Cold agglutinin disease

What is cold agglutinin disease?

Cold agglutinins are typically IgM antibodies directed against I/i antigens on erythrocytes leading to hemolytic anemia due to complement-mediated RBC destruction in the reticuloendothelial system (typically the liver). Furthermore, occlusion of superficial vessels can occur (most commonly at exposed, cooler, acral sites) due to slowing of blood flow by agglutinated RBCs. This can lead to symptoms of acrocyanosis; Raynaud’s-like symptoms; and skin ulcers on the ears, nose, and digits. Most cases with chronic manifestations are idiopathic or related to lymphoproliferative diseases. Treatment includes warmth, glucocorticoids, RTX, and rarely plasmapheresis. Alkylating agents are limited to those patients requiring them for treatment of their lymphoma. Cold agglutinin disease due to infections (mycoplasma pneumoniae, infectious mononucleosis, other viruses) typically are asymptomatic or cause transient clinical manifestations that usually do not require therapy other than warmth. Cold agglutinin disease has also been described in patients with ANCA vasculitis related to levamisole-tainted cocaine.

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