TY - JOUR
T1 - Myelodysplastic disorders carrying both isolated del(5q) and JAK2(V617F) mutation: concise review, with focus on lenalidomide therapy
AU - Fabiani, Emiliano
AU - Voso, Maria Teresa
AU - Musto, Pellegrino
AU - Simeon, Vittorio
AU - Guariglia, Roberto
AU - Bianchino, Gabriella
AU - Grieco, Vitina
AU - Nozza, Filomena
AU - La Rocca, Francesco
AU - Marziano, Gioacchino
AU - Lalinga, Anna Vittoria
AU - Scaravaglio, Patrizia
AU - Mecucci, Cristina
AU - D'Arena, Giovanni
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - JAK2
KW - del(5q)
KW - JAK2
KW - del(5q)
UR - http://hdl.handle.net/10807/60305
U2 - 10.2147/OTT.S59628
DO - 10.2147/OTT.S59628
M3 - Article
SP - 1043
EP - 1050
JO - Onco Targets Ther
JF - Onco Targets Ther
ER -