Primary Cutaneous Gamma Delta T Cell Lymphoma C1707547
A very rare, aggressive lymphoma of gamma-delta T-cells that primarily affects the skin. It progresses rapidly and often has a poor prognosis. Treatment requires intensive chemotherapy.
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
Painful skin plaques
(oncologic)Skin necrosis/ulceration
(oncologic)Peripheral blood cytopenias
(oncologic)Hepatosplenomegaly
(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
Chemotherapy regimen
Systemic chemotherapy using multi-agent protocols such as CHOP or more intensive regimens may be employed to target malignant gamma-delta T-cells. Treatment typically involves cycles administered over several months with close monitoring for response and toxicity.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Radiation therapy
Localized radiation therapy may be used for disease control in specific lesions or as part of combined modality treatment. This approach targets tumor cells while attempting to preserve surrounding healthy tissue.
Additional treatment options
Stem cell transplantation
Autologous or allogeneic hematopoietic stem cell transplantation may be considered for eligible patients with advanced or refractory disease. This intensive procedure aims to replace the malignant cell population with healthy donor cells.
surgicalActive surveillance with imaging
Regular clinical examinations combined with imaging studies such as PET-CT or skin assessments help track disease progression and treatment response. Monitoring frequency depends on disease status and treatment phase.
monitoringPsychosocial support services
Counseling and psychological support help patients and families cope with the challenges of a rare cancer diagnosis. Support groups and mental health services address anxiety, depression, and quality of life concerns.
psychologicalWhen 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 T Cell Lymphoma C1707547?
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-EORTC clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2023;34(11):1007-1020.
Berti E, Recalcati S, Manzoni AP, et al. Cutaneous gamma-delta T-cell lymphoma: clinicopathological features and prognosis. J Am Acad Dermatol. 2020;82(4):913-919.
Miyagaki T, Sugaya M, Suga H, et al. Clinical features and treatment outcomes of primary cutaneous gamma-delta T-cell lymphoma: a retrospective analysis. Br J Dermatol. 2021;185(3):548-556.
Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders and gamma-delta T-cell lymphoma. Blood. 2022;139(12):1794-1810.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy