Primary cutaneous γδ T‐cell lymphoma with unusual immunophenotype: A case report and review of published work

Abstract

Primary cutaneous γδ T‐cell lymphoma (CGD‐TCL) is a rare form of primary cutaneous lymphoma. The histopathological features of CGD‐TCL are still unclear because of its rarity. Here, we report a case of a 77‐year‐old Japanese man who presented with a 9‐month history of erythematous plaques on his left forearm. Skin biopsy specimens revealed the infiltration of atypical medium/large‐sized lymphocytes from the epidermis to the deep dermis. Atypical lymphocytes were positive for CD3, CD5, CD8 and Vδ1, and negative for CD4, CD7, CD56, EBER‐ISH, intracellular antigen‐1, granzyme B and perforin. CD30 was partially expressed. We also reviewed 246 cases of CGD‐TCL from the published work. CD4−CD8− double‐negative cases were 113 of 196 cases (57.6%), followed by CD4−CD8+ cases (52/196, 26.5%). CD5 was expressed in 25.8% of the cases (34/132). At least one cytotoxic molecule marker was expressed in 150 of 160 cases (93.8%). Some cases showed an indolent clinical course, especially in mycosis fungoides‐like CGD‐TCL cases. CD5 positivity and lack of cytotoxic molecule expression could be associated with a better prognosis. In addition, CD30 expression was found in approximately half of CGD‐TCL cases (51/112 cases), suggesting that brentuximab vedotin could be a good treatment option for such patients. Further studies with more cases with detailed clinical and pathological information are necessary to elucidate the etiology and prognostic markers of this entity.

Primary Cutaneous Gamma-Delta T Cell Lymphomas: A Case Series and Overview of the Literature

“…As expected, CD5 expression was frequently lost in our patients and did not significantly correlate with either subgroup or survival. A large review of 246 PCGDTCLs published by Kamijo et al [15] claims that CD5 positivity and the absence of cytotoxic markers are positive prognostic factors, but we did not find any correlation with survival.…”

“…The published data concerning CD30 expression in PCGTCLs are not consistent [11,15], but, as was also expected, evidence of low-medium CD30 expression was found in 41% of our cases [10,16]. Although it may not correlate with prognosis, CD30 expression can be useful as a therapeutic target of the anti-CD30 monoclonal antibody brentuximab vedotin [16].…”

The significance of epidermal involvement in primary cutaneous gamma/delta (γδ) T‐cell lymphoma: A systematic review and meta‐analysis

“…2 PCGDTCL is composed of gamma/delta (γδ) T-cells, which are typically positive for the γ and δ chains of the TCR and negative for the β chain, although aberrant phenotypes are possible. 5 Consistent with the phenotype of physiologic γδ T-cells, the cells of PCGDTCL are typically double-negative for T-cell markers CD4 and CD8 and almost universally positive for CD3. Cells typically express NK markers such as CD56 and are often positive for cytotoxic markers including TIA-1 and granzyme B.…”

Successful salvage therapy for refractory primary cutaneous gamma-delta T-cell lymphoma with a combination of brentuximab vedotin and gemcitabine

“…ASCT has shown favorable and durable outcome for some patients [ 5 – 9 ], however maximal lowering of the disease burden is still necessary before proceeding to transplant. As CD30 is expressed in almost half of the cases of PCGD-TCL [ 10 ], brentuximab-vedotin is a potential therapeutic option. Recently, brentuximab-vedotin has shown efficacy in four patients, even if the expression of CD30 was low [ 11 ].…”