Screening for paroxysmal nocturnal hemoglobinuria (PNH) in patients presenting with cerebral sinovenous thrombosis (CSVT): Results of a FLAER based flowcytometry study in Indian patients
- 38 Downloads
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.
KeywordsCerebral sinovenous thrombosis Paroxysmal nocturnal hemoglobinuria FLAER flow cytometry Antiphospholipid antibodies
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.
- 9.Wiedmer T, Ando B, Sims PJ (1997) Complement C5b-9 stimulated platelet secretion is associated with a Ca2+ initiated activation of cellular protein kinases. J Biol Chem 262:13674–13681Google Scholar
- 12.Sutherland DR, Illingworth A, Marinov I, Ortiz F, Andreasen J, Payne D et al (2018) ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 2: reagent selection and assay optimization for high-sensitivity testing. Cytometry B Clin Cytom 94:23–48CrossRefGoogle Scholar
- 16.Illingworth A, Marinov I, Sutherland DR, Wagner-Ballon O, Del Vecchio L (2018) ICCS/ESCCA consensus guidelines to detect GPI deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 3: data analysis, reporting and case studies. Cytometry B Clin Cytom 94:49–66CrossRefGoogle Scholar
- 19.Narayan D, Kaul S, Ravishankar K, Suryaprabha T, Bandaru VC, Mridula KR et al (2012) Risk factors, clinical profile, and long-term outcome of 428 patients of cerebral sinus venous thrombosis: insights from Nizam's Institute Venous Stroke Registry, Hyderabad (India). Neurol India 60:154–159CrossRefGoogle Scholar
- 29.Saposnik G, Barinagarrementeria F, Brown RD Jr, Bushnell CD, Cucchiara B, Cushman M et al (2011) Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 42:1158–1192CrossRefGoogle Scholar