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- Title
Should Criteria for Polycythemia Vera be Revised in Older persons? A Case Report.
- Authors
Choudhury, Bhaskar Roy; Talukdar, Arunansu; Das, Himadri; Maity, Suman; Mondal, Partha
- Abstract
Introduction: Polycythemia Vera (PV) is a myeloproliferative neoplasm. Incidence of Polycythemia Vera in older persons is 4.1 per 100,000 per year in persons 65 yrs and above. 2016 WHO Major Diagnostic Criteria for Polycythemia Vera are 1) Hb > 16.5 g/dl (men)/>16.0 g/dl (women) or Hematocrit > 49% (men) / > 48% (women) 2) BM biopsy showing hypercellularity with panmyelosis 3) Presence of JAK2 or JAK2 exon 12 mutation. Minor Criteria is the presence of subnormal EPO level. PV diagnosis requires meeting either all three major criteria or the first two major criteria and the minor criteria. Polycythemia Vera: historical views, diagnostic details and therapeutic views by Ayalew Tefferi et al (2021) highlights the concept of masked PV. In masked PV, patients do not match the Hb and Hematocrit criteria for PV but JAK2 mutation and bone findings are present. Here, we present a similar case. Case Description: A 65-year-old man presented with gradually worsening fatigue for past six months. Physical examination revealed hepatosplenomegaly and Clinical Frailty Scale of 4. Hb level was 16.0g/dl, hematocrit was 47%, and uric acid was elevated. After excluding other causes we did bone marrow biopsy because of high suspicion though it did not meet WHO 2016 Major Diagnostic Criteria Of Polycythemia Vera. It showed erythroid precursor hyperplasia. JAK2 mutation was positive and patient had subnormal EPO level further supporting the diagnosis. He was managed with low dose aspirin and hydroxyurea. Discussion: The case highlights the importance of a clinician having a high degree of suspicion in identifying PV in frail older persons patients. It gives us a lesson that we should follow a criteria very carefully. We know that the 2016 WHO Criteria is a revision of 2007 WHO Criteria where the Hb level and hematocrit criteria were lowered from the previous one. So is it time to revise the criteria once again? Else we may miss such cases.
- Subjects
POLYCYTHEMIA vera; OLDER people; MYELOPROLIFERATIVE neoplasms; OLDER patients; BONE marrow; PURE red cell aplasia; MYELOFIBROSIS
- Publication
Journal of the Indian Academy of Geriatrics, 2023, Vol 19, Issue 3, p192
- ISSN
0974-3405
- Publication type
Case Study