Correction: Genome-wide CRISPR screen identifies MAD2L1BP and ANAPC15 as targets for brentuximab vedotin sensitivity in CD30+ peripheral T-cell lymphoma.

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Updated classification and novel treatment prospective for nodal peripheral T-cell lymphomas.

Multicenter Phase II Study of Mogamulizumab (KW-0761), a Defucosylated Anti-CCR4 Antibody, in Patients with Relapsed Peripheral and Cutaneous T-Cell Lymphoma

Genome-Wide CRISPR Screen Identifies MAD2L1BP, ANAPC15 and SLC39A7 As Intrinsic Regulators for Brentuximab Vedotin Sensitivity in Peripheral T-Cell Lymphoma Cells

Introduction Recent studies have shown that CD30 expression can be an important feature of peripheral and cutaneous T-cell lymphomas (PTCLs and CTCLs) and CD30 testing has increased in importance with the emergence of CD30-directed therapy. Areas covered This article reviews the literature on CD30-related biology, prevalence, and therapy in patients with PTCL or CTCL. We searched the PubMed database from 1 January 2010 to 28 April 2020, using terms ‘CD30ʹ (‘peripheral T-cell lymphomas’ or ‘cutaneous T-cell lymphoma’) and ‘immunohistochemistry’ or ‘flow cytometry’ or ‘pathology,’ and synonyms including terms for T-cell lymphoma subtypes. Expert opinion CD30 is expressed at relatively high rates of prevalence across a broad range of PTCLs and CTCLs. CD30 expression may be critical to the development of a subset of PTCLs and also a biomarker for treatment choice in some subtypes. Large-scale randomized, controlled studies have shown that CD30-directed treatment with brentuximab vedotin is significantly more effective against CD30-expressing PTCL and CTCL than current standard-of-care regimens. However, accurate CD30 evaluation is limited by inconsistencies in detection methodology and expression cutoffs defining CD30-expressing disease. Greater understanding of CD30 testing and reporting will enable more patients with CD30-expressing PTCL and CTCL to be identified and treated appropriately.

Cutaneous and peripheral T-cell lymphomas in the u.S. population: A 25-year national review of epidemiological burden and racialdisparities

Peripheral T-cell lymphomas (PTCLs) represent the “ bad cousins ” of the non-Hodgkin lymphoma (NHL) family. PTCLs are a heterogeneous group of malignancies derived from post-thymic T cells, representing approximately 11% of all cases of NHL included in the International Lymphoma Study Group [1]. Th e most commonly encountered subtypes are the so-called nodal PTCLs: PTCL not otherwise specifi ed (PTCL-NOS), systemic anaplastic large cell lymphoma (ALCL) and angioimmunoblastic T cell lymphoma (AITL). With the exception of ALCL (particularly the subtype positive for anaplastic lymphoma kinase), they are mostly characterized by a dismal prognosis. Several clinical studies have reported a median survival of less than 2 years for patients with PTCLs and 5-year overall survival (OS) rates of less than 30% [2,3]. In their article published in this issue of Leukemia and Lymphoma , Broussais-Guillaumot et al . [4] report the daily experience in the treatment and outcome of patients with PTCL in “ reality. ” Th eir data are the refl ection of the true situation we in fact have and witness in hematologic institutions around the world: 48% of patients achieved a complete response to the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy induction regimen, with 5-year OS and event-free survival rates of 28% and 18%, respectively, for the entire population. Unfortunately, CHOP still remains the standard front-line chemotherapy regimen. Th e evolution of PTCL biology and therapy has lagged behind that of B-cell lymphomas, partly due to the fact that in the lymphoproliferative world, PTCLs have only relatively recently been identifi ed as distinct from their B-cell counterparts [5]. Th e explanation for the poor treatment outcome in patients with PTCLs in contrast to those with aggressive B-cell lymphomas remains uncertain. However, one possibility is that the rarity of these disorders has made them diffi cult to study, and the results for patients with PTCLs in studies relating to the therapy of aggressive NHL are “ lost, ” because these cases represent such a small proportion of all those being studied. Th e one thing that can be said with certainty is: what we currently consider optimal treatment for diff use large B-cell lymphoma is not equally effi cacious for PTCL. Durable remissions are uncommon with CHOP-based chemotherapy, particularly in those patients with high risk disease [6]; however, some patients respond to secondary treatment and are able to proceed to high dose chemotherapy and stem cell transplant [7], which is considered the standard of care in this setting. Relapsed/refractory PTCL is a rare disease that follows a more aggressive clinical course than other lymphomas and is associated with the poorest prognosis of all histological subtypes of NHL. Patients who do not respond to front-line treatment or who relapse after a response to such treatment are in fact often either ineligible for transplant or relapse after transplant. Th e availability of agents with the potential to induce a response and prevent the disease from further progression is critical for the treatment and continued survival of these patients. Th e survival rates in PTCL fall even lower following relapse from second and further lines of treatment, as evidenced by the survival after failure. Recognizing the fact that relapsed/refractory PTCL represents an unmet medical need, in the last several years there have been a number of novel therapies tested specifi cally in relapsed/refractory PTCLs [8]. Further, data are emerging to suggest that PTCLs may be selectively sensitive to drugs that are not as active in aggressive B-cell lymphomas [8], providing a strong rationale to study these diseases separately. Th e history of PTCL is abundant, and signifi cant advances have been made over the past few years. From a therapeutic perspective, the new biopathologic and pharmarmacologic advances have led to a “ boost ” in the development of novel therapeutic approaches for PTCLs. Among these new agents, most recently, three agents have obtained Food and Drug Administration (FDA) approval for relapsed/refractory patients: pralatrexate, a folate antagonist; romidepsin, a histone deacetylase inhibitor; and brentuximab vedotin (only for patients with ALCL), an anti-CD30 antibody – drug conjugate [9 – 12]. On the basis of these interesting advances on pretreated patients with PTCL, the next probable step

