1. T-cell prolymphocytic leukemia presenting with erythematous patches, plaques, and erythema gyratum–like lesions masquerading as Sézary syndrome
- Author
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Ling Zhang, Lubomir Sokol, Lucia Seminario-Vidal, Leah Cohen, Adel Haque, and Sophia Ma
- Subjects
Pathology ,medicine.medical_specialty ,T-PLL, T-cell prolymphocytic leukemia ,Case Report ,Dermatology ,cutaneous presentations ,Serology ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Immunophenotyping ,Bone marrow aspirate ,Gyrate erythema ,medicine ,Leukocytosis ,Prolymphocytic leukemia ,business.industry ,SS, Sèzary syndrome ,medicine.disease ,TCR, T-cell receptor ,Sézary syndrome ,030220 oncology & carcinogenesis ,gyrate erythema ,T-cell prolymphocytic leukemia ,MTCLs, mature T-cell leukemias ,Erythema gyratum ,medicine.symptom ,business - Abstract
Mature T-cell leukemias (MTCLs) are clonal proliferations of posttyhmic T cells that often exhibit systemic involvement including a variety of cutaneous manifestations.1 Because of the morphologic and phenotypic overlap, diagnosis of MTCLs requires comprehensive assessment of clinical presentation, immunophenotypic profiles, and serologic, cytogenetic, and molecular studies.2 T-cell prolymphocytic leukemia (T-PLL) accounts for only 2% of small lymphocytic leukemias in adults and classically manifests with striking leukocytosis, lymphadenopathy, and marked hepatosplenomegaly.1, 3 Unlike Sezary syndrome (SS), in which cutaneous presentation is a rule, only one-third of patients with T-PLL have cutaneous manifestations.4, 5, 6, 7 Cutaneous manifestations of T-PLL are heterogenous.5, 7, 8 We describe a patient who presented with erythematous patches and plaques, gyrate erythema, and bulky lymphadenopathy with an immunophenotype masquerading as SS. A diagnosis of T-PLL was confirmed using flow cytometry and cytogenetic analysis of the bone marrow aspirate demonstrating chromosome 14q32 rearrangement.
- Published
- 2019
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