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 eosinophili...

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Published inInternal Medicine Vol. 49; no. 12; pp. 1195 - 1200
Main Authors Tanaka, Hideo, Iwato, Koji, Asou, Hiroya, Kimura, Akiro
Format Journal Article
LanguageEnglish
Published Japan The Japanese Society of Internal Medicine 01.01.2010
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Abstract 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.
AbstractList 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.
Author Asou, Hiroya
Tanaka, Hideo
Kimura, Akiro
Iwato, Koji
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Snippet A 28-year-old man with marked eosinophilia is described. FIP1L1/PDGFRA mRNA showed multiple alternatively-spliced fusion transcripts. Sequencing analysis...
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StartPage 1195
SubjectTerms Adult
Benzamides
CEL
Chronic Disease
FIP1L1
Humans
Hypereosinophilic Syndrome - complications
Hypereosinophilic Syndrome - drug therapy
Hypereosinophilic Syndrome - genetics
imatinib
Imatinib Mesylate
Male
mRNA Cleavage and Polyadenylation Factors - biosynthesis
mRNA Cleavage and Polyadenylation Factors - genetics
Oncogene Proteins, Fusion - biosynthesis
Oncogene Proteins, Fusion - genetics
PDGFRA
Piperazines - therapeutic use
PRCA
Pyrimidines - therapeutic use
Receptor, Platelet-Derived Growth Factor alpha - biosynthesis
Receptor, Platelet-Derived Growth Factor alpha - genetics
Red-Cell Aplasia, Pure - drug therapy
Red-Cell Aplasia, Pure - etiology
Red-Cell Aplasia, Pure - genetics
Title Pure Red Cell Aplasia Associated with Imatinib-Treated FIP1L1-PDGFRA Positive Chronic Eosinophilic Leukemia
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