[Leukocytosis and skin lesions: consider malignancy; adult T-cell leukaemia/lymphoma].
BACKGROUND
Adult T-cell leukaemia/lymphoma (ATLL) is a rare mature T-cell malignancy that occurs in patients infected with human T-cell leukaemia virus type 1 (HTLV-1). Only a minority of HTLV-1 carriers develop neoplastic transformation to ATLL after a 40- to 60-year latency period.


METHODS
A 49-year-old 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 HTLV-1 associated ATLL. The patient was treated with multiagent cytotoxic chemotherapy, consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP).


CONCLUSIONS
Acute ATLL is a clinically aggressive disease and carries a poor prognosis. Treatment options include antiviral regimens, chemotherapy, monoclonal antibody-targeted therapy, and allogeneic stem-cell transplantation. The incidence of ATLL in non-endemic areas, such as the Netherlands, is increasing due to emigration of HTLV-1 carriers from endemic areas.
Erasmus Medisch Centrum, Rotterdam.
Yorick
Y
Sandberg
Anton W
AW
Langerak
King H
KH
Lam
Anita W
AW
Rijneveld
Kirsten
K
van Lom
D000998:Antiviral Agents / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D015368:Human T-lymphotropic virus 1; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000175:diagnosis* / Q000188:drug therapy; D007964:Leukocytosis; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D011241:Prednisone / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
D000998:Antiviral Agents; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone