Immune Thrombocytopenic Purpura and Paradoxical Thrombosis: A Systematic Review of Case Reports.

BACKGROUND AND AIMS:  Immune thrombocytopenic purpura (ITP) is an acquired bleeding disorder characterized by autoantibodies against platelets. The clinical presentation is variable; the main symptom is bleeding, and many patients are asymptomatic; others have nonspecific symptoms like fatigue. Uncommonly, ITP can present with paradoxical thrombosis. The risk of thrombosis in ITP may be higher than expected, which makes the management of ITP more challenging. This review aims to evaluate patients with ITP who develop thrombosis and identify potential risk factors related to thrombosis in this category of patients. MATERIALS AND METHODS:  English literature was searched using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for adults above 18 years with primary ITP who had infarctions or thrombotic events. Patients with secondary ITP were excluded. The search included articles published up to 20th October 2021. RESULTS:  A total of 73 articles were included. Seventy-seven patients with ITP had developed infarctions and various thrombotic events. Sixty-three patients had arterial events, and 14 patients developed venous thrombotic events. CONCLUSION:  Patients with ITP have low platelets, which predispose them to bleed; despite that, serious thrombotic complications can happen in these patients and are difficult to predict. Therefore, it is critical for physicians to understand that ITP is paradoxically a prothrombotic condition and to address preventive thromboembolic measures whenever possible.

Cerebral Venous Thrombosis in a Patient with Immune Thrombocytopenia, an Apparent Paradox.

We present a paradoxical case of immune thrombocytopenia (ITP) that presented with cerebral venous thrombosis. A 39-year-old female patient diagnosed with chronic ITP, who failed treatment on multiple-line agents, was started on eltrombopag (thrombopoietin receptor agonist), which she was not compliant to. The patient later developed extensive cerebral venous thrombosis, along with venous infarcts, and intracranial and subarachnoid hemorrhage. She was treated with intravenous immunoglobulins as well as steroid therapy and was simultaneously started on anticoagulation. The patient improved clinically and radiologically. This case is among few reported cases which signify that patients with ITP are inherently prone to thrombosis despite low platelet count and treating these patients can be a dilemma. Judicious use of anticoagulation and immunosuppressive therapy is recommended based on available evidence pending further recommendations and guidelines about treatment of thrombosis in ITP.