Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder characterized by intravascular hemolysis, bone marrow failure, and increased thrombotic risk. Previous studies using plasma thrombin generation tests in PNH patients yielded conflicting results. Given the central role of cellular blood components in PNH, we hypothesized that whole blood thrombin generation (WB-TG) may provide a more comprehensive assessment of patients' coagulation profile. We report the case of a 25-year-old woman with PNH and admitted to Padova University Hospital for hemolytic crisis following an influenza A virus infection. WB-TG performed upon admission revealed a hypercoagulable profile vs. healthy controls. The patient was immediately initiated on corticosteroids with good response (i.e., hemoglobin values) and subsequent resolution of hemolysis within ten days. Antithrombotic prophylaxis was administered and no thrombotic events occurred. WB-TG may be a valuable tool in the clinical management of PNH, particularly for the early identification of hypercoagulability and tailoring anticoagulant regimens.
Whole blood thrombin generation hypercoagulable profile in a patient with hemolytic crisis due to paroxysmal nocturnal hemoglobinuria: a case report
Spiezia L.
;Bulato C.;Campello E.;Simioni P.
2025
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder characterized by intravascular hemolysis, bone marrow failure, and increased thrombotic risk. Previous studies using plasma thrombin generation tests in PNH patients yielded conflicting results. Given the central role of cellular blood components in PNH, we hypothesized that whole blood thrombin generation (WB-TG) may provide a more comprehensive assessment of patients' coagulation profile. We report the case of a 25-year-old woman with PNH and admitted to Padova University Hospital for hemolytic crisis following an influenza A virus infection. WB-TG performed upon admission revealed a hypercoagulable profile vs. healthy controls. The patient was immediately initiated on corticosteroids with good response (i.e., hemoglobin values) and subsequent resolution of hemolysis within ten days. Antithrombotic prophylaxis was administered and no thrombotic events occurred. WB-TG may be a valuable tool in the clinical management of PNH, particularly for the early identification of hypercoagulability and tailoring anticoagulant regimens.
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