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Proposed diagnostic criteria and classification of basophilic leukemias and related disorders

Leukemia volume 31, pages 788–797 (2017)Cite this article

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Abstract

Basophils form a distinct cell lineage within the hematopoietic cell family. In various myeloid neoplasms, including chronic myeloid leukemia, basophilia is frequently seen. Acute and chronic basophilic leukemias, albeit rare, have also been described. However, no generally accepted criteria and classification of basophilic leukemias have been presented to date. To address this unmet need, a series of Working Conferences and other meetings were organized between March 2015 and March 2016. The current article provides a summary of consensus statements from these meetings, together with proposed criteria to delineate acute basophilic leukemia (ABL) from chronic basophilic leukemia (CBL) and primary forms of the disease where no preceding myeloid malignancy is detected, from the more common ‘secondary’ variants. Moreover, the term hyperbasophilia (HB) is proposed for cases with a persistent peripheral basophil count ⩾1000 per μl of blood. This condition, HB, is highly indicative of the presence of an underlying myeloid neoplasm. Therefore, HB is an important checkpoint in the diagnostic algorithm and requires a detailed hematologic investigation. In these patients, an underlying myeloid malignancy is often found and is then labeled with the appendix -baso, whereas primary cases of ABL or CBL are very rare. The criteria and classification proposed in this article should facilitate the diagnosis and management of patients with unexplained basophilia and basophil neoplasms in routine practice, and in clinical studies.

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Acknowledgements

This study was supported by the Austrian Science Fund (FWF) Grant #SFB-F4704-B20. DDM was supported by the Division of Intramural Research, NIAID/NIH.

Author contributions

All co-authors contributed by establishing the concept, by participating in essential discussions, by writing parts of the document and by correcting and approving the final version of the manuscript. Consensus statements were based on a 100% agreement (all faculty members agreed) and only those statements were included as consensus in this article.

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Authors and Affiliations

  1. Department of Internal Medicine I, Division of Hematology and Hemostaseology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria

    P Valent, K Blatt, I Sadovnik, W R Sperr, K Bauer & E Hadzijusufovic

  2. Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria

    P Valent, K Blatt, W R Sperr & E Hadzijusufovic

  3. Institute of Pathology, Paracelsus Medical University Salzburg, Salzburg, Austria

    K Sotlar

  4. Department of Dermatology, University of Luebeck, Luebeck, Germany

    K Hartmann

  5. Department of Hematology and Oncology, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany

    A Reiter

  6. Division of Laboratory Medicine, Elisabethinen Hospital Linz, Linz, Austria

    P Bettelheim

  7. Division of Rheumatology, Immunology and Allergy, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA

    C Akin

  8. Department of Pathology, University of New Mexico, Albuquerque, NM, USA

    T I George

  9. Medical Clinic III for Oncology, Haematology and Rheumatology, University Hospital Bonn, Bonn, Germany

    D Wolf

  10. Stanford Cancer Institute, Stanford University School of Medicine, Stanford, CA, USA

    J Gotlib

  11. Laboratoire d'Hématologie, CHU de Bordeaux, France

    F-X Mahon

  12. Laboratory of Allergic Diseases, NIAID, NIH, Bethesda, MD, USA

    D D Metcalfe

  13. Institute of Pathology, Ludwig-Maximilians University, Munich, Germany

    H-P Horny

  14. LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France

    M Arock

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Valent, P., Sotlar, K., Blatt, K. et al. Proposed diagnostic criteria and classification of basophilic leukemias and related disorders. Leukemia 31, 788–797 (2017). https://doi.org/10.1038/leu.2017.15

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