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Myeloproliferative hypereosinophilic syndrome

Last edited: 4/14/2026

Overview

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by persistent eosinophilia exceeding 1500/mm3 for more than six months, often leading to multi-organ damage without a definitive underlying cause 13.

Diagnosis

  • Key Diagnostic Criteria: Persistent peripheral blood eosinophilia >1500/mm3 on at least two occasions 3.
  • Recommended Tests: Comprehensive evaluation including cardiac, pulmonary, and gastrointestinal assessments to identify organ involvement 178.
  • Histopathological Findings: Skin biopsies may show nonspecific dermal infiltrates with eosinophils; notable findings include cutaneous microthrombi 13.
  • Genetic Testing: Consider molecular analysis for specific mutations (e.g., FIP1L1-PDGFRα) in suspected cases of chronic eosinophilic leukemia 4.
  • Differential Diagnosis: Rule out secondary causes of eosinophilia such as parasitic infections, malignancies, and allergic disorders 1.
  • Management

  • First-Line Treatments:
  • - Glucocorticoids: Prednisone is often used initially to control symptoms and reduce eosinophil counts 716. - Tyrosine Kinase Inhibitors: Imatinib for cases with FIP1L1-PDGFRα fusion gene 4.
  • Adjunctive Treatments:
  • - Vincristine: Used in combination with glucocorticoids for refractory cases 11. - Immunosuppressive Agents: Consider in severe or refractory cases, though specific agents are not detailed in abstracts 1.

    Special Populations

  • Pediatrics: Cases may involve chromosomal abnormalities like trisomy 8, which can predict malignant transformation 9.
  • Comorbidities: Patients with systemic lupus erythematosus can develop HES presenting with gastrointestinal symptoms and anemia 7.
  • Elderly and Pregnancy: Specific management guidelines are not detailed in the provided abstracts; individualized care based on organ involvement is recommended 116.
  • Key Recommendations

  • Prompt Diagnosis and Early Intervention: Shorten diagnostic delay to mitigate organ damage 1. (Evidence: Moderate)
  • Use of Glucocorticoids as Initial Therapy: Initiate with prednisone to control eosinophilia and alleviate symptoms 716. (Evidence: Moderate)
  • Consider Molecular Testing for Specific Mutations: Evaluate for genetic markers like FIP1L1-PDGFRα in chronic eosinophilic leukemia 4. (Evidence: Weak)
  • Monitor for Multi-Organ Involvement: Regular assessments of cardiac, pulmonary, and gastrointestinal systems are crucial 18. (Evidence: Expert opinion)
  • References

    1 Caminati M, Brussino L, Carlucci M, Carlucci P, Carpagnano LF, Caruso C et al.. Managing Patients with Hypereosinophilic Syndrome: A Statement from the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC). Cells 2024. link 2 Wu P, Chen Y, Zhang H, Li C. Idiopathic hypereosinophilic syndrome presenting with multiple organ damage: A case report. Medicine 2019. link 3 Mittal A, Ray A, Jadon RS, Manchanda S, Naik SR, Singh K et al.. Hypereosinophilia with Multi-organ Dysfunction - A Diagnostic Conundrum. The Journal of the Association of Physicians of India 2018. link 4 Ortiz C, Jimenez M, Matos NA. Chronic eosinophilic leukemia: a rare cause of hypereosinophilic syndrome. Boletin de la Asociacion Medica de Puerto Rico 2014. link 5 Jayakar V, Goldin RD, Bain BJ. Case 31: Eosinophilia and pruritus. Leukemia & lymphoma 2006. link 6 Marks AJ, Abdalla SH, Cross NC, Bain BJ. Case 30: hypereosinophilia. Leukemia & lymphoma 2006. link 7 Markusse HM, Schravenhoff R, Beerman H. Hypereosinophilic syndrome presenting with diarrhoea and anaemia in a patient with systemic lupus erythematosus. The Netherlands journal of medicine 1998. link00086-7) 8 Rauch AE, Amyot KM, Dunn HG, Ng B, Wilner G. Hypereosinophilic syndrome and myocardial infarction in a 15-year-old. Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association 1997. link 9 Guitard AM, Horschowski N, Mozziconacci MJ, Michel G, George F, Capodano AM et al.. Hypereosinophilic syndrome in childhood: trisomy 8 and transformation to mixed acute leukaemia. Nouvelle revue francaise d'hematologie 1994. link 10 Niikawa N, Deng HX, Abe K, Harada N, Okada T, Tsuchiya H et al.. Possible mapping of the gene for transient myeloproliferative syndrome at 21q11.2. Human genetics 1991. link 11 Lederman J, Hasselbalch H, Hippe E. Alcohol intolerance in the hypereosinophilic syndrome. Alcoholism, clinical and experimental research 1988. link 12 Shah AM, Joglekar M. Eosinophilic colitis as a complication of the hypereosinophilic syndrome. Postgraduate medical journal 1987. link 13 Fitzpatrick JE, Johnson C, Simon P, Owenby J. Cutaneous microthrombi: a histologic clue to the diagnosis of hypereosinophilic syndrome. The American Journal of dermatopathology 1987. link 14 Thompson AB, Barron MM, Lapp NL. The hypereosinophilic syndrome presenting with eosinophilic mastitis. Archives of internal medicine 1985. link 15 Lanham JG, Cooke S, Davies J, Hughes GR. Endomyocardial complications of the Churg-Strauss syndrome. Postgraduate medical journal 1985. link 16 Tønnesen P, Teglbjaerg CS. An "unexpected" fatal case of the hypereosinophilic syndrome. European journal of respiratory diseases 1984. link 17 Epstein DM, Taormina V, Gefter WB, Miller WT. The hypereosinophilic syndrome. Radiology 1981. link 18 Adams HW, Mainz DL. Eosinophilic ascites. A case report and review of the literature. The American journal of digestive diseases 1977. link

