We report an uncommon case of Waldenström's macroglobulinemia with prominent splenomegaly associated with acquired C1 inhibitor deficiency, lupus anticoagulant and red blood cell autosensitization. Each of these immune abnormalities has been occasionally reported separately in patients with diverse B-cell lymphoid malignancies, but never before have they appeared simultaneously in the same patient. In this case, the pathogeny of the C1 inhibitor deficiency is questionable and may possibly be directly related to the IgM monoclonal gammapathy through protein-protein interactions, as occurs for lupus anticoagulant.
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