| Autor: |
Musto P; Scientific Direction, Italy., Simeon V; Laboratory of Preclinical and Translational Research, Italy., Guariglia R; Unit of Hematology and Stem Cell Transplantation, Italy., Bianchino G; Laboratory of Clinical Research and Advanced Diagnostics, Italy., Grieco V; Laboratory of Clinical Research and Advanced Diagnostics, Italy., Nozza F; Laboratory of Clinical Research and Advanced Diagnostics, Italy., La Rocca F; Laboratory of Preclinical and Translational Research, Italy., Marziano G; Scientific Direction, Italy., Lalinga AV; Pathology Unit, IRCCS, Centro di Riferimento Oncologico della Basilicata, Rionero in Vulture, Italy., Fabiani E; Department of Hematology, Universita Cattolica del Sacro Cuore, Rome, Italy., Voso MT; Department of Hematology, Universita Cattolica del Sacro Cuore, Rome, Italy., Scaravaglio P; Laboratory of Internal Medicine and Hematology, S Luigi Gonzaga Hospital, Orbassano, Italy., Mecucci C; Hematology and Bone Marrow Transplantation Unit, University of Perugia, Perugia, Italy., D'Arena G; Unit of Hematology and Stem Cell Transplantation, Italy. |
| Abstrakt: |
The concomitant presence of del(5q) and JAK2(V617F) mutation is an infrequent event which occurs in rare patients with peculiar cytogenetic, molecular, morphological and clinical features, resembling those of both myelodysplastic syndromes and myeloproliferative neoplasms. Lenalidomide may induce rapid, profound, and long-lasting responses in a subset of these patients. However, the mechanism(s) by which the drug acts in these conditions remain not completely elucidated. A new case report and a review of all cases published so far in this setting are provided. Furthermore, the possibility of categorizing - from a clinical, pathological, and biological point of view - for at least some of these patients as a potential distinct entity is discussed. |
