Efficacy of imatinib mesylate (STI571) in chronic neutrophilic leukemia with t(15;19): case report.

Abstract

Chronic neutrophilic leukemia (CNL) is a rare hematologic disorder, for which there is no standard therapy. Recently, STI (imatinib mesylate) has been shown to be effective in treating patients with chronic myeloproliferative disorder (CMPD) displaying the translocation of the PDGFbetaR gene. Here, we present a case of a patient with CNL carrying t(15;19)(q13;p13.3) who achieved a cytogenetic remission following treatment with imatinib, 400 mg daily. After failure of alpha interferon and hydroxyurea therapy, a durable and complete clinical and cytogenic remission was induced by imatinib. To our knowledge, this is the first case with CNL who showed complete response with cytogenic remission after treatment of imatinib. The mechanism of response to this molecule is unknown in our case (other oncogenes than c-kit, tyrosine kinase, or PDGFR may be involved). The patient remains in complete remission with an excellent performance status after 7 months of therapy. We demonstrate here that imatinib can induce a clinical and cytogenetic response in a case of CNL associated with a novel translocation other than a 5q33 rearrangement. Further studies including the molecular cloning of the t(15;19)(q13;p13.3) will be important in understanding the pathophysiology of CNL with a heterogeneous clinical course and the exploitation of the basic mechanisms of imatinib treatment.

• Authors: In Keun Choi, Byung-Soo Kim, Kyung-A Lee, Sookwon Ryu, Hee Yun Seo, Hyeryoung Sul, Jong Gwon Choi, Hwa Jung Sung, Kyong Hwa Park, So Young Yoon, Sang Cheul Oh, Jae Hong Seo, Chul Won Choi, Sang Won Shin, Soo-Young Yoon, Yunjung Cho, Young-Kee Kim, Yeul Hong Kim, Jun Suk Kim
• Journal: American journal of hematology (Am J Hematol) Vol. 77 Issue 4 Pg. 366-9 (Dec 2004) ISSN: 0361-8609 [Print] United States
• PMID: 15551277 (Publication Type: Case Reports, Journal Article)
• Copyright: 2004 Wiley-Liss, Inc.

Chemical References

• Antineoplastic Agents
• Benzamides
• Piperazines
• Pyrimidines
• Imatinib Mesylate
• Receptor, Platelet-Derived Growth Factor beta

Topics

• Antineoplastic Agents (administration & dosage, therapeutic use)
• Benzamides
• Biopsy, Needle
• Bone Marrow (pathology)
• Humans
• Imatinib Mesylate
• Leukemia, Neutrophilic, Chronic (blood, drug therapy, genetics, pathology)
• Leukocyte Count
• Male
• Middle Aged
• Neutrophils (pathology)
• Piperazines (administration & dosage, therapeutic use)
• Pyrimidines (administration & dosage, therapeutic use)
• Receptor, Platelet-Derived Growth Factor beta (genetics)
• Remission Induction
• Translocation, Genetic
• Treatment Outcome