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Primary cutaneous CD30+ lymphoproliferative disorders with DUSP22 translocation.

Santiago Montes-Moreno1

  • 1Anatomic Pathology Service and Translational Hematopathology Lab, Hospital Universitario Marques de Valdecilla, Universidad de Cantabria, IDIVAL, Santander, Spain. santiago.montes@scsalud.es.

Pathologie (Heidelberg, Germany)
|November 27, 2023
PubMed
Summary

Primary cutaneous CD30+ lymphoproliferative disorders (LPD) are diverse T cell clonal proliferations. A subset, including lymphomatoid papulosis (LyP) and anaplastic large cell lymphoma (ALCL), share a DUSP22 translocation, defining a novel entity with specific molecular and histological features.

Keywords:
Anaplastic large T cell lymphomaCD30+ lymphoproliferative disorderCutaneous lymphomaDUSP22Lymphomatoid papulosis

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Area of Science:

  • Dermatology
  • Hematology
  • Oncology

Background:

  • Primary cutaneous CD30+ lymphoproliferative disorders (LPD) include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (ALCL).
  • These entities were traditionally viewed as distinct based on clinical presentation.
  • Recent molecular studies identified a DUSP22 translocation in a subset of LyP and ALCL cases.

Purpose of the Study:

  • To characterize the clinicopathological and molecular features of DUSP22-translocated primary cutaneous CD30+ LPD.
  • To define the novel entity "LyP with DUSP22t" and its associated diagnostic markers.

Main Methods:

  • Histopathological examination of cutaneous biopsies.
  • Immunohistochemical analysis for protein expression profiles.
  • Molecular detection of DUSP22 translocations.

Main Results:

  • DUSP22-translocated ALCL and LyP with DUSP22 rearrangements exhibit a biphasic pattern with epidermotropism.
  • Consistent protein expression profile includes preserved T Cell Receptor (TCR) expression, CD30, LEF1, HLA, and CD58 positivity.
  • Absence of cytotoxic markers and phospho-STAT3 protein expression is characteristic.

Conclusions:

  • The DUSP22 translocation is a defining molecular feature for a subset of primary cutaneous CD30+ LPD.
  • These findings support the recognition of a novel clinicopathological entity characterized by specific histological and molecular markers.
  • This molecular classification aids in differentiating these LPD subtypes and may have implications for treatment and prognosis.