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Screening for paroxysmal nocturnal hemoglobinuria (PNH) in patients presenting with cerebral sinovenous thrombosis (CSVT): Results of a FLAER based flowcytometry study in Indian patients

  • Saniya Sharma
  • Jasmina AhluwaliaEmail author
  • Charu Batra Atreja
  • Manish Modi
  • Manupdesh Singh Sachdeva
  • Narender Kumar
  • Parveen Bose
  • Sunil Kumar Bose
  • Varun Uppal
  • Vivek Lal
  • Neelam Varma
Article
  • 38 Downloads

Abstract

Patients with paroxysmal nocturnal hemoglobinuria (PNH) may present with thrombosis at unusual sites, of which cerebral sinovenous thrombosis (CSVT) is one and screening for PNH is recommended in this condition. Though many patients diagnosed with PNH develop CSVT, it is unclear how many patients with PNH would present for the first time with thrombosis. We analysed the results of screening for PNH by flowcytometry in our patients with CSVT. The laboratory data of patients referred for thrombophilia and PNH testing in CSVT was examined to assess the frequency of PNH at presentation in these patients. FLAER and CD24 on granulocytes and FLAER and CD14 on monocytes respectively were used to screen the leucocytes for PNH by flowcytometry. The data for Protein C, S and Antithrombin deficiency, antiphospholipid antibodies and the Factor V Leiden mutation was examined and circumstantial risk factors were also assessed. Of the 180 cases of CSVT screened by flowcytometry for PNH, not a single case tested positive. Positivity for anti-phospholipid antibodies was the most common thrombophilic risk factor (5%). Pregnancy was the most common circumstantial risk factor. Our data on FLAER based flowcytometry in the North Indian population with CSVT suggests that PNH is not a common risk factor in our patients with thrombosis at this unusual site.

Keywords

Cerebral sinovenous thrombosis Paroxysmal nocturnal hemoglobinuria FLAER flow cytometry Antiphospholipid antibodies 

Notes

Acknowledgements

The help rendered by Dr Harpreet Virk in data retrieval is gratefully acknowledged.

Compliance with ethical standards

The study was approved by Institute Ethics Committee, Postgraduate Institute of Medical Education and Research, Chandigarh, India (IEC no. INT/IEC/2018/EIC001147).

Conflicts of interest

The authors declare that they have no conflicts of interest to declare.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Saniya Sharma
    • 1
  • Jasmina Ahluwalia
    • 1
    Email author
  • Charu Batra Atreja
    • 3
  • Manish Modi
    • 2
  • Manupdesh Singh Sachdeva
    • 1
  • Narender Kumar
    • 1
  • Parveen Bose
    • 1
  • Sunil Kumar Bose
    • 1
  • Varun Uppal
    • 1
  • Vivek Lal
    • 2
  • Neelam Varma
    • 1
  1. 1.Department of HematologyPostgraduate Institute of Medical Education and ResearchChandigarhIndia
  2. 2.Department of NeurologyPostgraduate Institute of Medical Education and ResearchChandigarhIndia
  3. 3.Department of PathologyMaharishi Markandeshwar Institute of Medical Sciences & Research ( MMIMSR ), Maharishi Markandeshwar (Deemed to be University) (MMDU)Mullana, AmbalaIndia

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