The LEAST recommended diagnostic test is option 1. Bone marrow aspirate and biopsy
The patient shows the typical clinical picture of Sézary syndrome (cutaneous T-cell lymphoma, likely stage T4NxB2M0), characterized by erythroderma (erythema involving >80% of the body surface area), generalized lymphadenopathy and lymphocytosis with a TCR rearrangement shared between the skin and the blood. Skin biopsies, PET-CT and lymph node biopsies are indicated for diagnosis and staging. In the absence of unexplained cytopenia, a bone marrow study is generally not necessary.
First-line therapies include low-dose methotrexate, extracorporeal photopheresis, retinoids and interferon. Mogamulizumab (an anti-CCR4 monoclonal antibody) is the treatment of choice for relapsed/refractory disease. Allogeneic transplantation should be considered in selected cases.
Reference:
- Dummer R, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers. 2021;7(1):61. Published 2021 Aug 26.
- Latzka J, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023. Eur J Cancer. 2023;195:113343.
- Olsen E, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713-1722.
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