Pure red cell aplasia associated with imatinib-treated FIP1L1-PDGFRA positive chronic eosinophilic leukemia.
A 28-year-old man with marked eosinophilia is described. FIP1L1/PDGFRA mRNA showed multiple alternatively-spliced fusion transcripts. Sequencing analysis showed that the deduced DNA breakpoints were intron 10 in the FIP1L1 gene and exon 12 in the PDGFRA gene. Then, a diagnosis of chronic eosinophilic leukemia (CEL) was made. Whereas the response to the treatments with prednisolone and hydroxyurea were unsatisfactory, treatment with imatinib showed a rapid decrease of eosinophils. The hemoglobin level also dropped and bone marrow examination showed pure red cell aplasia. Continued administration of very low dose imatinib (100 mg every 5 days) led to and maintained complete molecular remission, with good tolerability.
Tanaka
Hideo
H
Department of Hematology, Hiroshima City Asa Hospital, Hiroshima, Japan.
Iwato
Koji
K
Asou
Hiroya
H
Kimura
Akiro
A
D000328:Adult; D001549:Benzamides; D002908:Chronic Disease; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D000068877:Imatinib Mesylate; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000096:biosynthesis* / Q000235:genetics; D010879:Piperazines / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000096:biosynthesis* / Q000235:genetics; D012010:Red-Cell Aplasia, Pure / Q000188:drug therapy / Q000209:etiology / Q000235:genetics*; D039221:mRNA Cleavage and Polyadenylation Factors / Q000096:biosynthesis* / Q000235:genetics
D001549:Benzamides; C479818:FIP1L1 protein, human; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha