AI Article Synopsis
- Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia where red blood cells clump together at lower temperatures, leading to anemia symptoms, and splenectomy is usually not effective for it.
- A 60-year-old man diagnosed with CAD experienced persistent anemia and was treated with conventional methods like blood transfusions, but his condition did not improve.
- After a splenectomy, aimed at investigating a possible malignancy and alleviating his anemia, the patient's condition improved significantly, suggesting splenectomy could be a potential treatment for refractory CAD.
Article Abstract
Background: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia characterized by agglutination of red blood cells at temperatures below the normal core body temperature. In patients with CAD, splenectomy is not indicated because of its low therapeutic effect on hemolytic anemia induced by extravascular hemolysis. Herein, we report a case of refractory hemolytic anemia with CAD successfully managed with splenectomy.
Clinical Case: A 60-year-old man visited a municipal hospital with the chief complaint of fatigue. He was found to have hemolytic anemia and icterus with increased cold agglutination and was diagnosed with CAD. Malignant lymphoma was suspected as the underlying disease; however, no clear underlying disease was identified. Hemolytic anemia progressed during the subsequent winter seasons, and he was treated with temperature control, warming, and weekly blood transfusions. However, despite the blood transfusions, his hemoglobin level did not improve during the summer 2 years after diagnosis, and his previously observed splenomegaly had progressed. He was referred to our department, and a splenectomy was performed to diagnose any occult malignant lymphoma and improve the refractory hemolytic anemia. Because histopathological examination revealed no evidence of malignant lymphoma, a diagnosis of primary CAD was made. The hemolytic anemia improved, and no blood transfusion was required after splenectomy.
Conclusions: Splenectomy significantly improved the patient's refractory hemolytic anemia due to primary CAD. Thus, it may be an effective treatment option in such cases, although further cases and studies are required to evaluate the effects of splenectomy.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10117625 | PMC |
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