[Refractory Evans syndrome complicated with transverse sinus thrombosis]

Area of Science:

• Neurology
• Hematology
• Immunology

Background:

• Evans syndrome is a rare autoimmune disorder characterized by co-existing autoimmune hemolytic anemia and immune thrombocytopenia.
• Refractory cases often require aggressive treatment, including splenectomy and immunosuppressive therapies.
• Cerebral sinus thrombosis is a serious but uncommon complication of various medical conditions and treatments.

Observation:

• A 29-year-old woman with refractory Evans syndrome, post-splenectomy, experienced a sudden decline in consciousness after high-dose dexamethasone therapy.
• Neurological examination revealed coma, and brain imaging demonstrated severe edema with massive hemorrhage, indicative of cerebral transverse sinus thrombosis.
• Despite rising platelet counts, the patient suffered a catastrophic neurological event.

Findings:

• The case presents a rare instance of cerebral transverse sinus thrombosis complicating Evans syndrome, potentially triggered by high-dose dexamethasone.
• This event underscores the critical need for vigilance regarding neurological complications in patients with autoimmune disorders undergoing intensive treatment.
• This is the fourth reported case linking idiopathic thrombocytopenic purpura (a component of Evans syndrome) with sinus thrombosis.

Implications:

• Clinicians should consider the risk of cerebral sinus thrombosis in patients with Evans syndrome, particularly those receiving high-dose corticosteroids.
• Early recognition and prompt management of neurological symptoms are crucial for improving outcomes in such complex cases.
• Further research is warranted to elucidate the precise mechanisms linking autoimmune disorders, their treatments, and thrombotic events.