Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma

J Am Acad Dermatol. 2011 Aug;65(2):404-410. doi: 10.1016/j.jaad.2010.05.029. Epub 2011 May 7.

Abstract

Background: Lymphoma-associated hemophagocytic syndrome (LAHS) is a rare clinicopathological entity. It has been described with primary cutaneous lymphomas, mostly of the subcutaneous panniculitis-like T-cell type, and only once with cutaneous T-cell lymphoma (CTCL).

Methods: We report the cases of 5 patients with epidermotropic CTCL who developed LAHS and died shortly thereafter. Unlike LAHS associated with systemic lymphomas, these CTCL-associated LAHS were late events, occurring several years after the initial lymphoma diagnosis.

Limitations: The small number of patients reported renders definite conclusions difficult. Further reports would be needed to confirm our statements.

Conclusion: LAHS is probably underdiagnosed in CTCL patients with acute inflammatory symptoms suggestive of infections but should be considered, especially when cytopenia and elevated triglyceride and ferritin levels are present.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Biopsy, Needle
  • Cause of Death*
  • Combined Modality Therapy
  • Disease Progression
  • Female
  • Humans
  • Immunohistochemistry
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / mortality
  • Lymphohistiocytosis, Hemophagocytic / therapy
  • Lymphoma, T-Cell, Cutaneous / diagnosis*
  • Lymphoma, T-Cell, Cutaneous / mortality
  • Lymphoma, T-Cell, Cutaneous / therapy
  • Male
  • Middle Aged
  • Mycosis Fungoides / diagnosis*
  • Mycosis Fungoides / mortality
  • Mycosis Fungoides / therapy
  • Neoplasm Staging
  • Sampling Studies
  • Sezary Syndrome / diagnosis*
  • Sezary Syndrome / mortality
  • Sezary Syndrome / therapy
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / mortality
  • Skin Neoplasms / therapy
  • Survival Rate