Here are some recommendations of some subtypes.
Key points: 1. Diagnosis: - If available, positron emission tomography (PET)-computed tomography (CT) at diagnosis, at first restaging (optional) and at end-of-treatment can be considered the preferred imaging modality for all nodal and extranodal (non-leukemic) peripheral T-cell and natural killer (NK)-cell lymphomas (PTCLs). - In all cases, a bone marrow biopsy is recommended for accurate staging, including studies of virus expression.
2. First-line treatment: All PTCL patients should, whenever possible, be offered the opportunity to participate in a clinical trial. See Figures 1 to 3 in the original publication.
● PTCL-NOS and follicular helper T-cell-derived lymphoma (angioimmunoblastic and follicular types and not otherwise specified): - Cyclophosphamide-doxorubicin-vincristine-prednisolone (CHOP) or CHOP-like chemotherapy (ChT) (e.g. CHOP-etoposide [CHOEP]) can be recommended as first-line therapy. - In patients with limited-stage, non-bulky disease and a favorable pre-therapeutic risk profile, an abbreviated course of ChT (e.g. three to four cycles of CHOP or CHOEP) can be considered. - Consolidative involved-site radiotherapy (ISRT) can be considered for responding patients with limited-stage disease after CHOP or CHOP-like ChT. - Consolidative autologous hematopoietic stem cell transplantation (auto-HSCT) can be considered in responding patients with high-risk limited-stage or advanced disease.
● Anaplastic large-cell lymphoma (ALCL), systemic types: - In patients with limited-stage, non-bulky ALK-positive or ALK-negative disease and a favorable pre-therapeutic risk profile (International Prognostic Index [IPI] 0-1), an abbreviated course of ChT (e.g. three to four cycles of brentuximab vedotin-cyclophosphamide-doxorubicin-prednisolone [BV-CHP] or CHOEP) can be considered. - Six cycles of BV-CHP is recommended for all other ChT eligible patients (i.e. both ALK-negative and ALK-positive). - Alternatively, six cycles of CHO(E)P can be considered. - Consolidative ISRT can be considered for limited-stage ALCL. - Consolidative auto-HSCT in first complete remission (CR) can be considered for HSCT-eligible, chemosensitive patients with ALK-negative ALCL. - In general, auto-HSCT cannot be recommended for patients with ALK-positive ALCL in first CR. It can, however, be considered for patients with high-risk features (IPI ≥2, bulky disease) with no CR after three cycles of ChT.
● Epstein–Barr virus (EBV)-associated extranodal NK- or T-cell lymphoma (ENKTCL): - EBV DNA in peripheral blood should be monitored by quantitative PCR at baseline and during therapy as a biomarker of response, in addition to imaging-based response assessment. - Fit patients with limited-stage disease should receive ISRT with concurrent, interposed or sequential anthracycline-free, L-asparaginase-containing ChT (e.g. dexamethasone-cisplatin-gemcitabine pegylated L-asparaginase [DDGP] or modified dexamethasone-methotrexate-ifosfamide- pegylated L-asparaginase-etoposide [mSMILE] for HSCT-eligible and L-asparaginase-methotrexate-dexamethasone [AspMetDex] or pegylated L-asparaginase-gemcitabine-oxaliplatin [P-GEMOX] for HSCT-ineligible patients). - A multiagent, anthracycline-free, L-asparaginase-containing regimen can be recommended for patients with stage III and IV nasal disease or stage I-IV extranasal disease (e.g. DDGP or mSMILE for HSCT-eligible and AspMetDex or P-GEMOX for HSCT-ineligible patients). Addition of ISRT can be decided on a case-by-case basis. - First-line treatment with anti-PD-1 antibodies such as sintilimab (not European Medicines Agency [EMA] or Food and Drug Administration [FDA] approved) in combination with L-asparaginase-containing regimens (e.g. P-GEMOX) should also be considered in patients with stage III and IV nasal disease or stage I-IV extranasal disease. - If available, crisantaspase (Erwinia chrysanthemi-derived L-asparaginase) is recommended for patients who have developed hypersensitivity or inactivating antibodies to Escherichia coli-derived L-asparaginase (not EMA or FDA approved in ENKTCL). - In HSCT-eligible responding high-risk patients, consolidative auto- or allogeneic-HSCT can be considered. The choice of HSCT should be made on a case-by-case basis considering factors such as pretherapeutic risk profile, response to first-line therapy, performance status and donor availability. ● T-cell prolymphocytic leukemia (T-PLL): - A watch-and-wait approach can be recommended for asymptomatic T-PLL. - Alemtuzumab is recommended for newly diagnosed symptomatic T-PLL (not EMA or FDA approved but available via named-patient access). - Assessment of CD52 status by flow cytometry can be recommended before, during and after treatment. - During alemtuzumab therapy, antimicrobial prophylaxis against herpes zoster and Pneumocystis jirovecii are recommended, as well as quantitative cytomegalovirus DNA monitoring. - Consolidative non-myeloablative allogeneic-HSCT can be recommended in eligible patients achieving CR who have a donor; otherwise, auto-HSCT can be considered on a case-by-case basis. |