Primary Cutaneous Anaplastic Large Cell Lymphoma
At a Glance
Primary cutaneous anaplastic large cell lymphoma is a primary cutaneous CD30 positive T-cell lymphoproliferative disorder. Lymphomatoid papulosis is a more indolent and self-healing condition. Cutaneous anaplastic large cell lymphoma is a type of cutaneous T-cell lymphoma.
What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?
Patients present at a median age of 60 with skin nodules or tumors, sometimes solitary, with rapid growth and ulceration, and patients often relapse. Only 10% of cutaneous anaplastic large cell lymphoma patients have dissemination to lymph nodes. It is a common form of T-cell lymphoma in HIV-infected patients. The diagnosis is made on skin biopsy, by the finding of sheets of large cells with anaplastic morphology. Mycosis Fungoides should be excluded, which can be done by clinical history and cellular features, as both can show expression of CD 30, a protein also expressed by the neoplastic cells of Hodgkin lymphoma. The neoplastic cells typically show aberrant loss of T-cell antigens, such as CD5, CD2, or CD3.
Cutaneous anaplastic large cell lymphoma usually lacks expression of anaplastic lymphoma kinase (ALK), usually tested by immunohistochemistry on paraffin embedded tissue. Distinction from a systemic form of ALK positive anaplastic large cell lymphoma is made by the lack of expression of the anaplastic lymphoma kinase and the absence of any evidence of lymphoma beyond the original skin lesion. Primary cutaneous anaplastic large cell lymphoma is an indolent disease. Up to 40% of cases show spontaneous regression, and 5-year survival rates are on the order of 90%.
Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved.
No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM.
Sign Up for Free e-newsletters
Regimen and Drug Listings
GET FULL LISTINGS OF TREATMENT Regimens and Drug INFORMATION
|Head and Neck Cancer||Regimens||Drugs|
|Renal Cell Carcinoma||Regimens||Drugs|
Cancer Therapy Advisor Articles
- Umbralisib: A Promising Therapy for Hematologic Malignancies
- Phase 2 Study of Tremelimumab Plus Durvalumab Alone or With Radiation for SCLC
- Phase 2 Study of Pembrolizumab vs Topotecan for Small-Cell Lung Cancer
- Checkpoint Inhibition for Non-Hodgkin and Hodgkin Lymphomas
- Cigar and Pipe Use Significantly Increase Mortality Risk