    Original source

    1. [1]
      Managing Patients with Hypereosinophilic Syndrome: A Statement from the Italian Society of Allergy, Asthma, and Clinical Immunology (SIAAIC).Caminati M, Brussino L, Carlucci M, Carlucci P, Carpagnano LF, Caruso C et al. Cells (2024)
    2. [2]
    3. [3]
      Hypereosinophilia with Multi-organ Dysfunction - A Diagnostic Conundrum.Mittal A, Ray A, Jadon RS, Manchanda S, Naik SR, Singh K et al. The Journal of the Association of Physicians of India (2018)
    4. [4]
      Chronic eosinophilic leukemia: a rare cause of hypereosinophilic syndrome.Ortiz C, Jimenez M, Matos NA Boletin de la Asociacion Medica de Puerto Rico (2014)
    5. [5]
      Case 31: Eosinophilia and pruritus.Jayakar V, Goldin RD, Bain BJ Leukemia & lymphoma (2006)
    6. [6]
      Case 30: hypereosinophilia.Marks AJ, Abdalla SH, Cross NC, Bain BJ Leukemia & lymphoma (2006)
    7. [7]
      Hypereosinophilic syndrome presenting with diarrhoea and anaemia in a patient with systemic lupus erythematosus.Markusse HM, Schravenhoff R, Beerman H The Netherlands journal of medicine (1998)
    8. [8]
      Hypereosinophilic syndrome and myocardial infarction in a 15-year-old.Rauch AE, Amyot KM, Dunn HG, Ng B, Wilner G Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association (1997)
    9. [9]
      Hypereosinophilic syndrome in childhood: trisomy 8 and transformation to mixed acute leukaemia.Guitard AM, Horschowski N, Mozziconacci MJ, Michel G, George F, Capodano AM et al. Nouvelle revue francaise d'hematologie (1994)
    10. [10]
      Possible mapping of the gene for transient myeloproliferative syndrome at 21q11.2.Niikawa N, Deng HX, Abe K, Harada N, Okada T, Tsuchiya H et al. Human genetics (1991)
    11. [11]
      Alcohol intolerance in the hypereosinophilic syndrome.Lederman J, Hasselbalch H, Hippe E Alcoholism, clinical and experimental research (1988)
    12. [12]
      Eosinophilic colitis as a complication of the hypereosinophilic syndrome.Shah AM, Joglekar M Postgraduate medical journal (1987)
    13. [13]
      Cutaneous microthrombi: a histologic clue to the diagnosis of hypereosinophilic syndrome.Fitzpatrick JE, Johnson C, Simon P, Owenby J The American Journal of dermatopathology (1987)
    14. [14]
      The hypereosinophilic syndrome presenting with eosinophilic mastitis.Thompson AB, Barron MM, Lapp NL Archives of internal medicine (1985)
    15. [15]
      Endomyocardial complications of the Churg-Strauss syndrome.Lanham JG, Cooke S, Davies J, Hughes GR Postgraduate medical journal (1985)
    16. [16]
      An "unexpected" fatal case of the hypereosinophilic syndrome.Tønnesen P, Teglbjaerg CS European journal of respiratory diseases (1984)
    17. [17]
      The hypereosinophilic syndrome.Epstein DM, Taormina V, Gefter WB, Miller WT Radiology (1981)
    18. [18]
      Eosinophilic ascites. A case report and review of the literature.Adams HW, Mainz DL The American journal of digestive diseases (1977)

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