MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia
Articolo
Data di Pubblicazione:
2020
Citazione:
MAP2K1-driven mixed Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease and Erdheim-Chester disease, clonally related to acute myeloid leukemia / A. Bonometti, G. Ferrario, A. Parafioriti, D. Giardino, F. Simonetti, A. Ginori, E. Passoni, E. Berti. - In: JOURNAL OF CUTANEOUS PATHOLOGY. - ISSN 0303-6987. - (2020). [Epub ahead of print] [10.1111/cup.13918]
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.
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Langerhans cell histiocytosis; Rosai‐ Dorfman‐ Destombes disease; Erdheim‐ Chester disease; clonal relation; acute myeloid leukemia
Elenco autori:
A. Bonometti, G. Ferrario, A. Parafioriti, D. Giardino, F. Simonetti, A. Ginori, E. Passoni, E. Berti
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