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

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. (J Am Acad Dermatol 2011;65:404-10.)