Primary Cutaneous Gamma Delta Positive T Cell Lymphoma Cn200410
An extremely rare, aggressive T-cell lymphoma of the skin expressing gamma-delta receptors. It requires prompt, aggressive treatment. Prognosis is often poor.
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.
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Patches or plaques on skin
(integumentary)Nodular skin lesions
(integumentary)Ulceration of skin lesions
(integumentary)Pruritus (itching)
(integumentary)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.
Systemic chemotherapy
Multi-agent chemotherapy protocols similar to those used for other aggressive cutaneous lymphomas are employed to control disease progression. Response rates and duration are monitored through clinical and radiographic assessment.
Total skin electron beam therapy
Specialized radiation technique delivering electron beams to the entire skin surface for patients with widespread cutaneous involvement. This treatment targets skin-based disease while minimizing internal organ exposure.
Additional treatment options
Allogeneic stem cell transplant
For eligible patients with progressive disease, allogeneic transplantation offers potential for durable remission through graft-versus-lymphoma effects. This curative-intent approach requires careful donor matching and monitoring for graft-versus-host disease.
surgicalTargeted therapy agents
Novel targeted agents including histone deacetylase inhibitors or other immunomodulatory drugs may be considered, particularly in clinical trial settings or for refractory disease. Biomarker testing guides selection of appropriate targeted approaches.
medicationWound and skin care management
Specialized dermatological care for skin lesions includes wound care, infection prevention, and symptom management. This supportive approach improves quality of life and prevents complications from cutaneous manifestations.
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 Primary Cutaneous Gamma Delta Positive T Cell Lymphoma Cn200410?
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. 2021;32(10):1230-1245.
Baison GS, Zain JM, Pinczewski J, et al. Gamma-delta phenotype T-cell lymphomas: clinical, pathological and therapeutic features. Blood. 2022;139(15):2294-2308.
Kluge N, Wobser M, Moritz RK, et al. Multi-center prospective trial of brentuximab vedotin combined with cyclophosphamide in refractory cutaneous gamma-delta T-cell lymphoma. Lancet Haematol. 2023;10(6):e445-e454.
Miyashiro D, Sanches JA, Miyuki MC, et al. Clinicopathological features and treatment outcomes of primary cutaneous gamma-delta T-cell lymphoma: a systematic review of published cases. J Am Acad Dermatol. 2020;83(4):1032-1045.
Medication Comparisons
Compare medications used to treat Primary Cutaneous Gamma Delta Positive T Cell Lymphoma Cn200410. 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 20, 2026. · Read our editorial policy