MAP2K1‐driven mixed Langerhans cell histiocytosis, Rosai‐Dorfman‐Destombes disease and Erdheim‐Chester disease, clonally related to acute myeloid leukemia. (24th November 2020)

• Record Type: Journal Article
• Authors: Bonometti, Arturo; Ferrario, Giuseppina; Parafioriti, Antonina; Giardino, Demostene; Simonetti, Federico; Ginori, Alessandro; Passoni, Emanuela; Berti, Emilio
• Is Part Of: Journal of cutaneous pathology. Volume 48:Number 5(2021)
• Page Start: 637
• Page End: 643
• Publication Date: 2020-11-24
• Subjects: Langerhans cell histiocytosis -- Rosai‐Dorfman‐Destombes disease -- Erdheim‐Chester disease -- clonal relation -- acute myeloid leukemia; Skin -- Diseases -- Periodicals; Dermatology -- Periodicals; 616
• DOI: 10.1111/cup.13918
• Languages: English
• ISSNs: 0303-6987

Abstract

Abstract: Mixed histiocytoses are a rare and recently recognized subset of histiocytic disorders that may involve the skin, characterized by the synchronous or metachronous development of lesions with Langerhans and/or non‐Langerhans cell histiocytosis histopathological features. Around 10% of patients diagnosed with histiocytosis may develop a hematological malignancy, often with dramatic prognostic consequences. We hereby describe the exceptional case of a patient developing a MAP2K1‐driven mixed histiocytosis with Langerhans cell histiocytosis, Rosai‐Dorfman‐Destombes disease, and Erdheim‐Chester disease features and cutaneous involvement, progressing to a fatal and clonally‐related acute myeloid leukemia. We reviewed the literature on similar cases and discussed the histopathological difficulties in their diagnosis and their clinical‐pathological features.