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
Subjects
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
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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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  fullname: Kimura, Akiro
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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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