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NCCN Flash Update: NCCN Guidelines® & NCCN Compendium® Updated for T-Cell Lymphomas

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) and the NCCN Drugs & Biologics Compendium (NCCN Compendium®) for T-Cell Lymphomas. These NCCN Guidelines® are currently available as Version 1.2018.

  • Peripheral T-Cell Lymphomas (PTCL)
    • Subtypes, "Nodal peripheral T-cell lymphoma with TFH phenotype" and "Follicular T-cell lymphoma" were added. (TCEL-1)
    • ALCL, ALK positive, Stage I, II (TCEL-3)
      • First-line therapy, "Multiagent chemotherapy x 3–4 cycles + ISRT (30–40 Gy)" was changed from a category 2A to a category 2B.
        • After first-line therapy, "Interim restaging with PET/CT or C/A/P CT scan with contrast" was added and the options for "Complete or partial response" and "Progressive or refractory disease" were added.
    • All other subtypes, Stage I-IV (TCEL-4)
      • After first-line, "Interim restaging with PET/CT or C/A/P CT scan with contrast" was added and the options for "Responding disease" and "Progressive or refractory disease" were added.
    • Relapsed/refractory disease, no intention to transplant (TCEL-5)
      • Best supportive care was added as an option.
      • Response assessment after second-line therapy was added with options for "complete or partial response" and "no response".
    • Two additional prognostic indices were added (TCEL-A)
      • Prognostic Index for PTCL-U (modified-PIT)
      • International T-Cell Lymphoma Project
    • Suggested treatment regimens
      • First-line therapy
        • ALCL, ALK+ histology
          • Dose-adjusted EPOCH was added.
        • Other histologies (ALCL, ALK-; PTCL, NOS; AITL; EATL; MEITL; Nodal PTCL, TFH; FTCL)
          • Other recommended regimens, HyperCVAD was changed from a category 2A to a category 3 recommendation.
    • Second-line and Subsequent Therapy for ALCL (TCEL-B 4 of 5)
      • Brentuximab vedotin was added as the "preferred regimen" for both with intention and no intention to transplant.
      • Crizotinib (ALK+ ALCL only) was added as an option under “other recommended single agents” for both with intention and no intention to transplant.

 

  • Breast Implant-Associated ALCL
    • Adjuvant treatment (BIAA-2)
      • RT dose was added as “24-36 Gy.”
    • The Proposed TNM Staging for Breast Implant-Associated Anaplastic Large-Cell Lymphoma was added to the algorithm. (BIAA-B)

 

  • Mycosis Fungoides/Sezary Syndrome
    • Stage IV (MFSS-9)
      • Sezary syndrome 
        • For Refractory disease to multiple previous therapies or progression, "Consider allogeneic HCT, as appropriate" was added.
      • Non Sezary or visceral disease
        • For Refractory disease to multiple previous therapies or progression, "Consider allogeneic HCT, as appropriate" was added.
    • Suggested Treatment Regimens (MFSS-A)
      • Systemic therapies, brentuximab vedotin was added to Category A (SYST-CAT A) as a category 2A recommendation with the following footnote:
      • "A randomized phase 3 trial comparing brentuximab vedotin (BV) with physician’s choice of oral bexarotene or methotrexate, showed superior clinical outcome of BV in patients with CD30+ MF and pcALCL. CD30 positivity was defined as CD30 expression >10% of total lymphoid cells in at least 1 of minimal 2 skin biopsies required to evaluate for eligibility. Forty-four percent of eligible patients with MF had at least 1 screening skin biopsy with CD30 <10%. In the two previously reported investigator-initiated studies, clinical responses with BV was observed across all CD30 expression levels including in those with negligible CD30 expression."
    • Principles of Radiation Therapy for MF/SS was added. (MFSS-C)

 

  • Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders
    • Primary cutaneous ALCL (PCTLD-4)
      • Multifocal lesions, the primary treatment recommendations were separated into "preferred" and "other"
        • Brentuximab vedotin was listed as "preferred" and was changed from a category 2A to a category 1 recommendation.
        • The remaining primary treatment options are listed under "other recommended regimens."
      • Cutaneous ALCL with regional node, the primary treatment recommendations were separated into "preferred" and "other"
        • Brentuximab vedotin ± ISRT was listed as "preferred."
        • The remaining primary treatment options are listed under "other recommended regimens."
    • Lymphomatoid papulosis (PCTLD-5)
      • Limited lesions was separated into "Limited lesions, asymptomatic" and "Limited lesions, symptomatic."
      •  After primary treatment, "asymptomatic disease" was changed to "response" and "symptomatic disease" was changed to "No response/refractory disease."
        • For relapsed/refractory disease after response, "continue observation" was changed to "Continue current management" and "topical steroids" was removed.
        • For relapsed/refractory disease after no response/refractory disease, "clinical trial" was added as an option.

 

  • T-Cell Prolymphocytic Leukemia
    • Primary Treatment (TPLL-2)
      • Intravenous alemtuzumab alone is listed as a preferred option.
      • FMC followed by IV alemtuzumab and IV alemtuzumab and pentostatin are recommended for selected patients.

 

  • Extranodal NK/T-Cell Lymphoma, nasal type
    • Induction therapy, Nasal, Stage I-II (NKTL-3)
      • For patients fit for chemotherapy, the last induction therapy option was revised as, "Sandwich chemoradiation in selected patients."
    • Post-RT Evaluation (Nasal, Stage I,II and Stage IV; Extranasal, stage I-IV), (NKTL-4)
      • Response to therapy: "Refractory disease" was changed to "No response."
      • Additional therapy:  "Second-line chemotherapy (pegaspargase-based)" was clarified as "combination chemotherapy regimen (asparaginase-based)"
      • Clinical trial (preferred) and Pembrolizumab were added as options for relapsed/refractory disease following second-line therapy with asparaginase-based regimens.
      • Footnote q was added to pembrolizumab, "Clinical trial is the preferred relapsed/refractory option. In the absence of a clinical trial, pembrolizumab is an appropriate option."
  • Supportive Care
    • Tumor Lysis Syndrome (LYMP-A 1 of 2)
      • Laboratory hallmarks of TLS, "elevated creatinine" was added.
    • Anti-CD52 Antibody Therapy: Alemtuzumab, three bullets were added: (LYMP-A 2 of 2)
      • Herpes virus prophylaxis with acyclovir or equivalent
      • PJP prophylaxis with sulfamethoxazole/trimethoprim or equivalent
      • Consider antifungal prophylaxis

 

  • Principles of Radiation Therapy (LYMP-C 3 of 4)
    • General dose guidelines, "RT in conventional fraction sizes" was added to the heading.
    • 3rd bullet, dosing for primary cutaneous anaplastic large cell lymphoma was revised from "30–36 Gy" to "24–36 Gy.

 

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium®), the NCCN Biomarkers Compendium®, the NCCN Chemotherapy Order Templates (NCCN Templates®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

To view the NCCN Guidelines for Patients®, please visit NCCN.org/patients.

Free NCCN Guidelines apps for iPhone, iPad, and Android smartphones & tablets are now available! Visit NCCN.org/apps

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