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We report the case of a 68\u2010year\u2010old man which presented an exceptional association of cutaneous<jats:styled-content style=\"fixed-case\">LCH<\/jats:styled-content>and<jats:styled-content style=\"fixed-case\">RDD<\/jats:styled-content>and splenic marginal zone lymphoma. He was stable for few years. Suddenly, the patient was admitted into Hematology Department with a remarkable enlargement of spleen and liver without enlargement of lymphadenopathies or skin lesions flare. He died 24\u2009h later despite treatment with systemic chemotherapy combined with prednisone. Pre\u2010mortem biopsy showed infiltration with histiocytic sarcoma. We think that a transdifferentiation phenomenon could explain our case, although we could not show a clonal relationship between the cutaneous and the liver diseases. We also want to pay attention to the fact that a fast transformation to a more aggressive disease can occur long time after the presentation of the first lesion, a problem that stresses the importance of performing a close and permanent follow\u2010up of these patients.<\/jats:p>","DOI":"10.1111\/cup.12013","type":"journal-article","created":{"date-parts":[[2012,10,8]],"date-time":"2012-10-08T13:07:43Z","timestamp":1349701663000},"page":"1125-1130","source":"Crossref","is-referenced-by-count":16,"title":["Coexistence of Langerhans cell histiocytosis, Rosai\u2013Dorfman disease and splenic lymphoma with fatal outcome after rapid development of histiocytic sarcoma of the liver"],"prefix":"10.1111","volume":"39","author":[{"given":"Mar","family":"Llamas\u2010Velasco","sequence":"first","affiliation":[{"name":"Department of Dermatology Hospital Universitario de La Princesa Madrid 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