LAUSR

A 7-year-old girl born in China and adopted in Canada when 2 years of age presented with erythematous to violaceous oval plaques on her arms, hands and knees, slowly evolving over the previous 4 years (top). The patient had no facial oedema, photosensitivity, atrophic scars or systemic symptoms. A biopsy of a plaque showed a focally-necrotic epidermis infiltrated by prominent atypical lymphocytes with sheath-like angiotropism. We demonstrated a CD3-positive T-cell lineage, with expression of CD2, CD25, CD30, CD43 and CD56. CD68 highlighted several histiocytes. TIA-1, CD4, CD7 and CD5 were weakly positive within the lymphoid population. Epstein–Barr virus (EBV)-encoded small RNA 1 (EBER1) positivity by in situ hybridization confirmed the rare diagnosis of EBV-associated hydroa vacciniforme-like Tcell lymphoma (bottom). In the span of a few months following diagnosis, her disease extended to muscles, lungs and liver. Initially, she was treated with ciclosporin, etoposide and dexamethasone but she developed pancreatic extension of the disease. A course of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) was then administered in the hope of proceeding to an urgent bone marrow transplant. Following pulmonary disease progression despite R-CHOP, a regimen of granulocyte colony-stimulating factor, fludarabine, cytarabine and idarubicin (FLAG-Ida) with alemtuzumab was attempted as salvage chemotherapy but 2 days after initiation of this therapy she developed severe peripheral and pulmonary oedema refractory to diuretics, leading to hospitalisation in the intensive care unit for an acute respiratory distress syndrome. She died of multiorgan failure within a year of diagnosis, with bone marrow aplasia, acute respiratory distress, pulmonary haemorrhages and acute liver and renal failure.