Cerebral venous sinus thrombosis with cold agglutinin disease in summer: illustrative case

Autoimmune hemolytic anemia (AIHA) is an autoimmune condition characterized by the production of autoantibodies against the surface antigens of red blood cells, leading to their peripheral destruction. Cold agglutinin disease (CAD) is a rare form of AIHA. The risk of thrombotic incidents is not well understood.

A 68-year-old man presented with left-sided unilateral chronic headache. CT revealed high-density lesions around the falx and cerebellar tent. CT angiography revealed contrast defects in the right transverse sinus and sigmoid sinus. A blood test revealed macrocytic anemia. There was no obvious source of bleeding. Although the cause of the anemia was still unclear, edoxaban was started for cerebral venous sinus thrombosis (CVST) during blood transfusion. After that, the patient was diagnosed with primary CAD, receiving therapy with sutimilimab, and has no neurological defects.

AIHA sometimes induces life-threatening thrombosis such as CVST. The dose of anticoagulant therapy should not be reduced for severe hemolytic anemia. The authors propose some tips for angiography to decrease the risk of thrombosis. Recently, new anticomplement therapies have been proven effective for CAD and the related hemolysis, suggesting the potential for decreasing thrombotic events. https://thejns.org/doi/10.3171/CASE24884.