Indolent Primary Cutaneous γ/δ T-Cell Lymphoma Localized to the Subcutaneous Panniculus and Its Association With Atypical Lymphocytic Lobular Panniculitis

Abstract

The 2005 classification of lymphoma proposed the designation of subcutaneous panniculitis-like T-cell lymphoma exclusively for those tumors composed of α/β neoplastic cells. Subcutaneous lymphomas that comprised γ/δ T cells are termed primary cutaneous γ/δ T-cell lymphoma. The different clinical outcomes justified this separation; a more indolent course was characteristic of the α/β variants vs the poor 5-year survival of the γ/δ forms. We describe 5 patients with γ/δ T-cell lymphoma localized to the subcutis with a less aggressive clinical course. Two patients were alive 5 years after presentation, and in the remaining 3, the disease is in complete remission after simple intervention. Three patients had a waxing and waning phase, likely representing a prelymphomatous phase, which then progressed to an overt malignant tumor. Therefore, it is important to recognize that not all patients with γ/δ T-cell lymphomas have a poor prognosis.

Peripheral T‐cell and NK cell lymphoproliferative disorders: cell of origin, clinical and pathological implications

“…Patients tend to present clinically with predominant involvement of the lower extremities followed by the torso and arms with multiple deep plaques or nodules, some of which may show ulceration; lymphadenopathy and bone marrow involvement are uncommon. The prognosis of this entity is worse than the TCRαβ + panniculitis‐like subcutaneous T‐cell lymphoma, and hemophagocytic syndrome occurs at a high frequency although more indolent cases have been reported . Treatment with CHOP or CHOP‐like regimens has not been very successful.…”

Primary Marginal Zone Lymphoma of the Subcutis Associated With Panniculitis and Fat Necrosis

“…1 It has been postulated that a subset of subcutaneous T-cell lymphoma arises from a benign precursor known as "atypical lobular lymphocytic panniculitis," which is typically associated with lupus profundus. 2, 3 We present an exceptional case of primary subcutaneous marginal zone B-cell lymphoma associated with panniculitis and extensive hyaline fat necrosis, which, to our knowledge, has not been described previously.…”

A rare case of fever of unknown origin: subcutaneous panniculitis-like T-cell lymphoma (SPTCL)

“…According to the 2005 classification of lymphoma, the designation SPTCL should be considered exclusively for those patients showing α/β neoplastic T lymphocytes. Subcutaneous lymphomas exhibiting γ/δ T lymphocytes are termed as primary cutaneous γ/δ T-cell lymphoma 4. Clinically, α/β T-cell lymphoma is more indolent than γ/δ T-cell lymphoma, which is more aggressive.…”