MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia

for AIRI, Associazione Italiana Ricerca Istiocitosi ONLUS

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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.

Original languageEnglish
Pages (from-to)637-643
Number of pages7
JournalJournal of Cutaneous Pathology
Volume48
Issue number5
DOIs
Publication statusPublished - May 2021

Keywords

  • acute myeloid leukemia
  • clonal relation
  • Erdheim-Chester disease
  • Langerhans cell histiocytosis
  • Rosai-Dorfman-Destombes disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

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