Astrid Blom1, Marie Beylot-Barry2, Michel D'Incan3, Liliane Laroche4. 1. Department of Dermatology, University of Paris 13, AP-HP, Paris, France. 2. Department of Dermatology, University and CHU of Bordeaux 2, Bordeaux, France. 3. Department of Dermatology, University and CHU of Clermont-Ferrand, Clermont-Ferrand, France. 4. Department of Dermatology, University of Paris 13, AP-HP, Paris, France. Electronic address: liliane.laroche@avc.aphp.fr.
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.
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 CTCLpatients with acute inflammatory symptoms suggestive of infections but should be considered, especially when cytopenia and elevated triglyceride and ferritin levels are present.