Type of skin eruption is an independent prognostic indicator for adult T-cell leukemia/lymphoma

Abstract

Cutaneous involvement is seen in ϳ 50% of adult T-cell leukemia/lymphoma (ATLL) patients. We investigated the association between skin eruption type and prognosis in 119 ATLL patients. ATLL eruptions were categorized into patch (6.7%), plaque (26.9%), multipapular (19.3%), nodulotumoral (38.7%), erythrodermic (4.2%), and purpuric (4.2%) types. When the T stage of the tumor-nodemetastasis-blood (TNMB) classification of mycosis fungoides/Sézary syndrome was applied to ATLL staging, 16.0% were T1, 17.7% T2, 38.7% T3, and 4.2% T4, and the remaining 23.5% were of the multipapular and purpuric types. For the patch type, the mean survival time (median survival time could not be estimated) was 188.4 months. The median survival times (in months) for the remaining types were as follows: plaque, 114.9; multipapular, 17.3; nodulotumoral, 17.3; erythrodermic, 3.0; and purpuric, 4.4. Kaplan-Meier curves of overall survival showed that the erythrodermic type had the poorest prognosis, followed by the nodulotumoral and multipapular types. The patch and plaque types were associated with better survival rates. Multivariate analysis demonstrated that the hazard ratios of the erythrodermic and nodulotumoral types were significantly higher than that of the patch type, and that the eruption type is an independent prognostic factor for ATLL. The overall survival was worse as the T stage became more advanced: the multipapular type and T2 were comparable, and the purpuric type had a significantly poorer prognosis than T1. (Blood. 2011;117(15): 3961-3967)

Adult T-Cell Leukemia/Lymphoma

“…21 Clinically, skin lesions may include patch, plaque, multipapular, nodulotumoral, erythrodermic, and purpuric lesions. 24 All erythrodermic lesions are seen in the acute variant, whereas the patch-type lesions are predominantly noted in the smoldering subtype. 24 Histopathologically, 3 different patterns of infiltration are seen: perivascular, nodular, and diffuse.…”

“…24 All erythrodermic lesions are seen in the acute variant, whereas the patch-type lesions are predominantly noted in the smoldering subtype. 24 Histopathologically, 3 different patterns of infiltration are seen: perivascular, nodular, and diffuse. 21 Presence of epidermotropism and Pautrier microabscess is not uncommon.…”

“…26 Monoclonal insertion of HTLV-1 provirus DNA is found in virtually all cases of ATLL and is identified by Southern blotting or polymerase chain reaction. 24 Positive HTLV-1 serology with appropriate clinical and morphologic findings of ATLL is sufficient to reach a diagnosis. HTLV-1 molecular testing should be performed in a seronegative patient with a high suspicion of ATLL.…”

“…25 Smoldering type associated with nodulotumoral-type skin lesions has a worse prognosis compared with smoldering type without skin lesions. 24 Additional poor prognostic indicators include age older than 40 years, advanced performance status (2 or more grade) with performance status (PS) based on the 5-grade scale of the World Health Organization, hypercalcemia, high blood lactate dehydrogenase level, b 2 -micro globulin, high serum CD25 levels, more than 3 extranodal lesions, liver and spleen enlargement, lymphadenopathy, and expression of drug-resistance protein and lung-resistance protein. 18,23,24 Proviral load may affect prognosis in ATLL and serve as a marker of tumor burden in diagnosed ATLL.…”

“…24 Additional poor prognostic indicators include age older than 40 years, advanced performance status (2 or more grade) with performance status (PS) based on the 5-grade scale of the World Health Organization, hypercalcemia, high blood lactate dehydrogenase level, b 2 -micro globulin, high serum CD25 levels, more than 3 extranodal lesions, liver and spleen enlargement, lymphadenopathy, and expression of drug-resistance protein and lung-resistance protein. 18,23,24 Proviral load may affect prognosis in ATLL and serve as a marker of tumor burden in diagnosed ATLL. 18 Indolent ATLLs are usually not treated, because they have a relatively better prognosis and because many of the patients are either too old or have additional systemic diseases at presentation.…”