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Primary cutaneous follicle centre lymphoma natural history, complications and prognosis

Introduction

Overview

Overview

Prognosis is generally excellent, and the 5-year survival rate of patients with primary cutaneous follicle centre lymphoma is approximately 97%.

Natural History

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

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

  • 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

References

  1. ↑ Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphomaβ€”An Unusual Presentation. Hindawi. http://www.hindawi.com/journals/crim/2010/296523/. Accessed on March 02, 2016
  2. ↑ Primary cutaneous follicle centre lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf5ae3e27c3994bd549b/. Accessed on March 02, 2016
  3. ↑ 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. PMIDΒ 25535037.


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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]