Ng Heng Joo, Tan Daryl C L, Yiu Richard C, How Gee Fung
Department of Haematology, Singapore General Hospital, Outram Road, Singapore 169608, Singapore.
Leuk Res. 2008 Jan;32(1):169-71. doi: 10.1016/j.leukres.2007.04.004. Epub 2007 Jun 4.
Patients with primary hypereosinophilic disorders who are positive for the FIP1L1-PDGFRA fusion gene mutation are highly responsive to therapy with imatinib mesylate. A 35-year-old man with FIP1L1-PDGFRA positive hypereosinophilic syndrome and cardiac involvement, was treated with imatinib 100 mg daily. Hematologic and molecular remission and reversal of end-organ damage was achieved. He was then lost to follow up for 19 months. Imatinib successfully reinduced hematologic and molecular remission but worsening cardiac involvement was not reversed. Our experience and a review of limited literature suggest that imatinib maintenance therapy is necessary despite molecular remission of the FIP1L1-PDGFRA mutation.
携带FIP1L1-PDGFRA融合基因突变阳性的原发性嗜酸性粒细胞增多症患者对甲磺酸伊马替尼治疗反应高度敏感。一名35岁男性，患有FIP1L1-PDGFRA阳性嗜酸性粒细胞增多综合征并累及心脏，接受每日100mg伊马替尼治疗。实现了血液学和分子学缓解以及终末器官损伤的逆转。随后他失访了19个月。伊马替尼成功再次诱导了血液学和分子学缓解，但心脏受累情况恶化未得到逆转。我们的经验以及对有限文献的回顾表明，尽管FIP1L1-PDGFRA突变实现了分子学缓解，但伊马替尼维持治疗是必要的。
