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Cold Agglutinin Disease: a Reminder for the Hospi ...
Cold Agglutinin Disease: a Reminder for the Hospitalist
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This document is a case report and brief review of cold agglutinin disease (CAD), a rare form of autoimmune hemolytic anemia that can range from mild to life-threatening. CAD accounts for about 20–25% of autoimmune hemolytic anemia cases and may be primary, when no underlying cause is found, or secondary, when associated with infection, autoimmune disease, or malignancy.<br /><br />The report describes a 65-year-old man with a history of prostate cancer who presented with fatigue and dyspnea. Laboratory studies showed anemia with hemolysis: low hemoglobin, elevated LDH, low haptoglobin, increased reticulocytes, and elevated indirect bilirubin. Peripheral smear showed red cell agglutination and spherocytes without schistocytes. Direct antiglobulin testing was positive for C3 and negative for IgG, confirming CAD. Extensive workup, including infectious, malignancy, and bone marrow evaluation, did not identify an underlying cause.<br /><br />His hospital course was severe and prolonged, requiring nearly daily warmed blood transfusions over four weeks. He developed complications including iron overload and deep vein thrombosis. His disease was refractory to steroids, IVIG, rituximab alone, and eculizumab, but ultimately responded to combination rituximab-bendamustine therapy. The cold agglutinin titer was negative at discharge, and he has not had recurrence.<br /><br />The article emphasizes important differences between cold and warm autoimmune hemolytic anemia, including prevalence, DAT patterns, and typical etiology. It also highlights management principles for CAD: supportive care with cold avoidance and warmed transfusions, plus disease-modifying anti-B-cell therapies. The case underscores that CAD can cause significant morbidity through severe anemia, transfusion-related iron overload, treatment toxicities, and increased thromboembolic risk.
Asset Subtitle
Shabbir Matcheswalla
Meta Tag
Author List
Shabbir Matcheswalla, Taylor Koenig
Category
Clinical Vignettes
Concept
Cold Agglutinin Disease
Concept
Direct Antiglobulin Test
Concept
Anemia
Concept
Hemolysis
Concept
Warm Autoimmune Hemolytic Anemia
Distinguished
Non-Finalist
Presenter Organization
Wexner Medical Center, Ohio State University
Presenting Author
Shabbir Matcheswalla
Track
Adult
Keywords
cold agglutinin disease
autoimmune hemolytic anemia
hemolysis
direct antiglobulin test
red cell agglutination
warmed blood transfusion
rituximab-bendamustine
iron overload
deep vein thrombosis
B-cell therapy
Cold Agglutinin Disease
Direct Antiglobulin Test
Anemia
Hemolysis
Warm Autoimmune Hemolytic Anemia
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