Open AccessMarkers of Thrombin Generation and Inflammation in Patients with Paroxysmal Nocturnal Hemoglobinuria
Author(s) -
Rishi Dhawan,
Jasmina Ahluwalia,
Pankaj Malhotra,
Manoranjan Mahapatra,
Neelam Varma,
Subhash Varma
Publication year - 2019
Publication title -
indian journal of hematology and blood transfusion/indian journal of hematology and blood transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.213
H-Index - 15
eISSN - 0974-0449
pISSN - 0971-4502
DOI - 10.1007/s12288-019-01239-x
Subject(s) - paroxysmal nocturnal hemoglobinuria , medicine , thrombosis , d dimer , hemoglobinuria , gastroenterology , platelet , venous thrombosis , p selectin , population , thrombin , platelet activation , immunology , anemia , environmental health
Paroxysmal nocturnal hemoglobinuria (PNH) presents with intravascular hemolysis, bone marrow failure and thrombosis. Various studies have reported geographic and ethnic variation in prevalence of thrombosis in PNH. There is limited data on thrombosis in PNH from the Indian subcontinent. In this study we describe disease burden and risk factors for thrombosis in 18 Indian PNH patients. We studied markers of thrombin generation (Thrombin-antithrombin complexes; TAT and D-Dimer), endothelium and platelet activation (soluble P-selectin) and inflammation (interleukin-6; IL-6) in PNH patients and compared their levels with healthy controls. Thrombosis was identified in 17% of PNH patients. TAT, sP-selectin and D-Dimer levels were significantly elevated in PNH patients (TAT: 5.06 ± 1.08 ng/ml; sP-selectin: 80.57 ± 19.5 ng/ml; D-Dimer mean: 936 ng/ml 95% CI 559, 1310) compared to control population (TAT: 3.39 ± 0.769 ng/ml P = 0.016; sP-selectin: 44.67 ± 5.17 ng/ml P = 0.002). Using Youden's J statistic, the cut-off values for TAT and sP-selectin in our cohort of PNH patients were 2.90 ng/ml and 58.41 ng/ml respectively. TAT, sP-selectin and D-Dimer levels were elevated beyond the cut-off values in PNH patients with thrombosis compared to those without thrombosis. A positive correlation was noted between TAT, sP-selectin and D-Dimer levels. Increased TAT, sP-selectin, and D-Dimer levels may indicate impending thrombosis in PNH.