Leukocytosis and skin lesions: Consider malignancy; adult T-cell leukaemia/lymphoma

Background Adult Tcell leukaemia/lymphoma (ATLL) is a rare mature Tcell malignancy that occurs in patients infected with human Tcell leukaemia virus type 1 (HTLV1). Only a minority of HTLV1 carriers develop neoplastic transformation to ATLL after a 40to 60year latency period. Case description A 49yearold Columbian male presented to the Emergency Department with abdominal pain, weight loss, generalized cutaneous papules and epistaxis. Extensive cytomorphological, histopathological, immunophenotypical, serological, and molecular (cyto)genetic analysis all contributed to reach the diagnosis of acute HTLV1 associated ATLL. The patient was treated with multiagent cytotoxic chemotherapy, consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). Conclusion Acute ATLL is a clinically aggressive disease and carries a poor prognosis. Treatment options include antiviral regimens, chemotherapy, monoclonal antibodytargeted therapy, and allogeneic stemcell transplantation. The incidence of ATLL in nonendemic areas, such as the Netherlands, is increasing due to emigration of HTLV1 carriers from endemic areas.