Allogeneic haematopoietic stem cell transplantation in a patient with cutaneous γ/δ-T-cell lymphoma.

Reported cases of subcutaneous panniculitis-like T-cell lymphoma (SPTL) show different clinical courses depending on the immunophenotype of the tumour cells. They can either express α and β or γ and δ polypeptide chains in the T-cell receptor (TCR). These differences caused the World Health Organization's European Organization for Research and Treatment of Cancer (WHO-EORTC) to classify this type of lymphoma into an α/β-TCR subtype (SPTL-AB) solely constituting the group of panniculitis-like T-cell lymphoma, and a γ/δ-TCR subtype (CGD-TCL), which was regrouped into the broad category of peripheral T-cell lymphoma, not otherwise specified (1). Clinically, CGD-TCL appears as indurated red plaques and nodes with predominance to the trunk and the extremities often leading to diagnostic confusion with differential diagnosis such as lupus panniculitis, cel-lulitis or other erythematous skin diseases. Ulcerations have rarely been reported. A 51-year-old woman presented with a 10-month history of partly ulcerating painful subcutaneous nodules and plaques with predominance of the trunk and the extremities (Fig. 1 a, b). She did not report any B-symptoms. The patient's medical history revealed chronic seronegative rheumatoid polyarthritis, myo-cardial infarction at the age of 45 years, arterial hypertension, lung emphysema, allergic rhinoconjunctivitis and substituted hypothyroidism. Blood count on first presentation showed moderate leucopaenia and neutropaenia (leukocytes: 3,360/µl A representative histological specimen showed a superficial and deep angiocentric infiltrate of medium-to large-sized pleo-morphic lymphocytes with focal " fat-cell-rimming " as well as necrosis of the dermis and subcutis (Fig. 1c). Immunohistochemi-cal staining revealed a CD4-, CD8-and CD56-negative infiltrate, whereas granzyme B, and especially Ki67 showed high positivity (Fig. 1d). Beta-F1 staining was negative in 80% of the tumour cells. Furthermore, we found a positive TCR-γ-rearrangement in the monoclonal lymphocytes, while TCR-δ-rearrangement was lacking. In situ hybridization for Epstein Barr virus (EBER) was negative in the tumour material. A γ/δ-T-cell lymphoma was diagnosed with regard to the histological appearance and the immune profile (Table SI; available from EORTC classification of T-cell lymphomas (2). Staging included computed tomography scan of the chest and abdomen , sonography of the lymph nodes, bone marrow smear, bone marrow biopsy, and peripheral blood smear, all showing no signs of lymph node, organ or bone marrow involvement. The resulting tumour-node-metastasis (TNM)-classification before treatment according to the EORTC was T3N0M0 at first presentation (1). Our patient underwent combined topical psoralen and ultraviolet A (PUVA)-therapy, as well as treatment with metho trexate and systemic corticosteroids, all with no response. After establishment of …