Diagnosis and investigation

• FBC: WBC usually markedly 4 (up to 500 x 109/L) but may be normal; anaemia and thrombocytopenia common.

• Blood film: large numbers of lymphoid cells with marked nuclear irregularity occasionally multilobulated with 'floral' or 'clover leaf appearance.

• Immunophenotyping: generally CD4+, CD8-, CD25+, HLA-DR+ T cells (Mp174).

• Cytogenetics: multiple abnormalities described; no consistent pattern.

• Serum chemistry: hypercalcaemia in 33-50% of patients at diagnosis.

• Bone marrow: diffuse infiltration by ATLL cells.

• Serology: positive for HTLV-I.

Blood film in ATLL: note clover-leaf cell (centre). From Oxford Textbook of Oncology, 2E, with permission.

Prognostic factors and staging

Poor prognostic features are

• Hypercalcaemia.

• Hyperbilirubinaemia.

Four clinical subtypes are described: acute, chronic, smouldering and lymphomatous forms. Acute subtype most common (66%), median survival 6 months despite therapy. Other forms have longer survival but often progress to the acute form after several months. The smouldering form is most indolent and is associated with few circulating cells, skin lesions and occasional pulmonary involvement and survival >24 months.

Management and prognosis

Treatment of ATLL is unsatisfactory. Short responses including CRs (6-12 months) have been achieved with combination chemotherapy (e.g. CHOP) for acute and lymphomatous forms. Infectious complications are frequent with this and more intensive therapy. Single agent deoxyco-formycin has produced responses in relapsed or refractory patients. Patients with acute ATLL or lymphomatous ATLL have median survivals of 6 and 10 months respectively. Death is usually due to opportunistic infection.

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