Virchows Archiv

, Volume 475, Issue 5, pp 637–648 | Cite as

Two cases of phenotypic switch of primary cutaneous T cell lymphoma after treatment with an aggressive course and review of the literature

  • Etan MarksEmail author
  • Yang Shi
  • Yanhua Wang
Original Article


A “phenotypic switch” (PS) is a well-known phenomenon that occurs in hematopoietic neoplasms, often after treatment. However, in cutaneous T cell lymphoma (CTCL), this event has rarely been reported, and thus, very little is known about its relevance to disease prognosis. We report two cases of patients that were diagnosed with a CD4+ mycosis fungoides with positive T cell receptor gene rearrangement studies. Both patients originally responded to treatment, but subsequently, their CTCL came back with a different phenotype of a CD4− CTCL. Gene rearrangement studies were performed on the second occurrence in order to prove that this was the same lymphoma. Both patients died from their CTCL. Additionally, we collected seven cases of primary CTCL from the literature with tissue samples from before and after treatment with molecular studies confirming these neoplasms contained the same T cell clone, providing evidence of a true PS. This too revealed a poor prognosis in the majority of these cases. CTCL should be worked up to determine whether a PS has occurred after therapy since it could confuse management of patients and appears to portend a poor prognosis.


Phenotypic switch Cutaneous T cell lymphoma Mycosis fungoides Poor prognosis 


Compliance with ethical standards

All three authors made substantial contributions to the conception and design of the work as well as the acquisition, analysis, and interpretation of data for the work. All authors participated in drafting the work and revising it critically for important intellectual content. All authors gave final approval of the version submitted and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of DermatologyUniversity of Texas Southwestern Medical CenterDallasUSA
  2. 2.Department of PathologyMontefiore Medical Center/Albert Einstein College of MedicineBronxUSA

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