Concomitant cryoglobulinemic vasculitis and cold agglutinin disease successfully treated with bortezomib: A case report.

RATIONALE: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation.

PATIENT CONCERNS: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Laboratory data demonstrated hemolysis. Cold agglutinin and cryoglobulin tests were positive. There was no evidence for malignancies after blood, image, and pathologic tests.

DIAGNOSES: Concomitant cryoglobulinemic vasculitis and CAD.

INTERVENTIONS: The patient was treated with bortezomib-based regimen, including bortezomib, cyclophosphamide, and dexamethasone.

OUTCOMES: The patient responded well to the treatment. Both symptoms and laboratory tests significantly improved. The patient's condition was in a state of sustained remission in the 6-month follow-up.

LESSONS: This rare case promotes further understanding of these 2 diseases and suggests that bortezomib is a promising treatment in type I cryoglobulinemic vasculitis.