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SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA

Therapeutic Approach

A standard treatment guideline for subcutaneous panniculitis-like T-cell lymphoma (SPTCL) has not been established. When SPTCL and primary cutaneous gamma delta T-cell lymphoma were considered the same disease, the standard therapeutic regimen was chemotherapy. Currently, treatment options include systemic corticosteroids, bexarotene, methotrexate, chlorambucil, cyclosporine, and conventional cytotoxic chemotherapy including etoposide and doxorubicin-based regimens.1,2 Radiation therapy has been used to achieve long-term remissions in patients with localized disease.3 The reported 5-year overall survival (OS) rate in patients with SPTCL-alpha/beta is 82% and up to 91% in the absence of hemophagocytic syndrome.4 The 5-year OS in patients with SPTCL-gamma/delta is 11%.5 Allogeneic stem cell transplant is reserved for relapsed or refractory cases.1,6 See Table 116-1.

Table 116-1SPTCL Treatment Table

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB evidence includes evidence from ≥1 randomized controlled trial; level IIA evidence includes evidence from ≥1 controlled study without randomization; level IIB evidence includes evidence from ≥1 other type of experimental study; level III evidence includes evidence from nonexperimental descriptive studies, such as comparative studies, correlation studies, and case control studies; and level IV evidence includes evidence from expert committee reports or opinions or clinical experience of respected authorities, or both.

REFERENCES

1. +
Fujii  K. New therapies and immunological findings in cutaneous T-cell lymphoma. Front Oncol. 2018;8:198.  [PubMed: 29915722]
2. +
López-Lerma ...

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