A novel method for demonstrating cold agglutinin disease: a case report
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CASE REPORT
Open Access
A novel method for demonstrating cold agglutinin disease: a case report Thomas A. Vo1, Zack Oakey1, Yasir A. Khan2 and Donald S. Minckler1*
Abstract Background: Cold agglutinin disease is a rare disorder characterized by an autoimmune hemolytic anemia occurring at low temperatures. Physical examination findings, often limited to acrocyanosis, are combined with a thermal amplitude test to help establish the diagnosis. Thermal amplitude testing determines the highest temperature at which the cold agglutination will occur and is an important parameter in diagnosing cold agglutinin disease. Case presentation: Here we describe a 57-year-old white man of German and Nicaraguan descent with known chronic cold agglutinin disease who presented to our ophthalmology clinic for evaluation of a cataract. During routine cataract surgery, the lowered temperature of the conjunctiva from intermittent flow of balanced salt solution at room temperature induced a cold agglutination reaction in conjunctival vessels easily visible under a surgical microscope. Conclusions: To the best of our knowledge, this method of demonstrating cold agglutinin disease has not been described in the literature and could easily be performed utilizing an ordinary slit lamp. This method could be used as an alternative and rapid screening method for cold agglutinin disease. Keywords: Cold agglutinin disease, Anemia, Autoimmune, Conjunctival vessels
Background Autoimmune hemolytic anemia cold agglutinin disease (CAD) is a rare disorder characterized by an immune reaction against red blood cell (RBC) self-antigens. “Cold agglutinin” describes the binding of the immunoglobulin (Ig) with erythrocytes at low temperatures, causing them to agglutinate and consequently induce hemolysis in those with CAD. CAD typically affects patient in their seventh decade of life, with clinical manifestations including livedo reticularis, Raynaud disease, and acrocyanosis [1–4]. Due to both its rarity and limited findings on physical examination, diagnosing CAD can be difficult. Because many patients with cold agglutinins may never develop any symptoms, titer levels help determine which patients have clinically significant levels of cold agglutinin and which do not. Titer levels are not fully concordant though and may vary indirectly with disease severity, making it less reliable in some patients. Thermal activity, however, demonstrates the disorder and may be the most
valuable test for preventing overdiagnosis of patients with CAD [1, 2]. Demonstrating thermal activity is important to determine the thermal amplitude, which is the highest temperature at which the cold agglutination reaction is observed [4]. Such testing not only confirms cold agglutinin activity, but also determines whether such activity is clinically significant [2]. Here we describe a 57-year-old white man with chronic CAD who presented to our ophthalmology clinic for evaluation of a cataract. During cataract surgery, it was observed that the lowered temperature environment produced
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