NCCN Guidelines® Updates: T-Cell Lymphomas

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for T-Cell Lymphomas, published in this issue (page 285), include the latest updates. To assist readers interested in noting how the guidelines were updated, highlights of major changes pertaining to the portion of the guidelines published in this issue are provided below. To view the most recent version of the guidelines, visit NCCN.org.

Updates in Version 2.2022 from Version 1.2021 include:

Global Changes

  • • Suggested treatment regimen references were updated throughout the guidelines.

  • • Diagnosis, molecular and IHC testing were clarified as "may include" as appropriate.

Peripheral T-Cell Lymphomas

TCEL-1

  • • Diagnosis:

    • ➤ Essential, 3rd bullet, 1st sub-bullet: EBER-ISH was removed and added as a separate bullet. (also for HSTCL-1 and NKTL-1)

    • ➤ Useful, 3rd bullet was revised: “... cell origin (CXCL13, ICOS, PD1), and....”

TCEL-3

  • • After first-line therapy, algorithm was revised by moving footnote, "Consider consolidative HDT/ASCR for high-risk IPI patients in CR1" into the algorithm flow. End-of treatment staging and follow-up recommendations were added.

  • • Footnote “l” was revised: “…and treatment according to the ALCL, ALK-positive algorithm may be considered for ALK-negative ALCL with DUSP22 rearrangement.

TCEL-4 and TCEL-5

  • • Both ALCL, ALK-positive and Other histologies, Interim Restaging and Additional Therapy: Follow-up recommendations were added.

TCEL-7

  • • Follow-up recommendations were added after completion of treatment for relapsed/refractory disease.

TCEL-B 2 of 7

  • • Initial palliative intent therapy, suggested treatment regimens for PTCL-NOS; EATL; MEITL and AITL, including Nodal PTCL, TFH and FTCL and AITL were moved to same page.

    • ➤ Duvelisib was added as a category 2A, other recommended regimen for all subtypes. Corresponding footnote “k” was added.

    • ➤ Alectinib was added as a category 2A, other recommended regimen for ALK+ ALCL only. Corresponding footnote “o” was added.

TCEL-B 3 of 7 and TCEL-B 5 of 7

  • • Second-line therapy and subsequent therapy for PTCL-NOS; EATL; MEIT and ALCL:

    • ➤ Subtype, Nodal PTCL, TFH and FTCL was moved to AITL page of recommendations.

    • ➤ Duvelisib was added as a category 2A, other recommended regimen for both intention to proceed to transplant and no intention to transplant. Corresponding footnote “k” was added.

    • ➤ Alectinib was added as a category 2A, other recommended regimen for ALK+ ALCL only. Corresponding footnote “o” was added.

TCEL-B 4 of 7

  • • Second-line therapy and subsequent therapy for AITL, INCLUDING NODAL PTCL, TFH and FTCL:

    • ➤ Duvelisib was added as a category 2A, other recommended regimen for both intention to proceed to transplant and no intention to transplant. Corresponding footnote “k” was added.

Supportive Care

TYLYM-B 1 of 3

  • • Treatment of TLS, First-line and at retreatment for hyperuricemia was revised:

    • Glucose-6-phosphate dehydrogenase (G6PD) testing is required prior to use of rasburicase. Rasburicase is contraindicated in patients with a history consistent with G6PD. In these patients, rasburicase should be substituted with allopurinol.

      • Low Risk Disease: Allopurinol or febuxostat if intolerant to allopurinol beginning 2–3 days prior to chemoimmunotherapy and continued for 10–14 days

        or

      • Intermediate Risk Disease: Stage I/II and LDH <2X ULN:

        Allopurinol or febuxostat

        OR

        Rasburicase if renal dysfunction and uric acid, potassium, and/or phosphate >ULN

      • High Risk Disease: Stage III/IV and/or LDH ≥2X ULN:

        Rasburicase

TCLYM-B 3 of 3

  • • Mogamulizumab and Drug Eruption bullet was added: “Mogamulizumab has been associated with a drug eruption that can clinically mimic cutaneous T-cell lymphoma. Skin biopsy is recommended to distinguish progression of disease versus drug eruption (Chen L, et al JAMA Dermatology 2019;155:968-971; Hirotsu K, et al JAMA Dermatol 2021;157:700-707).”

Principles of Radiation Therapy

TCLYM-D 1 of 4

  • • A bullet was added with a link: “See NCCN Guidelines for Hodgkin Lymphoma - Radiation Dose Constraints.”

The goal of the NCCN Guidelines® Updates is to provide readers with important changes that the NCCN Guidelines Panel has incorporated into the algorithm since it was last published. For a more complete detailing of the updated guideline‘s modifications, access the NCCN Guidelines in this issue or, for the complete and most up-to-date version, at NCCN.org.

Note: The addition of new language is indicated in italics. Wording that has been removed from the previous version is indicated in strikeout.

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