Cutaneous manifestations of peripheral T‐cell lymphoma, not otherwise specified: A case series highlighting the diagnostic challenges for this heterogeneous group

Abstract

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a rare, heterogeneous group of nodal and extranodal mature T-cell lymphomas that do not correspond to any of the defined T-cell entities, according to the World Health Organization classification. Most cases present with late stage nodal disease; however extranodal involvement is common. Skin and subcutaneous involvement is reported in approximately 20% of cases. Little attention has been given to the highly variable skin manifestations in the literature. It is our experience that lesions can present in ways other than previously described nodular or tumourous lesions that often ulcerate. We present a case series from a large tertiary institution of seven cases of PTCL, NOS with skin involvement, highlighting the variable presentations and diagnostic challenges for this heterogeneous group.

The clinical phenotype and a novel COL7A1 mutation in a Chinese family with dystrophic epidermolysis bullosa pruriginosa

“…3,4,7 Global median OS and PFS (27 and 16.5 months, respectively) corroborate the concept that PC-PTCL-NOS patients overall have a bad clinical outcome notwithstanding the aggressive therapeutic regimens. 3,4 Indeed, the observed OS is quite different to that recently described by Tolkachjow et al 7 : 27 vs. 69 months. As previously reported, the disease has a male predilection (M:F ratio 3:1).…”

“…[4][5][6] PTCLs-NOS featuring skin lesions present at least six months before a hypothetical systemic involvement are regarded as a separate entity, named primary cutaneous PTCLs-NOS (PC-PTCLs-NOS). 3,4,7 Owing to the fact that an increasing number of cutaneous T-cell lymphoma (CTCL) other than mycosis fungoides (MF) has been recognized in recent years, 2,8 the prevalence of PC-PTCL-NOS cases has significantly decreased. In the present study, all PC-PTCL-NOS cases of the last ten years were retrospectively analysed.…”

Peripheral T-cell lymphoma, not otherwise specified, presenting with generalized ulcerated plaques and hypereosinophilia

“…Other investigators have noted that the diagnosis can be challenging because a dense inflammatory infiltrate may surround tumor cells, malignant T cells may have mild cytologic atypia, and PTCL cells lack a pathognomonic T-cell immunophenotype (typically CD3 + and CD4 + , but many immunophenotypes occur). 2 , 7 …”

Rapidly progressive renal failure due to tubulointerstitial infiltration of peripheral T-cell lymphoma, not otherwise specified accompanied by uveitis: a case report

“…Skin and subcutaneous tissues are involved in more than 20% of the cases, which might appear as a primary cutaneous disease (pcPTCL-NOS) or as a part of systemic lymphoma (sPTCL-NOS) [ 8 ]. Other common extranodal sites are bone marrow, liver, spleen, lungs, and gastrointestinal tract [ 7 – 9 ].…”