Subcutaneous panniculitis-like T-cell lymphoma: A clinicopathologic study of 5 cases

Abstract

Three out of the five cases are alive of which two are in complete remission. Our series stresses on the need to be aware of this uncommon entity in a dermatology clinic. Facial and upper extremity skin nodules with involvement of the lower dermis on light microscopical examination and suggestive immunohistochemical findings were frequently observed in our patients in contrast to previously described cases.

Subcutaneous Panniculitis- like T-Cell Lymphoma: Report of two cases

“…Cytopenias in the form of anemia, leucopenia, thrombocytopenia or combined can be seen to a mild degree in these patients. Individuals belonging to older age group (median age, 59 years), presenting with ulcerated nodules and disseminated plaques are likely to have the more aggressive PCGD-TC [4,5]. Systemic manifestations and cytopenias are more common and severe in these patients usually with a fatal outcome.…”

“…The absence of malignant lymphocytes rimming individual fat cells classically seen in SPTCL and presence of reactive B-follicles with germinal centers admixed with plasma cells is indicative of lupus panniculitis [4,7,8]. The treatment of SPTCL includes systemic steroids, multidrug chemotherapy, radiotherapy, and/ or bone marrow transplantation [4][5][6][7]. Since SPTCL behaves in an indolent manner, these patients can have long term remission with high dose systemic steroids.…”

“…Previously, two phenotypic subsets had been described based on T-cell receptor and CD56 expression [2][3][4][5]. SPTCL has a CD3 + , CD4 -, CD8 + , βF1 + , TCR δ1-, CD56 -, CD30 -phenotype and a favorable prognosis; while SPTCL γδ or as per recent WHO classification Primary Cutaneous Gamma Delta T cell Lymphoma (PCGD-TC) have a CD3 + , CD4 -, CD8 -, βF1 -, TCR δ1 + , CD56 + , CD30 + and a poor prognosis [2][3][4][5][6][7]. SPTCL lesions show only subcutaneous involvement by the mildly pleomorphic neoplastic lymphoid cells as was observed in our patients.…”

“…Association with autoimmune disease is more common in SPTCL than PCGD-TC. Distinguishing SPTCL from other types of cutaneous lymphomas is principally based on the subcutaneous localization of these lesions unlike the predominantly dermal involvement seen in mycosis fungoides, anaplastic large cell lymphoma, Natural Killer cell/ T-cell lymphoma [2][3][4][5]7,8]. Immunohistochemistry helps in clinching the diagnosis.…”

“…The annual incidence of cutaneous lymphoma is estimated to be from 0.5 to 1 per 1, 00,000 persons per year. [2] SPTCL is a rare extranodal non Hodgkin lymphoma (NHL) accounting for less than one percent of all NHL [1][2][3][4][5] and resembles certain benign panniculitis like lupus panniculitis and erythema nodosum clinically and histologically. We present the clinicopathological features of two such cases and discuss the differential diagnosis, immunohistochemical and molecular features of SPTCL…”