Cytotoxic γ/δ subcutaneous panniculitis‐like T‐cell lymphoma: report of a case with pulmonary involvement unresponsive to therapy

Abstract

Peripheral subcutaneous panniculitis-like T-cell lymphoma (PSPTCL) is a rare form of cutaneous lymphoma recently proposed as a distinct clinicopathological entity. It usually presents with multiple indurated subcutaneous plaques or tumours, most commonly located on the extremities and trunk and clinically mimicking lobular panniculitis. Associated constitutional symptoms due to haemophagocytic syndrome may advance or, more often, complicate the clinical course in about 40-70% of cases. Finding of TIA-1+ and perforin + cytolytic granules in atypical pleomorphic lymphocytes suggests PSPTCL origin from granular cells of T-cell or natural killer cell phenotype. Cells have a CD3+ CD4+ CD8- or CD3+ CD4- CD8+ T-cell phenotype. Moreover, these lymphomas can express natural killer cell associated antigens, such as CD56, especially in gamma/delta variants. PSPTCL following an indolent clinical course with recurrent self-healing lesions have been described. The prognosis of most PSPTCL is poor even when treated with aggressive chemotherapy. This paper reports a case of PCTCL in a young woman with T-cytotoxic differentiation, with rapid progression unresponsive to several treatments.

Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases

“…Clinical and/or histologic data of 34 of these cases have been part of previous studies. [6][7][8]11,18,20 …”

“…5,6 While SPTL-ABs generally have a CD4 Ϫ , CD8 ϩ , CD56 Ϫ phenotype and a favorable prognosis, SPTL-GDs typically have a CD4 Ϫ , CD8 Ϫ T-cell phenotype with frequent coexpression of CD56 and a poor prognosis. [7][8][9][10][11][12] Based on these observations, in the WHO-EORTC classification for cutaneous lymphomas the term SPTL is used only for SPTL-ABs, whereas SPTL-GDs are included in the group of cutaneous ␥␦ T-cell lymphomas (CGD-TCLs). This group is included as a provisional entity within the broad category of peripheral T-cell lymphoma, unspecified.…”

“…13 It should be emphasized that this subdivision of SPTL into 2 distinct groups is based on only a few studies and still needs further confirmation. Evaluation of existing literature, which concerns mainly case reports, small case series, and reviews, [5][6][7][8][9][10][11][12][14][15][16][17][18][19][20][21] is hampered seriously by lack of complete immunophenotypical data and/or inclusion of malignant lymphomas with predominant subcutaneous involvement other than SPTL. 8 This subdivision of SPTL may also have major therapeutic consequences.…”

Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma γ/δ Subtype (Cutaneous γ/δ T-Cell Lymphoma): Report of a Case and Review of the Literature

“…SPTCL is a distinct type of peripheral T-cell lymphoma involving the subcutaneous tissue. [1][2][3][4][5][6][9][10][11][12][13][14] . Clinically, the lesions resemble those of panniculitis.…”

Subcutaneous Panniculitis-like T-cell Lymphoma: Review of Therapies

“…Cutaneous lesions can occur with systemic symptoms such as fever, malaise, anorexia, and weight loss; they can also cause lipodystrophy after resolution. While extra-cutaneous involvement is uncommon, there have been reports of bone marrow, lymph node, liver, spleen, lung, peripheral blood involvement, and even spontaneous regression [2][3][4][5][6][7][8][9][10]. The average age of presentation is mid to late thirty with a female predominance (male: female=0.5) [2].…”