Sézary syndrome – a rare malignant diagnosis in older age
Authors:
Šaková Rita; Dvořáková Markéta; Richter Tomáš; Topinková Eva
Authors‘ workplace:
Geriatrická klinika 1. lékařské fakulty Univerzity Karlovy a Všeobecné fakultní nemocnice v Praze
Published in:
Geriatrie a Gerontologie 2024, 13, č. 1: 21-26
Category:
Overview
Sézary syndrome (SS) is a rare variant of T-cell lymphoma. It is characterised by: erythroderma, generalised lymphadenopathy, presence of Sézary cells in peripheral blood (more than 1000/mm3) and skin infiltrates. This disease is typically diagnosed in seniors over 55–60 years. Therefore patients with this malignant condition may be seen in clinical geriatric practice. As a rule the care of patient with SS has to be multidisciplinary especially with haematooncologic and dermatologic expertise. Despite the therapy, which is complex – and new treatment approaches are being researched – the prognosis is unfavourable. In this case we present 77 years old patient with Sezary syndrome.
Keywords:
Sezary syndrome, older age, case study
Sources
1. Polívka J. Primární kožní lymfomy: současný pohled na vzácná hematoonkologická onemocnění. Inter Med 2016; 18(5): 236–241.
2. Wilcox RA. Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management. Am J Hematol 2016; 91(1): 151–165.
3. Latzka J, Assaf C, Bagot M, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – update 2023. Eur J Cancer 2023; 195: 113343.
4. Atlas hematologické cytologie [online]. 2016 [cit. 2024-2-05]. Dostupné z: http://www.leukemia-cell.org/atlas.
5. Langerová E. Sézaryho syndrom a primární CD 30+ lymfoproliferativní onemocnění Dermatol Praxi 2016; 10(3): 152–154.
6. Cetkovská P. Primární kožní T-lymfomy: mycosis fungoides a Sézaryho syndrom. Onkologie 2010; 4(4): 233–236.
7. Saulite I, Cozzio A, Guenova E. Mycosis fungoides a Sézaryho syndrom. Healthbook TIMES Onkologie Hematologie 2021; 8(2): 22–31.
8. Lebowitz E, Geller S, Flores E, et al. Survival, disease progression and prognostic factors in elderly patients with mycosis fungoides and Sézary syndrome: a retrospective analysis of 174 patients. J Eur Acad Dermatol Venereol 2019; 33: 108–114.
9. Jawed SI, Myskowski PL, Horwitz S, et al. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome), part II: prognosis, management, and future directions. J Am Acad Dermatol 2014; 70(2): 223.e1–17.
10. Polívka J. Novinky v léčbě mycosis fungoides a Sézaryho syndromu. Transfuze a hematologie dnes 2022; 1: 17–26.
11. RESMAIN Study: A multicentre, double blind, randomised, placebo-controlled, phase II trial to evaluate resminostat for maintenance treatment of patients with advanced stage (stage IIB-IVB) mycosis fungoides or Sézary syndrome that have achieved disease control with systemic therapy. Dostupné z: Study Details | Resminostat for Maintenance Treatment of Patients With Advanced Stage Mycosis Fungoides (MF) or Sézary Syndrome (SS) | ClinicalTrials.gov.
Labels
Geriatrics General practitioner for adults Orthopaedic prostheticsArticle was published in
Geriatrics and Gerontology
2024 Issue 1
Most read in this issue
- Interprofessionl recommendations for Alzheimer’s disease diagnostics and therapy: Palliative care in contemporary context
- Arterial hypertension in elderly
- Evaluation of the nutritional status and sarcopenia in a geriatric patient. Clinical guidelines of the board of the Czech Society of Gerontology and Geriatrics
- Evaluation and optimization of medication in seniors with polypharmacy and multimorbidity. Clinical guidelines of the Board of the Czech Society of Gerontology and Geriatrics, Czech Medical Association JEP