Background: Myelodysplastic syndromes with chromosome 5 long arm deletion (5q-MDS) may benefit from lenalidomide treatment. However, unresponsive patients have a high risk for clonal evolution and progression to acute myeloid leukemia. Case: We describe a 5q-patient treated with lenalidomide, who concomitantly developed acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm, a rare and highly aggressive lymphoma. Conclusions: Evolution of 5q-syndrome to acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm may have occurred through various mechanisms, including persistence of neoplastic lenalidomide-resistant stem cells and selection of a more aggressive clone via lenalidomide augmentation of the ARPC1B gene, or because of lenalidomide stimulation on dendritic cells. Further studies are needed to clarify lenalidomide oncogenic potential.

Concomitant Occurrence of Blastic Plasmacytoid Dendritic Cell Neoplasm and Acute Myeloid Leukaemia after Lenalidomide Treatment for del(5q) Myelodysplastic Syndrome / N.S. Fracchiolla, A. Iurlo, V. Ferla, B. Fattizzo, A. Freyrie, G. Reda, A. Cortelezzi. - In: CLINICAL LABORATORY. - ISSN 1433-6510. - 63:9(2017 Sep 01), pp. 1513-1517. [10.7754/Clin.Lab.2017.170322]

Concomitant Occurrence of Blastic Plasmacytoid Dendritic Cell Neoplasm and Acute Myeloid Leukaemia after Lenalidomide Treatment for del(5q) Myelodysplastic Syndrome

N.S. Fracchiolla;V. Ferla;B. Fattizzo;G. Reda;A. Cortelezzi
2017

Abstract

Background: Myelodysplastic syndromes with chromosome 5 long arm deletion (5q-MDS) may benefit from lenalidomide treatment. However, unresponsive patients have a high risk for clonal evolution and progression to acute myeloid leukemia. Case: We describe a 5q-patient treated with lenalidomide, who concomitantly developed acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm, a rare and highly aggressive lymphoma. Conclusions: Evolution of 5q-syndrome to acute myeloid leukemia and blastic plasmacytoid dendritic cell neoplasm may have occurred through various mechanisms, including persistence of neoplastic lenalidomide-resistant stem cells and selection of a more aggressive clone via lenalidomide augmentation of the ARPC1B gene, or because of lenalidomide stimulation on dendritic cells. Further studies are needed to clarify lenalidomide oncogenic potential.
myelodysplastic syndromes; blastic plasmacytoid dendritic cell neoplasm; acute myeloid leukaemia; lenalidomide
Settore MED/15 - Malattie del Sangue
1-set-2017
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/551189
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