Journal
JOURNAL OF CUTANEOUS PATHOLOGY
Volume 48, Issue 5, Pages 637-643Publisher
WILEY
DOI: 10.1111/cup.13918
Keywords
Langerhans cell histiocytosis; Rosai‐ Dorfman‐ Destombes disease; Erdheim‐ Chester disease; clonal relation; acute myeloid leukemia
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Mixed histiocytoses are a rare subset of histiocytic disorders, with around 10% of patients developing a hematological malignancy which can have severe prognostic consequences. This article describes an exceptional case of a patient with mixed histiocytosis progressing to fatal acute myeloid leukemia, and discusses the diagnostic challenges and clinical-pathological features of similar cases in the literature.
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.
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