T-Cell Lymphoma Clinical Practice Guidelines (NCCN, 2020)

National Comprehensive Cancer Network

This is a quick summary of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

November 23, 2020

The guideline update on T-cell lymphoma was released on November 2, 2020 by the National Comprehensive Cancer Network.[1]

Diagnosis of Hepatosplenic T-Cell Lymphoma  

A core biopsy of bone marrow or liver is required for diagnosis; bone marrow aspirate, fine needle aspiration of the liver, or peripheral blood studies may be helpful but by themselves are insufficient.

Adequate immunophenotyping to establish diagnosis may comprise immunohistochemistry (IHC) or cell surface marker analysis. IHC panel may include CD20, CD3, CD10, Ki-67, CD5, CD30, CD2, CD4, CD8, CD7, CD56, EBER-ISH, TCRβ, TCRδ, TIA-1, or granzyme B. Cell surface marker analysis by flow cytometry may include kappa/lambda, CD45, CD3, CD5, CD19, CD10, CD20, CD30, CD4, CD8, CD7, CD2, TCRαβ, or TCRγδ.

Workup of Hepatosplenic T-Cell Lymphoma  

For the workup, the following are essential:

  • Physical examination including full skin exam; attention to node-bearing areas, including Waldeyer ring; evaluation of size of liver and spleen, nasopharynx

  • Performance status

  • B symptoms

  • Complete blood cell count with differential

  • Bone marrow biopsy ± aspirate

  • Lactate dehydrogenase

  • Comprehensive metabolic panel

  • Hemophagocytic lymphohistiocytosis (HLH) workup

  • Uric acid

  • PET/CT scan and/or chest/abdominal/pelvic CT with contrast

  • Echocardiogram or MUGA scan if anthracycline-based regimen is indicated

  • Pregnancy testing in women of childbearing age, if chemotherapy or radiation therapy planned

  • HLA typing

Treatment of Hepatosplenic T-Cell Lymphoma  

The optimal treatment approach for hepatosplenic T-cell lymphoma remains undefined; enrollment in a clinical trial is the preferred initial treatment option.

Ifosfamide (with mesna)/carboplatin/etoposide (ICE) is the preferred regimen for induction therapy.

Other recommended regimens include the following:

  • IVAC (ifosfamide/etoposide/cytarabine)

  • Hyper-CVAD (cyclophosphamide/vincristine/doxorubicin/dexamethasone) alternating with high-dose methotrexate and cytarabine

  • Alemtuzumab plus pentostatin

  • CHOEP (cyclophosphamide/doxorubicin/vincristine/etoposide/prednisone)

  • Dose-adjusted EPOCH (etoposide/prednisone/vincristine/cyclophosphamide/doxorubicin)

Consolidation therapy with allogeneic hematopoietic cell transplantation (HCT) is recommended for eligible patients experiencing complete or partial response after initial induction or second-line therapy. Autologous HCT can be considered if a suitable donor is not available or the patient is ineligible for allogeneic HCT.

For more information, see Non-Hodgkin Lymphoma. For more Clinical Practice Guidelines, please go to Guidelines.

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