Primary Cutaneous T-Cell–Rich B-Cell Lymphoma: Clinically Distinct from Its Nodal Counterpart?

Abstract

The cases of two patients with Stage IE primary cutaneous T-cell-rich B-cell lymphoma (TCRBCL) are described. In both, the lesion showed a dense infiltrate by numerous small T lymphocytes with scattered histiocytes and large atypical B-lymphoid cells. Polymerase chain reaction assays demonstrated that the B cells were monoclonal, with immunoglobulin heavy-chain gene rearrangement. No clonal rearrangements of the T-cell receptor gamma gene were observed. Both patients were disease-free at 4 months and at 5 years after therapy, respectively. Although rare, primary cutaneous T-cell-rich B-cell lymphoma appears to have a better prognosis than its nodal counterpart, with or without skin involvement.

WHO-EORTC classification for cutaneous lymphomas

“…Rare cases of primary cutaneous T-cell/histiocyte-rich B-cell lymphoma, characterized by the presence of large scattered B cells in a background of numerous reactive T cells, have been reported. 173,174 Clinically, they show similarities with the groups of PCFCL and PCMZL. These lymphomas commonly present with skin lesions on the head, the trunk or the extremities, and may in fact represent an exaggerated T-cell infiltrate in association with other forms of CBCL.…”

“…Unlike their nodal counterparts, they appear to have an excellent prognosis. 173,174 In addition, rare cases of primary cutaneous intravascular large B-cell lymphoma may be included in this category.…”

Cutaneous T-Cell/Histiocyte-Rich B-Cell Lymphoma: A Case Report and Review of the Literature

“…Usually, THRLBCL occurs in the nodal presentation with or without cutaneous involvement, as an aggressive lymphoma that often presents in stage IV disease, with frequent bone marrow involvement and requiring systemic chemotherapy [2,3,6]. …”

“…However, the clinical behavior of the extranodal counterparts, particularly the primary cutaneous one, is unclear [3]. …”

Canine Inflamed Nonepitheliotropic Cutaneous T‐Cell Lymphoma: A Diagnostic Conundrum

“…[23][24][25][26] Two variants of human MF, namely granulomatous MF and granulomatous slack skin, have a prominent histiocytic component, which can mimic sarcoidosis, granuloma annulare or granulomatous dermatitis. 23,25,26 In humans, NE-CTCL can also have a prominent histiocytic infiltration, which has led to misdiagnosis of granulomatous dermatitis.…”