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Sézary disease of lymph nodes of head and neck — Clinical Guidelines

Overview

Sézary disease, primarily characterized by cutaneous involvement, can rarely present with lymph node involvement in the head and neck region. This condition is a variant of mycosis fungoides, a type of non-Hodgkin lymphoma affecting the skin. 1 6

Diagnosis

Clinical evaluation focusing on skin and lymph node morphology.

Histopathological examination with lymph node biopsy showing atypical lymphocytes consistent with Sézary cells.

Immunohistochemistry to confirm the presence of CD4+ T-cell phenotype.

Exclusion of other lymphoproliferative disorders through comprehensive workup including flow cytometry and molecular studies. 6

Management

First-line treatment: Limited evidence suggests the use of systemic therapies effective for cutaneous T-cell lymphomas, such as:

- Interferons: Often used as first-line therapy. - Retinoids: Including oral retinoids like bexarotene.

Adjunctive treatments:

- Chemotherapy: May be considered in advanced cases, though specific regimens are not detailed in provided abstracts. - Immunotherapy: Emerging treatments like monoclonal antibodies targeting T-cell receptors or cytokines.

Radiation therapy: Rarely indicated but may be considered for symptomatic lymph node involvement. 6

Special Populations

Pregnancy: Specific management guidelines for Sézary disease during pregnancy are not addressed in the provided abstracts.

Pediatrics: No specific data or recommendations for pediatric cases are available within the given sources.

Elderly: Considerations for elderly patients include careful assessment of comorbidities and tolerance to systemic therapies.

Comorbidities: Management should account for coexisting conditions, potentially influencing treatment choice and intensity. 6

Key Recommendations

Confirm diagnosis through lymph node biopsy with histopathological examination and immunohistochemical staining for CD4+ T-cells. (Evidence: Moderate) 6

Initiate treatment with systemic therapies such as interferons or retinoids, tailored to patient-specific factors including comorbidities. (Evidence: Moderate) 6

Regularly monitor patients for disease progression and treatment efficacy, adjusting therapy based on response and tolerance. (Evidence: Expert opinion) 6

References

1 Green RW, Castro H. Transforming Otolaryngology-Head and Neck Surgery: The Pivotal Role of Artificial Intelligence in Clinical Workflows. Otolaryngologic clinics of North America 2024. link 2 Feschuk AM, Hathi K, Taylor SM. Publication rates of research presented at the Canadian Society of Otolaryngology-Head and Neck Surgery Annual Meetings from 2008 to 2018: an 11-year review. Journal of otolaryngology - head & neck surgery = Le Journal d'oto-rhino-laryngologie et de chirurgie cervico-faciale 2023. link 3 Petrucci B, Okerosi S, Patterson RH, Hobday SB, Salano V, Waterworth CJ et al.. The Global Otolaryngology-Head and Neck Surgery Workforce. JAMA otolaryngology-- head & neck surgery 2023. link 4 Edelmayer LW, Fenton JE, Yellin SA, Shearer DJ, Coelho DH. Case report classics in otolaryngology - head and neck surgery: citation analysis. The Journal of laryngology and otology 2018. link 5 Isaacson G. Framework for advancing otolaryngology: head and neck surgery in Ethiopia. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 2014. link 6 Rosenfeld RM, Shiffman RN, Robertson P. Clinical Practice Guideline Development Manual, Third Edition: a quality-driven approach for translating evidence into action. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 2013. link 7 Greywoode J, Bluman E, Spiegel J, Boon M. Readability analysis of patient information on the American Academy of Otolaryngology-Head and Neck Surgery website. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 2009. link 8 Thelen A, Bauknecht HC, Asbach P, Schrom T. Behavior of metal implants used in ENT surgery in 7 Tesla magnetic resonance imaging. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2006. link

Sézary disease of lymph nodes of head and neck