Clinicopathologic Analysis of 124 Cases of Adult T‐Cell Leukemia/Lymphoma with Cutaneous Manifestations: The Smouldering Type with Skin Manifestations Has a Poorer Prognosis than Previously Thought

Abstract

Adult T-cell leukemia/lymphoma (ATLL) commonly involves the skin as well as peripheral blood and lymph nodes. During the last 15 years we have studied 124 cases of ATLL with specific skin manifestations. Twenty-one patients (16.9%) were classified as acute, 21 (16.9%) as chronic, 26 (21.0%) as lymphoma, and 56 (45.2%) as smouldering according to Shimoyama's classification. Many patients had nodules/tumors (34.7%), erythematous plaques (22.6%), and erythematous papules (19.4%) similar to those occurring with other cutaneous T-cell lymphomas. Some patients displayed characteristic skin manifestations resembling non-neoplastic cutaneous disorders. The median survival time (MST) of all patients was 12.0 months. The MSTs of individual clinical types were: acute type, 4 months; chronic type, 14 months; lymphoma type, 7 months; and smouldering type, 16 months. In the smouldering type, cases with a deeper infiltration pattern (MST, 14 months) had a more aggressive course than those with a superficial infiltration pattern (MST, 24 months) (p < 0.05). The results indicate that smouldering type ATLL with skin manifestations may have a worse prognosis than without skin manifestations. Moreover, some cases of the smouldering type with specific skin lesions should be classified into another group with a much poorer prognosis.

Adult T-cell leukemia/lymphoma (ATL) presenting in the skin: Clinical, histological and immunohistochemical features of 52 cases

“…In addition, patients with primary cutaneous smoldering ATL mostly died of infections or of causes not directly related to ATL. The MST observed in this study for the primary cutaneous smoldering ATL was higher than those reported by Setoyama et al (1999) [30]. Provably this difference may be due to different criteria used by these authors.…”

Long-term study of indolent adult T-cell leukemia-lymphoma

“…Some previous studies suggested that skin involvements might be a risk factor for poor prognosis of smoldering ATL. 6,[20][21][22] In the present study, the frequency of patients with skin lesion was a little higher in smoldering ATL (n ϭ 14; 56%) than in chronic ATL (n ϭ 32; 49%). The OS of smoldering ATL with skin lesion was worse than that of chronic ATL without skin lesion (supplemental Figure 2), although there was no statistical difference (P ϭ .5).…”

“…However, some studies reported that the presence of skin lesions was a possible poor prognostic factor in indolent ATL, 6,20-22 as described earlier. Setoyama et al 21 reported that smoldering cases with a deeper infiltration pattern had a more aggressive course than cases with a superficial infiltration pattern. Degree of skin involvement might be associated with prognosis in indolent ATL.…”

WHO-EORTC classification for cutaneous lymphomas

“…Most patients present with acute ATLL characterized by the presence of leukemia, lymphadenopathy, organomegaly, hypercalcemia, and in about 50% skin lesions, most commonly nodules or tumors (33%), generalized papules (22%), or plaques (19%). 96 Chronic and smoldering variants frequently present with skin lesions, which may closely resemble MF, whereas circulating neoplastic T cells are few or absent.…”