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Journal of the American Academy of Dermatology
Volume 74, n° 1
pages 59-67 (janvier 2016)
Doi : 10.1016/j.jaad.2015.09.013
accepted : 3 September 2015
Original Articles

Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients
 

Iris Wieser, MD a, b, , Chee Won Oh, MD a, Rakhshandra Talpur, MD a, Madeleine Duvic, MD a
a Department of Dermatology, The University of Texas, MD Anderson Cancer Center, Houston, Texas 
b Department of Dermatology, Paracelsus Medical University, Salzburg, Austria 

Reprint requests: Iris Wieser, MD, Department of Dermatology, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030-4095.
Abstract
Background

Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology.

Objective

The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center.

Methods

Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed.

Results

A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma.

Limitations

The limitation of this study is the retrospective study design.

Conclusion

Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas.

The full text of this article is available in PDF format.

Key words : associated lymphomas, CD30+ lymphoproliferative disorders, cutaneous T-cell lymphoma, lymphomatoid papulosis, lymphomatoid papulosis treatment

Abbreviations used : ALCL, AML, CI, CLL, CR, EBV, FMF, HD, LGL, LyP, MF, NR, OR, PR, SD, TCR



 Funding sources: None.
 Conflicts of interest: Dr Duvic: Seattle genetics (research funding for clinical trials and advisory board); all other authors have no conflict of interest to declare.



© 2015  American Academy of Dermatology, Inc. All Rights Reserved.