Final Results of a Phase II Trial of Belinostat (PXD101) in Patients with Recurrent or Refractory Peripheral or Cutaneous T-Cell Lymphoma.

Preliminary Results from a Phase 2 Trial of Brentuximab Vedotin Plus Cyclophosphamide, Doxorubicin, Etoposide, and Prednisone (CHEP-BV) Followed By BV Consolidation in Patients with CD30-Positive Peripheral T-Cell Lymphomas

A Phase II Clinical Study Exploring the Safety and Efficacy of the Oral PI3Kδ Inhibitor, Linperlisib, in Relapsed Refractory T Cell Lymphoma

e19524 Background: T-cell lymphomas are a heterogeneous group of lymphomas, including the cutaneous T cell lymphoma (CTCL) and the peripheral T cell lymphomas (PTCL). Regarding the PTCL, it’s a heterogeneous group including approximately 23 different diseases with the peripheral T-cell lymphoma not otherwise specified (PTCL NOS), angioimmunoblastic lymphoma (AITL), and anaplastic large cell lymphoma (ALCL) being the most frequent subtypes. In contrast with the B cell lymphomas, most of the PTCL have a worse prognosis. We aim in our study to quantify the prognosis in each of the most frequent subtypes of PTCL. Methods: We analyzed patients with either PTCL NOS, AITL, or ALCL treated at Sylvester Comprehensive Cancer Center between 2010 and 2020. We calculated overall survival (OS) using the Kaplan-Meier method with Log-Rank Test to estimate the 95% confidence interval. Results: 98 patients belonged to 1 of the 3 major T-cell lymphoma subtypes: 43 to PTCL NOS, 33 to AITL, and 20 to ALCL, being 7 ALK-positive and 13 ALK-negative. Mean age in PTCL NOS, AITL and ALCL was 56 years (ranging from 8-88), 62 (8-89), and 52 (1-79), respectively. In PTCL NOS, AITL and ALCL respectively, 21 (46%), 15 (45%) and 7 (35%) of patients were female. The three-year and five-year overall survival was 62% (95% CI 42-82) and 30% (95% CI 6-54%) in PTCL NOS, 64% (95% CI 44-84) and 42% (95% CI 4-78%) in AITL, 75 (95% CI 51-99) and 67% (95% CI 33-100) in ALK-negative ALCL. There were no reported deaths in ALK-positive ALCL. The mean survival was lowest in PTCL NOS (p = 0.02), being 3,6 years, while AITL it is 5.2 and in ALCL it is 8.4. Conclusions: Amongst the three major subtypes of PTCL, PTCL NOS have the worse prognosis. Future research is needed to develop a risk stratification tool in each subtype.

Efficacy and Safety of Retreatment with Brentuximab Vedotin in Patients with Relapsed or Refractory Classical Hodgkin Lymphoma or CD30-Expressing Peripheral T Cell Lymphoma

Final results from the embolden trial: pembrolizumab in combination with decitabine and pralatrexate in heavily pretreated patients with peripheral and cutaneous T-cell lymphoma

Brentuximab Vedotin As Single Agent in the Treatment of Relapsed/Refractory CD30 Positive Peripheral T-Cell Lymphoma Patients: A Phase 2 Study of the Fondazione Italiana Linfomi

Belinostat (PXD101) in Patients with Recurrent or Refractory Peripheral or Cutaneous T-Cell Lymphoma: Results of a Phase II Study.