A Case Report of Idiopathic Catastrophic Thrombotic Syndrome with Purpura Fulminans

Abstract

Ngan Nguyen, Manjari Pandey

Background: Catastrophic thrombotic syndrome (CTS) is a rare life-threatening condition defined as rapid onset of multi-organ thrombosis affecting diverse vascular beds. Predisposing conditions include catastrophic antiphospholipid syndrome (APS), atypical thrombotic thrombocytopenic purpura (TTP), delayed heparin-induced thrombocytopenia and Trousseau syndrome. Patients who do not meet any of these criteria are diagnosed with idiopathic catastrophic thrombotic syndrome.

Case description: A 44-year-old Caucasian woman with type II diabetes and hypothyroidism presented with acute onset of myalgia and extensive bruising over a period of four days. Physical exam revealed hypotension, tachycardia, and extensive purpuric and bullous skin lesions. Laboratory evaluation demonstrated microangiopathic hemolysis, thrombocytopenia, elevated D-dimer and coagulopathy suggesting disseminated intravascular coagulation (DIC) along with acute kidney injury (AKI) and transaminitis. Aggressive transfusions including packed red blood cells, fresh frozen plasma, platelets and cryoprecipitate were required to reverse her severe coagulopathy. Ultrasound showed occlusive thrombus in the left basilic vein and the greater saphenous veins bilaterally and heparin infusion was started. IV methylprednisolone, all-trans retinoic acid and doxycycline were empirically given. Workup was negative for any coagulation factor deficiency or hypercoagulable state although heterozygous factor V Leiden (FVL) mutation was found. Bone marrow biopsy was normal. Infectious and auto-immune workups were unremarkable. Skin biopsy showed diffuse intravascular thrombi but no evidence of vasculitis. Two weeks later, she developed Enterobacter bacteremia from infection of her bullous lesions. She was started on broad spectrum antibiotics and transferred to a burn unit. Eventually, her coagulopathy, bacteremia, AKI and transaminitis resolved, she was discharged with indefinite anticoagulation.

Discussion: CTS presented in our patient with rapidly progressive thrombosis with consumptive coagulopathy. No obvious instigating source was found, her clinical presentation was out of proportion for the isolated heterozygous FVL mutation. Anticoagulation remained the main therapy in the acute setting and aggressive supportive care in multi-disciplinary setting to manage acute and late complications was required.

Conclusion: Through this report, we emphasize the need for early recognition of CTS with this constellation of clinical findings and advocate for urgent interventions to prevent untoward outcomes.

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