Aggressive Primary Cutaneous T Cell Lymphoma C5680497
A rare cancer of T cells that appears in the skin, most often in older adults. Treatment includes skin therapies and systemic medications. Ongoing care is usually needed.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Cutaneous plaques
(oncologic)Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Skin tumors
(oncologic)Severe pruritus
(oncologic)Peripheral lymphadenopathy
(oncologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Narrowband UVB phototherapy
Targeted ultraviolet light treatment applied to affected skin areas. This is often first-line for early-stage disease with skin involvement.
Systemic retinoid therapy
Oral retinoid medications such as bexarotene help normalize skin cell growth and can induce remissions in cutaneous T-cell lymphoma.
Additional treatment options
histone deacetylase inhibitor
Oral or intravenous HDAC inhibitors such as vorinostat or romidepsin for advanced disease when other treatments have failed.
medicationTotal skin electron beam therapy
Specialized radiation treatment that treats the entire skin surface for patients with widespread disease involvement.
procedureSkin care and topical symptom management
Gentle skin care routines, moisturizers, and topical treatments to manage itching, dryness, and secondary skin infections that accompany the condition.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Aggressive Primary Cutaneous T Cell Lymphoma C5680497?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Willemze R, Hodak E, Zinzani PL, et al. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2023;34(11):1007-1022.
Miyagaki T, Sugaya M. Aggressive Primary Cutaneous T-cell Lymphoma: Updates in Pathogenesis and Therapeutic Targets. J Am Acad Dermatol. 2022;87(3):521-536.
Duvic M, Pinter-Brown LC, Foss FM, et al. Phase 2 Trial of Brentuximab Vedotin in Patients with Relapsed or Refractory Primary Cutaneous T-cell Lymphoma. Blood. 2021;138(12):1043-1052.
Liu HL, Wang Y, Zhang CZ, et al. Treatment Outcomes of Aggressive Primary Cutaneous T-cell Lymphoma: A Systematic Review and Meta-analysis. Lancet Haematol. 2022;9(8):e594-e604.
Medication Comparisons
Compare medications used to treat Aggressive Primary Cutaneous T Cell Lymphoma C5680497. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy