The Japanese separate patients with adult T-cell leukemia/lymphoma (ATLL) into 4 clinical types.
Type
Clinical and Laboratory Features
smoldering
normal lymphocyte count, >= 5% abnormal T-cells in the peripheral blood, absence of hypercalcemia, LDH <= 1.5 times the ULN, no lymphadenopathy, no involvement of organs, no ascites, no pleural effusion
chronic
absolute lymphocytosis, >= 5% abnormal T-cells in the peripheral blood with absolute count > 3500 per µL, LDH <= 2 times ULN, absence of hypercalcemia, no ascites, no pleural effusion, variable involvement of lymph nodes, spleen, skin and/or lung
lymphoma
normal lymphocyte count, <= 1% abnormal T cells in the peripheral blood, lymphadenopathy and/or extranodal lesions due to T-cell infiltrate
acute
leukemia and/or lymphoma and/or abnormal chemical profile, not classified as one of the other types
where:
• A normal lymphocyte count is < 4,000 per µL (< 4 * 10^9/L)
• ULN = upper limit of normal
• Nonlymphoid organs that may be involved: gastrointestinal tract, bone, CNS, skin, lung
The acute and lymphoma types have a poor prognosis. The smoldering type may be associated with long-term survival.
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