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Updated: May 8, 2026

Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome

Use of Hematopoietic Stem Cell Transplantation to Assess the Origin of Myelodysplastic Syndrome

Published on: October 3, 2018

[8p11 myeloproliferative syndrome].

Feng Li¹, Yong-Ping Zhai

¹Department of Hematology, Nanjing General Hospital, Nanjing Military Area, Nanjing 210002, Jiangsu Province, China.

Zhongguo Shi Yan Xue Ye Xue Za Zhi

|September 4, 2013

Summary

8p11 myeloproliferative syndrome (EMS), a rare leukemia, involves FGFR1 gene mutations causing aggressive T-cell lymphoma. Stem cell transplant is the only cure for this stem cell leukemia/lymphoma syndrome.

Area of Science:

Hematology
Oncology
Genetics

Context:

8p11 myeloproliferative syndrome (EMS), also known as stem cell leukemia/lymphoma syndrome, is an aggressive hematologic neoplasm.
It is characterized by specific chromosomal translocations involving the FGFR1 gene.

Purpose:

This review focuses on the molecular genetic characteristics, pathogenic mechanisms, and therapeutic strategies for EMS.
To provide an updated overview of the current understanding of this rare hematologic malignancy.

Summary:

EMS is defined by leucocytosis with eosinophilia, myeloid hyperplasia, and T-cell lymphoblastic leukemia/lymphoma.
Molecularly, it involves chimeric proteins leading to ligand-independent activation of FGFR1 tyrosine kinase, driving proliferation and malignant transformation.

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Currently, 13 translocations and 1 insertion involving the FGFR1 gene have been identified.

Impact:

Understanding the molecular basis of EMS is crucial for developing targeted therapies.
This review highlights the poor prognosis of EMS and the critical role of stem cell transplantation as the sole curative option.
Further research into FGFR1-driven pathways may reveal novel therapeutic targets for EMS and related malignancies.