Abstract
Abstract Introduction/Objective Adult T-cell Leukemia/Lymphoma (ATLL) is an aggressive peripheral T-cell neoplasm caused by the human T-cell lymphotropic virus-1 (HTLV1). Approximately half of the patients diagnosed with ATLL have heterogeneous cutaneous manifestations and 1/3 of those patients have skin changes e.g. rashes, papules, and nodules at initial presentation. There is clinical and morphologic overlap between ATLL and other cutaneous T-cell neoplasms such as Cutaneous T-Cell Lymphoma/mycosis fungoides (CTCL/MF) which could pose a potential diagnostic challenge. Methods A retrospective study was conducted using PathNet system to search for HTLV1 positive ATLL patients. Clinicopathologic features of the patients with cutaneous involvement were analyzed. Results Total 31 patients with ATLL were identified. Nine patients (29%, median 54.5 years, range 47-67 years, male: female ratio 2:7) showed skin manifestations, and the cutaneous involvement with ATLL was confirmed by skin biopsy. Five (55.5%, 5/9) cases were initially misdiagnosed as CTCL/MF. Among the 5 patients, 2 presented with skin rash or diffuse erythematous patch/plaque before developing generalized lymphadenopathy or overt circulating atypical lymphocytosis; 2 developed severe pruritic rash with erythematous skin changes resembling Sezary syndrome; and 1 patient had folliculotrophic MF diagnosed 12 years before. Notably, atypical lymphocytosis (0.46- 41.19/µL) occurred in 3 of the 4 remaining cases. In addition, eight of the 9 patients displayed a variable level of CD3+/CD4+/ CD25strong+ abnormal T-cells on flow cytometry. A low-level bone marrow involvement (2-10%) was found in 8 of 9 cases. Elevated calcium levels were identified in 3 of 9 cases (33%). There were 7 of 8 patients (87.5%) who developed generalized lymphadenopathy when diagnosis of ATLL was rendered. Conclusion In patients with cutaneous manifestations, features including hypercalcemia, atypical lymphocytosis, lymphadenopathy, CD3/CD4/strong CD25 coexpression, and bone marrow involvement should prompt a test for HTLV1. Early diagnosis of ATLL can initiate proper treatment and improve patient clinical outcomes.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.