Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders

Am J Surg Pathol. 2014 Sep;38(9):1203-11. doi: 10.1097/PAS.0000000000000217.

Abstract

Intravascular large B-cell lymphomas and EBV NK/T-cell lymphomas commonly follow an aggressive clinical course. We recently reported an entirely intravascular anaplastic large cell lymphoma (ALCL) in the skin with a surprisingly indolent clinical course; interestingly, this lymphoma involved the lymphatic rather than the blood vasculature. We hypothesized that intravascular skin-limited ALCL is distinct from aggressive systemic intravascular lymphomas in its intralymphatic localization and clinical course. We now describe 18 cases of cutaneous intravascular large cell lymphoproliferations from 4 institutions. All 12 intravascular large T-cell lesions were intralymphatic; the majority (9) were CD30 T-cell lymphoproliferative disorders (TLPDs), 5 further classified as intravascular ALK ALCL. One ALK ALCL and 2 benign microscopic intravascular T-cell proliferations were also intralymphatic. A single case of otherwise typical cutaneous follicle center lymphoma contained intralymphatic centroblasts. The clinical and pathologic characteristics of the CD30 TLPDs were similar to those of their extravascular counterparts, including extralymphatic dermal involvement in a subset, DUSP22-IRF4 translocations in half of tested ALK ALCLs, and associated mycosis fungoides in 1; most were skin-limited at baseline and remained so at relapse. All 5 cases of intravascular large B-cell lymphoma involved the blood vasculature and behaved in a clinically aggressive manner; the ALK ALCL, although intralymphatic, was systemic and clinically aggressive. We propose that cutaneous ALK ALCL and related CD30 ALK TLPDs involving the lymphatics are part of an expanding spectrum of CD30 TLPDs. The identification of intralymphatic as distinct from blood vascular localization may provide critical prognostic and therapeutic information.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Anaplastic Lymphoma Kinase
  • Biomarkers, Tumor / analysis*
  • Biopsy
  • Blood Vessels / immunology
  • Blood Vessels / pathology
  • Cell Proliferation
  • Dual-Specificity Phosphatases / genetics
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Interferon Regulatory Factors / genetics
  • Ki-1 Antigen / analysis*
  • Lymphatic Vessels / immunology*
  • Lymphatic Vessels / pathology
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / genetics
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / immunology*
  • Lymphoma, Primary Cutaneous Anaplastic Large Cell / pathology
  • Lymphomatoid Papulosis / genetics
  • Lymphomatoid Papulosis / immunology*
  • Lymphomatoid Papulosis / pathology
  • Male
  • Middle Aged
  • Mitogen-Activated Protein Kinase Phosphatases / genetics
  • Phenotype
  • Prognosis
  • Receptor Protein-Tyrosine Kinases / genetics
  • Skin Neoplasms / genetics
  • Skin Neoplasms / immunology*
  • Skin Neoplasms / pathology
  • T-Lymphocytes / immunology*
  • T-Lymphocytes / pathology
  • Translocation, Genetic
  • United States

Substances

  • Biomarkers, Tumor
  • Interferon Regulatory Factors
  • Ki-1 Antigen
  • interferon regulatory factor-4
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Receptor Protein-Tyrosine Kinases
  • Mitogen-Activated Protein Kinase Phosphatases
  • DUSP22 protein, human
  • Dual-Specificity Phosphatases