Sézary syndrome
Last Updated: 2021-06-28
ICD11: 2B02
Last Updated: 2021-06-28
Author(s): Anzengruber F., Navarini A.
ICD11: 2B02
Sézary & Bouvrain 1938.
Aggressive, leukemic cutaneous T-cell lymphoma.
Typically, light, skin-colored maculae are localized in the skin lesions.. They are called nappes claires (note differential diagnosis pityriasis rubra pilaris).
Edema (anasarca)
Facies-leontina
Palmoplantar hyperkeratoses are visible in almost half of all patients.
Diffuse alopecia and onychodystrophy may occur.
Loco-regional lymphadenopathy
Bone marrow infestation (rare).
ESR/CRP, differential blood count (leukocytosis, relative lymphocytosis), liver and kidney values, LDH, electrolytes.
Sézary syndrome suspected at > 1000 cells/μL
FACS analysis, CD4/CD8 ratio, determination of CD4+CD7 cells
Clonality detection in blood
A bone marrow biopsy is usually not indicated
If necessary, immune electrophoresis
HTLV serology, if applicable
If necessary, Lyme disease serology
CD4+/CD7- T cells > 40% or CD4/CD8 ratio > 10
T-helper phenotype: CD3+, CD4+, CD8-.
Molecular biology: Clonal rearrangement of T cell receptor genes
Staging --> consult most recent SOP, currently being updated.
Generalized.
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