Many Patients with Subcutaneous Panniculitis-Like TCL Achieve Complete Remission

Nearly three-quarters of patients with subcutaneous panniculitis-like T-cell lymphoma (TCL) or adipotropic lymphoproliferative disorder achieved complete remission (CR) after a median of three cumulative therapies, according to a recent study.

Joan Guitart, MD, of Northwestern University, and colleagues conducted the research and published their findings in JAMA Dermatology.

The retrospective clinical and pathological review was conducted because “there is a knowledge gap about subcutaneous panniculitis-like [TCL] owing to its rarity and diagnostic difficulty, resulting in an absence of well-documented large case series published to date,” Dr. Guitart and colleagues wrote.

Researchers reviewed 95 cases initially diagnosed as subcutaneous panniculitis-like TCL between 1998 and 2018 at six large U.S. academic centers. A group of pathologists, dermatologists, and oncologists with expertise in cutaneous lymphomas reviewed all cases and classified them as subcutaneous panniculitis-like TCL (n=75) or adipotropic lymphoproliferative disorder (n=20) in “similar cases with incomplete histopathological criteria for [subcutaneous panniculitis-like] TCL designation,” according to the researchers. The median patient age was 38 years (range, 2-81 years), with a female-to-male ratio of 2.7.

CR occurred in 67% of patients who received a median of three cumulative therapies (range, 1-7 therapies) at a mean follow-up of 56 months. Relapse was common, but none of the patients died due to disease progression or hemophagocytic lymphohistiocytosis.

“The experience in this study confirmed an indolent course and favorable response to a variety of treatments ranging from immune modulation to chemotherapy followed by hematopoietic stem cell transplantation,” the researchers wrote.

Patients with subcutaneous panniculitis-like TCL and patients with adipotropic lymphoproliferative disorder had similar clinical presentations, with multiple (85%) or single (15%) tender nodules “mostly involving extremities, occasionally resulting in lipoatrophy,” Dr. Guitart and colleagues reported. There was no evidence of systemic tumor progression outside of adipose tissue.

Hemophagocytic lymphohistiocytosis only occurred in patients with subcutaneous panniculitis-like TCL, and two patients with adipotropic lymphoproliferative disorder progressed to subcutaneous panniculitis-like TCL but did not have associated systemic symptoms or hemophagocytic lymphohistiocytosis.

“Subcutaneous panniculitis-like [TCL] can be challenging to diagnose and has a diverse spectrum of clinical presentation, response, and outcome; the rarity of this condition calls for international collaboration to establish diagnostic and therapeutic guidelines,” Dr. Guitart and colleagues wrote.

Guitart J, Mangold AR, Martinez-Escala ME, et al. Clinical and pathological characteristics and outcomes among patients with subcutaneous panniculitis-like T-cell lymphoma and related adipotropic lymphoproliferative disorders. JAMA Dermatol. 2022. doi:10.1001/jamadermatol.2022.3347