title: Lymphoma-associated hemophagocytic syndrome (LAHS) in advanced-stage mycosis fungoides/Sézary syndrome cutaneous T-cell lymphoma.
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.
  
  
  CONCLUSIONS
  The small number of patients reported renders definite conclusions difficult. Further reports would be needed to confirm our statements.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, University of Paris 13, AP-HP, Paris, France.
authors.forename: Astrid
authors.initials: A
authors.lastname: Blom
authors.affiliation: Department of Dermatology, University and CHU of Bordeaux 2, Bordeaux, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Beylot-Barry
authors.affiliation: Department of Dermatology, University and CHU of Clermont-Ferrand, Clermont-Ferrand, France.
authors.forename: Michel
authors.initials: M
authors.lastname: D'Incan
authors.affiliation: Department of Dermatology, University of Paris 13, AP-HP, Paris, France. Electronic address: liliane.laroche@avc.aphp.fr.
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
mesh_terms: D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D002423:Cause of Death; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D009367:Neoplasm Staging; D012494:Sampling Studies; D012751:Sezary Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate