The prognosis of ATLL is generally poor. Clinical subtype, age, increased serum calcium and LDH levels have been identified as major prognostic factors, whereas the size of tumor cells does not have prognostic implications (15). Patients with acute or lymphomatous forms of ATLL have poor prognosis (weeks to one year) and often die due to concurrent infections (4). Chronic and smoldering forms are mostly slowly progressive, but may undergo transformation into acute form of ATLL. Rarely, Hodgkin lymphoma associated with EBV may develop in patients with HTLV-1 associated ATLL (16).
Adult T-cell leukemia/lymphoma Clinical features
Most common in Japan, Caribbean, rare in Europe and U.S.A. Purpuric papules, nodules or tumors, or erythroderma Histological features
Perivascular or diffuse infiltrate of medium to large pleomorphic cells. Epidermotropism. Immunophenotype
CD2+, CD3+, CD4+, CD25+, CD30+/-, some cases CD8+ Molecular biology
Clonal TCR rearrangement in the majority of cases Laboratory evaluation
Leukocytosis with circulating atypical lymphocytes (flower cells). Hypoalbuminemia, hypergammaglobulinemia, and hypercalcemia Treatment
Multiagent chemotherapy. Zidovudine (AZT) and interferon alfa (IFN)
Clinical course and prognosis
Course dependent on subtype. Poor prognosis.
Was this article helpful?