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Primary Cutaneous Anaplastic Large Cell Lymphoma

Indolent CD30 positive skin lymphoma with favorable prognosis

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Primary Cutaneous Anaplastic Large Cell Lymphoma?

Primary cutaneous anaplastic large cell lymphoma belongs to the spectrum of CD30 positive cutaneous lymphoproliferative disorders together with lymphomatoid papulosis. It presents in adults as solitary or multifocal red purple nodules or tumors on the trunk, head, neck, or extremities with frequent ulceration but rare extracutaneous spread.

Histology shows sheets of large pleomorphic cells with abundant cytoplasm and irregular nuclei expressing CD30 in more than seventy five percent of tumor cells, often CD4 positive with variable cytotoxic markers. ALK protein is usually negative in contrast to systemic anaplastic large cell lymphoma. Spontaneous regression is common but relapses also occur.

Management depends on extent and includes radiotherapy or surgical excision for solitary lesions, low dose methotrexate for multifocal disease, and brentuximab vedotin for refractory or extensive cases. Multiagent chemotherapy is reserved for systemic progression. Long term skin surveillance is needed because of relapse and second malignancy risk.

Symptoms

Solitary or grouped red purple nodules
Frequent ulceration of skin lesions
Slow growth on trunk or extremities
Spontaneous regression in some cases
Local relapse without systemic spread

Risk Factors

Adult age at presentation
Underlying lymphomatoid papulosis
Chronic skin inflammation
Immunosuppression in some patients
Male predominance

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When persistent skin nodule does not heal
  • When ulcerated tumor appears on skin
  • When new skin lesion develops in known disease
  • When systemic symptoms accompany skin disease
  • When lymph node involvement is suspected

Treatment Methods

01
Local radiotherapy for solitary lesion
02
Surgical excision when feasible
03
Low dose methotrexate weekly
04
Brentuximab vedotin for refractory disease
05
Topical or intralesional corticosteroids
06
Surveillance for systemic transformation
07
Multidisciplinary dermatology and oncology care

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.