorubicin or idarubicin has been reached. The purpose of this study is to determine the efficacy of Aclarubicin, Cyclophosphamide, Vincristine, and Prednisone (CAOP) in elderly patients with newly diagnosed PTCLs. The investigators hope to try to replace doxorubicin in CHOP with aclarubicin, which is less toxic, without reducing the efficacy of patients while ensuring safety.

This study is conducted to evaluate the efficacy and safety of linperlisib combined with CHOP regimen followed by autologous hematopoietic stem cell transplantation and linperlisib monotherapy maintenance for newly diagnosed nTFHL patients.