Single Institutional Experience on Cutaneous Manifestation of Adult T-cell Leukemia/Lymphoma - Potential Diagnostic Pitfall. (28th October 2020)

• Record Type: Journal Article
• Authors: Bridgelall, S; Michalski, J; Zhang, X; Sokol, L; Dong, N; Zhang, L
• Is Part Of: American journal of clinical pathology. Volume 154(2020)Supplement 1
• Page Start: S96
• Page End: S97
• Publication Date: 2020-10-28
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqaa161.211
• Languages: English
• ISSNs: 0002-9173

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.