gd cutaneous lymphoma

Cutaneous lymphoma showing TCRgd gene rearrangements

Epidemiology

This is a rare lymphoma with only 23 cases reported1.

Clinical features

Patients cover a wide age range,with a male predominance. There are erythematous skin plaques (sometimes resembling classical mycosis fungoides), nodules or tumours. Mucosal involvement has been reported (possibly related to enteropathy-type T-cell lymphoma and nasal NK/T-cell lymphoma).

Histopathology

May resemble mycosis fungoides; the lymphocytes are enlarged but lack cerebriform nuclei. Other cases show involvement of the reticular dermis and subcutis, resembling subcutaneous panniculitis-like T-cell lymphoma. Most cases show a band of lymphocytes along the base of the epidermis with only single intra-epidermal lymphocytes, but on occasion there is extensive epidermotropism with pagetoid reticulosis-like features.

Immunohistochemistry

CD3	positive
CD4	negative
CD8	negative, rarely positive
CD56	negative
CD57	negative
TIA-1	positive
perforin	positive
granzyme-B	positive
TCRd1	positive

Differential diagnosis

see cutaneous T-cell lymphoproliferative disorders

Prognosis

Aggressive with 36% survival at five years.

References

1Liu, V. and McKee, P.H. Cutaneous T-cell lymphoproliferative disorders: approach for the surgical pathologist: recent advances and clarification of confused issues. Adv Anat Pathol 2002;9:79-100.

This page last revised 26.12.2002.