Olverembatinib for 8p11 Myeloproliferative Syndrome with a Positive BCR-FGFR1 Fusion Gene - A Case Report

Sep 29, 2026

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8p11 myeloproliferative syndrome (EMS) is a rare aggressive hematologic malignancy that can rapidly progress to acute leukemia and is marked by translocation involving fibroblast growth factor receptor-1 (FGFR1). Effective, reliable treatment options remain limited. This case report documents an EMS patient with a positive BCR-FGFR1 fusion gene whose clinical manifestations resemble chronic myeloid leukemia. Diagnosis used FISH and RNA sequencing, followed by olverembatinib therapy, achieving partial molecular response after over one year with hematologic and cytogenetic remissions and excellent safety.

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Abstract

Introduction

Case description

Laboratory and diagnostic evaluation

Treatment and follow-up outcomes

Annals of Hematology (2025) 104:6403–6410 [https://doi.org/10.1007/s00277-025-06522-8](https://doi.org/10.1007/s00277-025-06522-8) CASE REPORT Olverembatinib for 8p11 myeloproliferative syndrome with a positive BCR-FGFR1 fusion gene: a case report Man Wu1 · Lin Huang1 · Yibin Yao1 · Yuling Xu1 · Yang Yang1 · Jun Luo1 · Zhenfang Liu1,2 Received: 5 June 2025 / Accepted: 17 July 2025 / Published online: 9 December 2025 © The Author(s) 2025 Abstract 8p11 myeloproliferative syndrome (EMS) is a rare aggressive hematologic malignancy with a poor prognosis that can rapidly develop into acute leukemia. It is characterized by the translocation of fibroblast growth factor receptor-1 (FGFR1), and there is still a lack of effective and reliable treatment methods at present. This report provides a new therapeutic strategy for EMS patients diagnosed with BCR-FGFR1 fusion. This report describes a case of EMS patient with a positive BCR-FGFR1 fusion gene, whose manifestations are similar to those of chronic myeloid leukemia (CML) . After diagnosis by fluorescence in situ hybridization (FISH) and RNA sequencing (RNA-seq), olverembatinib, the third-generation tyrosinase inhibitor (TKI) developed in China, was used for treatment. After monotherapy and follow-up for more than one year, partial molecular response (PR) was achieved. During this period, hematologic remission and cytogenetic remission were achieved. The treatment safety of the entire process was excellent. In summary, olverembatinib provides more treatment options for rare diseases such as 8p11 myeloproliferative syndrome. Keywords Olverembatinib · 8p11 myeloproliferative syndrome · EMS · FGFR1 Introduction 8p11 myeloproliferative syndrome (EMS) is a very rare aggressive hematologic malignancy. To date, only aroud a hundred cases have been reported in the literature [1] . Its pathogenesis is related to the rearrangement of fibroblast growth factor receptor 1 (FGFR1) in myeloid/lymphoid tumors. EMS patients usually present with myeloproliferative neoplasms with lymphoblastic lymphoma, with or without eosinophilia. The prognosis of this disease is poor and it can develop into acute leukemia within a short period of time [2, 3] . For EMS patients, the current treatment consensus is that allogeneic hematopoietic stem cell transplantation Man Wu and Lin Huang contributed equally to this work. 􀀍 Zhenfang Liu [liuzhenfang@gxmu.edu.cn](liuzhenfang@gxmu.edu.cn) (allo-HSCT) is the only curative treatment method. Although many compounds have been shown to inhibit the activity of the FGFR1 fusion protein [4–6], no drug that can be truly applied in clinical treatment has emerged yet. Olverembatinib is a third-generation tyrosine kinase inhibitor TKI developed in China, targeting BCR::ABL1 [7] and also inhibiting other tumor-associated kinases, including FLT3, PDGFR and FGFR1 [8, 9]. In recent years, olverembatinib has demonstrated good efficacy and safety in clinical studies of various hematologic malignancies. On the basis of its inhibitory activity against FGFR1, we attempted to apply olverembatinib to EMS patients carrying the BCRFGFR1 fusion gene. Excitingly, hematologic remission was achieved after 7 months of treatment and cytogenetic remission was achieved after 13 months. After more than one year of follow-up, the patient’s therapeutic effect was evaluated as partial response (PR). The patient has always maintained an effective treatment state and the drug has good safety. 1 Hematology Department, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China 2 Key Laboratory of Hematology, Department of Guangxi Zhuang Autonomous Region, Guangxi Medical University, Education, Nanning, Guangxi, China Table 1 Blood routine examination | | At local
hospital | At
admission | Follow-up | Normal range | | --- | --- | --- | --- | --- | | WBC, n(×109/L) | 161.04 | 8.78 | 7.8 | (4–10) | | Hb, n (g/L) | 101 | 109.1 | 145 | (120–

FAQs

What is 8p11 myeloproliferative syndrome (EMS) and why is treatment challenging?

EMS is a rare aggressive hematologic malignancy with poor prognosis that may rapidly develop into acute leukemia. It is characterized by FGFR1 translocation, and effective reliable treatments are still lacking.

How was the patient diagnosed as having BCR-FGFR1 fusion-positive EMS?

The diagnosis used fluorescence in situ hybridization (FISH) and RNA sequencing (RNA-seq), after which the case was classified as EMS with a positive BCR-FGFR1 fusion gene.

What treatment was used and what responses were achieved?

Olverembatinib was used as therapy. After monotherapy and follow-up for more than one year, the patient achieved partial molecular response, with hematologic remission and cytogenetic remission during the course.

Sep 29, 2026

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