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Hypereosinophilic Syndrome in Behcet’s Disease : Unearthing a Hidden Clonality


Affiliations
1 Empire Centre Haematology and Oncology Specialty Clinic, Bandra West, Mumbai, India
2 Dr Tahiliani’s Clinic, A-201/202, Gasper Enclave, Dr Ambedkar Road, Bandra West, Mumbai, India
3 Empire Centre Haematology and Oncology Specialty Clinic, Hill Road, Bandra West, Mumbai, India
     

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The association of hypereosinophilic syndrome (HES) and Behcet’s disease is not well understood. Commonly, patients are treated with standard medications for hypereosinophilia, with variable responses. However, persistent hypereosinophilia (absolute eosinophilic count >1500 cells/μL) not responding to standard treatment may be predictive of a primary clonal haematological disorder. According to the revised World Health Organization (WHO) classification, HES has six categories; one of which is myeloproliferative neoplasm (clonal eosinophilia) associated with FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFRα). We report a case of a 38-year-old Indian male with Behcet’s disease with concomitant HES, who tested positive for clonal eosinophilia (FIP1L1-PDGFRα fusion) which is a rare imatinib-sensitive mutation. The patient was administered low-dose imatinib mesylate (100 mg daily), and showed an excellent response with complete resolution of HES. The causal relationship between Bechet’s disease and HES requires further investigation and research. Left untreated, HES has the potential to cause severe end-organ damage and this necessitates early detection and treatment which can significantly reduce patient morbidity and mortality.

Keywords

Hypereosinophilia, FIP1L1-PDGFRα, Gene, Behcet’s Disease, Imatinib.
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  • Hypereosinophilic Syndrome in Behcet’s Disease : Unearthing a Hidden Clonality

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Authors

Abhay Bhave
Empire Centre Haematology and Oncology Specialty Clinic, Bandra West, Mumbai, India
Nawal Kazi
Empire Centre Haematology and Oncology Specialty Clinic, Bandra West, Mumbai, India
Sushil Tahiliani
Dr Tahiliani’s Clinic, A-201/202, Gasper Enclave, Dr Ambedkar Road, Bandra West, Mumbai, India
Lakshmi Iyer
Empire Centre Haematology and Oncology Specialty Clinic, Hill Road, Bandra West, Mumbai, India

Abstract


The association of hypereosinophilic syndrome (HES) and Behcet’s disease is not well understood. Commonly, patients are treated with standard medications for hypereosinophilia, with variable responses. However, persistent hypereosinophilia (absolute eosinophilic count >1500 cells/μL) not responding to standard treatment may be predictive of a primary clonal haematological disorder. According to the revised World Health Organization (WHO) classification, HES has six categories; one of which is myeloproliferative neoplasm (clonal eosinophilia) associated with FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFRα). We report a case of a 38-year-old Indian male with Behcet’s disease with concomitant HES, who tested positive for clonal eosinophilia (FIP1L1-PDGFRα fusion) which is a rare imatinib-sensitive mutation. The patient was administered low-dose imatinib mesylate (100 mg daily), and showed an excellent response with complete resolution of HES. The causal relationship between Bechet’s disease and HES requires further investigation and research. Left untreated, HES has the potential to cause severe end-organ damage and this necessitates early detection and treatment which can significantly reduce patient morbidity and mortality.

Keywords


Hypereosinophilia, FIP1L1-PDGFRα, Gene, Behcet’s Disease, Imatinib.

References