Fatal ALK-negative systemic anaplastic large cell lymphoma presenting with disseminated cutaneous dome-shaped papules and nodules

Dermatol Online J. 2012 May 15;18(5):5.

Abstract

Anaplastic large cell lymphoma (ALCL) is classified as a CD30 positive non-Hodgkin lymphoma. Systemic ALCL (S-ALCL) is further subdivided into two subgroups based on anaplastic lymphoma kinase (ALK) expression. In systemic ALCL, positive ALK expression correlates with a favorable prognosis, whereas negative ALK expression correlates with poorer overall survival. By definition, primary cutaneous ALCL (cut-ALCL) is limited to the skin and is uniformly ALK-negative. Cut-ALCL closely resembles LyP with regards to its benign clinical course and CD30 positivity. We describe a unique case of ALK-negative (ALK-) S-ALCL presenting with cutaneous disseminated dome-shaped papules.

Publication types

  • Case Reports

MeSH terms

  • Anaplastic Lymphoma Kinase
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bone Marrow / pathology
  • Cyclophosphamide / administration & dosage
  • Dexamethasone / administration & dosage
  • Diagnosis, Differential
  • Fatal Outcome
  • Humans
  • Lymphocytes / pathology
  • Lymphoma, Large-Cell, Anaplastic / diagnosis
  • Lymphoma, Large-Cell, Anaplastic / drug therapy
  • Lymphoma, Large-Cell, Anaplastic / enzymology*
  • Lymphoma, Large-Cell, Anaplastic / pathology*
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / diagnosis
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / enzymology
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology
  • Male
  • Middle Aged
  • Receptor Protein-Tyrosine Kinases / analysis*
  • Salvage Therapy
  • Skin / pathology*
  • Skin Neoplasms / enzymology
  • Skin Neoplasms / pathology

Substances

  • Dexamethasone
  • Cyclophosphamide
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Receptor Protein-Tyrosine Kinases