| Summary: | Cold agglutinin disease (CAD) is a rare form of autoimmune hemolytic anemia, which shows IgM antibodies in the serum of the patient against red blood cell antigens, showing maximal reactivity at 4°C. CAD can be idiopathic or secondary to infections such as mycoplasma, Epstein–Barr virus, cytomegalovirus, lymphoma, and plasma cell dyscrasia. It can present either as hemolytic anemia or acrocyanosis. We are reporting a case of CAD presenting with acrocyanosis, which initially did not show any secondary etiology despite extensive work-up. However, after a gap of 1½ years, the patient presented with intestinal obstruction, which was diagnosed to be due to malignant stricture secondary to intestinal diffuse large B-cell lymphoma. This association makes it important to follow-up cases of CAD without secondary etiology for the development of lymphoma in future.
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