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Chapter 116: Subcutaneous Panniculitis-Like T-Cell Lymphoma

Leena Rahmat; Sima Rozati

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
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Table 116-1 SPTCL Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Systemic Therapy
Oral corticosteroids SPTCL Immunosuppression Dexamethasone 10 mg; prednisone 40 mg GI sensitivity, infection None III7
Methotrexate +/− oral corticosteroid SPTCL Immunosuppression 15-20 mg weekly Rash, GI/Liver, hematologic, pulmonary toxicity CBC with differential, chemistries, renal function q1-3mo IIIa8
Cyclosporine +/− oral corticosteroid SPTCL Immunosuppression 4 mg/kg/d Hypertension, hematologic toxicity, nephrotoxicity, infections Renal function, CBC with diff, chemistries, magnesium, blood pressure q1-3mo IIIa9
Bexarotene SPTCL Anti-inflammatory, T-cell apoptosis 300 mg/m2/day Central hypothyroidism, hypertriglyceridemia, rash. CBC with differential, chemistries, renal function q1-3mo III10
Anthracycline-based multiagent chemotherapy

SPTCL + HLH

Or refractory SPTCL

 Cytotoxic chemotherapy Cyclophosphamide 750 mg/m2; doxorubicin 50 mg/m2; vincristine 1.4 mg/m2; prednisone 100 mg; etoposide 300 mg/m2. Myelosuppression, GI toxicity, infections, neuropathy. CBC with differential, chemistries, renal function monthly. IIIa2
Autologous or allogenic bone marrow transplant

SPTCL+ HLH

Or refractory SPTCL

 Immune reconstitution varies Infections, myelosuppression, dermatologic, GI, and renal toxicity. CBC with differential, chemistries, renal function daily-weekly. IIIa2
Local therapy
Radiation therapy Local/limited SPTCL without HLH Ablative 40 Gy or more Skin irritation None IIIa3,5,10

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

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

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