Primary cutaneous follicle centre lymphoma natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.
Natural History
- Primary cutaneous follicle centre lymphoma shows a predilection for the scalp, forehead, and trunk and dissemination to extracutaneous sites rarely occurs.[1]
- Cutaneous relapses noted in ~30% of patients.[2]
- Recurrences usually proximate to the initial site of the tumor.
- Untreated skin lesions increase in size, rarely metastases to other sites.
Complications
- Transformation of systemic follicular lymphoma (FL) into aggressive non-Hodgkin's lymphoma is associated with poor prognosis and has been reported with a wide range of frequency (range between 10%–70%).
- While the annual risk of transformation of systemic follicular lymphoma (FL) is 3%.
Prognosis
- Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.
- The International Extranodal Lymphoma Study Group identified three prognostic factors, that are used to assess a cutaneous lymphoma international prognostic index (CLIPI), which is prognostic of disease-free status.[3]
- Elevated LDH
- More than two skin lesions
- Nodular lesions
- If left untreated, the lesions typically increase in size over years.
- In approximately 10 percent of patients may disseminate to extracutaneous sites.
References
- ↑ Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma—An Unusual Presentation. Hindawi. http://www.hindawi.com/journals/crim/2010/296523/. Accessed on March 02, 2016
- ↑ Primary cutaneous follicle centre lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf5ae3e27c3994bd549b/. Accessed on March 02, 2016
- ↑ Wilcox RA (2015). "Cutaneous B-cell lymphomas: 2015 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 90 (1): 73–6. doi:10.1002/ajh.23863. PMID 25535037.