Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, FL, United States.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
Leuk Res. 2020 Dec;99:106460. doi: 10.1016/j.leukres.2020.106460. Epub 2020 Oct 6.
Myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement are a unique category in the WHO classification, and include cases with rearrangement of PDGFRA, PDGFRB, FGFR1, and PCM1-JAK2. We report three patients presented with eosinophilia and FLT3 rearrangement: the first case with chronic eosinophilic leukemia, not otherwise specified and T-lymphoblastic leukemia/lymphoma; the second case with myeloid sarcoma; and the last case with high-grade myelodysplastic syndrome. The first case showed t(13;14)(q12;q32), which encoded FLT3-TRIP11. The patient was treated with intense chemotherapy and subsequently sorafenib with clinical improvement. Unfortunately, the patient showed persistent residual disease and passed away 9 months after the diagnosis from pneumonia. The other two cases both showed ETV6-FLT3. The second patient was treated with local radiation and systemic chemotherapy including sorafenib and was alive. The third patient was treated with chemotherapy but showed transformation to acute myeloid leukemia and died 15 months after diagnosis. These cases are among a growing number of cases with FLT3 rearrangement that all showed similar clinicopathologic features characterized by myeloproliferative neoplasm with eosinophilia and frequent T lymphoblastic leukemia/lymphoma. Therefore, we propose that the myeloid/lymphoid neoplasms with eosinophilia and FLT3 rearrangement be included in the WHO category of myeloid/lymphoid neoplasms with eosinophilia and gene rearrangement.
伴嗜酸性粒细胞增多和基因重排的髓系/淋系肿瘤是世界卫生组织分类中的一个独特类别,包括 PDGFRA、PDGFRB、FGFR1 和 PCM1-JAK2 重排的病例。我们报告了三例伴有嗜酸性粒细胞增多和 FLT3 重排的患者:第一例为慢性嗜酸性粒细胞白血病,非特指型和 T 淋巴母细胞白血病/淋巴瘤;第二例为髓系肉瘤;最后一例为高级别骨髓增生异常综合征。第一例表现为 t(13;14)(q12;q32),编码 FLT3-TRIP11。患者接受了强化化疗,随后接受了索拉非尼治疗,临床状况得到改善。不幸的是,患者仍有持续性残留疾病,并在诊断后 9 个月因肺炎去世。另外两例均显示 ETV6-FLT3。第二位患者接受了局部放疗和包括索拉非尼在内的全身化疗,目前仍存活。第三位患者接受了化疗,但转化为急性髓系白血病并在诊断后 15 个月死亡。这些病例是越来越多伴有 FLT3 重排的病例之一,所有病例均表现出相似的临床病理特征,表现为伴嗜酸性粒细胞增多的髓系增殖性肿瘤和频繁的 T 淋巴母细胞白血病/淋巴瘤。因此,我们建议将伴嗜酸性粒细胞增多和 FLT3 重排的髓系/淋系肿瘤纳入世界卫生组织伴嗜酸性粒细胞增多和基因重排的髓系/淋系肿瘤类别中。