Lymphoplasmacytic lymphoma associated with polyradiculoneuritis and cryoglobulinemia mimicking polyarteritis nodosa

Systemic vasculitis is sometimes associated with malignant blood disease.

We describe the case of a 77-year-old woman who had extensive livedo racemosa, acute polyradiculoneuritis, and meningeal hemorrhage. The skin biopsy showed evidence of necrotizing angiitis. This vascular involvement resembled polyarteritis nodosa (PAN). Despite corticosteroid and cyclophosphamide treatment, the polyradiculoneuritis worsened and the patient died of acute respiratory failure. Type II cryoglobulinemia was detected late, and the autopsy revealed lymphoplasmacytic lymphoma involving the spleen and infiltrating nerve roots, together with necrotizing angiitis involving small-sized arteries.

This case shows that necrotizing angiitis involving small arteries may occur with type II cryoglobulinemia.