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Impact of World Health Organization (WHO) Revised Criteria-2016 on the Diagnosis of Polycythemia Vera

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Abstract

The diagnosis of polycythemia vera (PV) requires the integration of clinical and laboratory findings, bone marrow morphologic features, and JAK2 analysis. JAK2V617F (exon 14) mutation is found in 95% of PV cases. In PV, addition of characteristic bone marrow morphology as one of three major diagnostic criteria allowed reduced hemoglobin/hematocrit threshold for diagnosis to 16.5 g/dL/49% in men and 16 g/dL/48% in women. JAK2 mutation is still the third major diagnostic criterion in PV. Low serum erythropoietin level is now considered as minor criterion in PV and is used to detect cases, which are negative for JAK2 mutation. In this retrospective study, cases diagnosed as PV from January 2013 to December 2015 were reclassified using WHO 2016 criteria. Their clinical and laboratory parameters along with treatment and outcome were studied. Out of 26 patients of previously diagnosed PV, either definitively or provisionally, twenty-one were found to comply with the new 2016 revision of the WHO Criteria. Median age was 55.5 years, with a male preponderance. The median values of hemoglobin, hematocrit and platelets were 17.5 gm/dL, 56.7% and 493 × 109/L, respectively. JAK2V617F was mutated in 17 cases. Bone marrow showed hypercellularity, panmyelosis and marked megakaryocyte dyspoiesis in all patients. All patients had normal oxygen saturation, confirming the primary nature of the disease. Our study, first of its kind in India, underscores the importance of the 2016 revision of the WHO document in detecting cases of masked PV.

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Funding

This study has not received any funding from any agency.

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Authors and Affiliations

  1. Department of Pathology, Rajiv Gandhi Cancer Institute and Research Center, Rohini, New Delhi, 110085, India

    Shrinidhi Nathany

  2. Department of Pathology, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Udupi, Karnataka, 576104, India

    Sindhura Lakshmi Koulmane Laxminarayana, Siddharth Tewari, Sushma Belurkar, Ruchee Khanna & Chethan Manohar

Authors
  1. Shrinidhi Nathany
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  2. Sindhura Lakshmi Koulmane Laxminarayana
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  3. Siddharth Tewari
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  4. Sushma Belurkar
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  5. Ruchee Khanna
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  6. Chethan Manohar
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Contributions

SN: reviewed slides, wrote the manuscript. SL: involved in initial diagnosis, jointly prepared manuscript and critically reviewed the manuscript and is the corresponding author, also reviewed data of all patients from laboratory archives and verified the accuarcy of the data. ST: collected data from patient records. SB, RK: faculty involved in initial diagnosis. CM: mentor guide for the project, reviewed slides and manuscript.

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Correspondence to Sindhura Lakshmi Koulmane Laxminarayana.

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The authors declare that they have no conflict of interest.

Ethical Standards

The study was reviewed and approved by institutional committee at Kasturba Medical College, Manipal. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.

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Nathany, S., Koulmane Laxminarayana, S.L., Tewari, S. et al. Impact of World Health Organization (WHO) Revised Criteria-2016 on the Diagnosis of Polycythemia Vera. Indian J Hematol Blood Transfus 36, 477–483 (2020). https://doi.org/10.1007/s12288-019-01202-w

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  • DOI: https://doi.org/10.1007/s12288-019-01202-w

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