Complete and long-lasting cytologic and molecular remission of FIP1L1-PDGFRA-positive acute eosinophil myeloid leukaemia, treated with low-dose imatinib monotherapy.
Myeloproliferative neoplasms associated with FIP1L1-PDGFR rearrangements represent a rare subset of myeloid and lymphoid malignancies, characterised by the presence of eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1 genes. The fusion product of such genes is a tyrosine kinase oncoprotein sensitive to imatinib, which to date results to be the standard of care for FIP1L1-PDGFRA-positive chronic myeloproliferative disorders with eosinophilia. However, the coexistence of FIP1L1-PDGFRA rearrangement associated with acute myeloid leukaemia is extremely rare. Here, we report a rare case of FIP1L1-PDGFRA-positive acute myeloid leukaemia, with marked peripheral blood and bone marrow eosinophilia, treated with low dose of imatinib monotherapy, achieving a rapid and long-lasting complete cytologic and molecular remission, without need for intensive chemotherapy.
Division of Haematology and Bone Marrow Transplantation, Azienda Ospedaliero Universitaria SM Misericordia, University of Udine, Udine, Italy.
Daniela
D
Barraco
Francesca
F
Carobolante
Anna
A
Candoni
Erica
E
Simeone
Pierpaolo
P
Piccaluga
Valentina
V
Tabanelli
Renato
R
Fanin
D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001549:Benzamides / Q000008:administration & dosage*; D015415:Biomarkers / Q000378:metabolism; D001853:Bone Marrow / Q000473:pathology; D001842:Bone and Bones / Q000473:pathology; D006801:Humans; D000068877:Imatinib Mesylate; D015472:Leukemia, Eosinophilic, Acute / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics*; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010879:Piperazines / Q000008:administration & dosage*; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage*; D011743:Pyrimidines / Q000008:administration & dosage*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics*; D016896:Treatment Outcome; D039221:mRNA Cleavage and Polyadenylation Factors / Q000235:genetics*
D000970:Antineoplastic Agents; D001549:Benzamides; D015415:Biomarkers; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha
Acute myeloid leukaemia; FIP1L1-PDGFRA fusion gen; eosinophilia; imatinib