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Indeterminate Dendritic Cell Tumor as Cutaneous Involvement of Chronic Myelomonocytic Leukemia Successfully Treated With Phototherapy

AuthorsSantos-Briz, Ángel; Medina-Miguelañez, Mario; Moyano-Bueno, David; Viñolas-Cuadros, Alex; Martínez Martínez, Teresa ; Izquierdo, Marta ; García-Sánchez, María S.
KeywordsIndeterminate dendritic cell tumor
Indeterminate cell histiocytosis
Myelomonocytic leukemia
Next-generation sequencing
Issue Date2020
PublisherLippincott Williams & Wilkins
CitationAmerican Journal of Dermatopathology 42(11): 876-880 (2020)
AbstractIndeterminate dendritic cell tumor (IDCT) is a rare disease composed of so-called indeterminate cells, a dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells, but lacking Birbeck granules. We report a case of cutaneous IDCT occurring in a patient with chronic myelomonocytic leukemia (CMML) successfully treated with UV-A phototherapy. Next-generation sequencing studies of the CMML demonstrated mutations in TET2, ASXL1, and ZRS2 genes, also detected in the IDCT, demonstrating a clonal relationship between both tumors and confirming IDCT as a specific subtype in the spectrum of CMML-related cutaneous lesions.
Publisher version (URL)http://dx.doi.org/10.1097/DAD.0000000000001703
Appears in Collections:(IBMCC) Artículos
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