Primary Cutaneous CD30+ Lymphoproliferative Disorders: a Comprehensive Review

Curr Hematol Malig Rep. 2020 Aug;15(4):333-342. doi: 10.1007/s11899-020-00583-4.

Abstract

Purpose of review: Primary cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ LPDs) are the second most common cutaneous lymphomas after mycosis fungoides and Sezary syndrome. They include primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP), and borderline lesions. The purpose of this literature review is to consolidate the available evidence on the primary cutaneous CD30+ LPD in order to define the tools for correct diagnosis and appropriate treatment.

Recent findings: The current body of knowledge regarding the clinical features, histopathologic changes, recently described genetic alterations, and therapeutic options will be covered in this comprehensive review. Primary cutaneous CD30+ LPD represent rare cutaneous lymphomas that have significant histologic overlap within the defined group as well as with other neoplastic and reactive entities. The importance of differentiating these entities is crucial, as each one has a different clinical course and prognosis.

Keywords: CD30+ lymphoproliferative disorders; Cutaneous lymphoma; Lymphomatoid papulosis; Primary cutaneous anaplastic large cell lymphoma.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Ki-1 Antigen / immunology*
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell* / genetics
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell* / immunology
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell* / therapy
  • Lymphomatoid Papulosis* / genetics
  • Lymphomatoid Papulosis* / immunology
  • Lymphomatoid Papulosis* / therapy
  • Lymphoproliferative Disorders* / genetics
  • Lymphoproliferative Disorders* / immunology
  • Lymphoproliferative Disorders* / therapy
  • Predictive Value of Tests
  • Prognosis
  • Skin Neoplasms* / genetics
  • Skin Neoplasms* / immunology
  • Skin Neoplasms* / therapy

Substances

  • Ki-1 Antigen