A new case has been reported in which splenectomy resulted in the successful improvement of refractory hemolytic anemia in a man with primary cold agglutinin disease (CAD). The case report, published in the Kobe Journal of Medical Sciences, noted that the patient required no further transfusions after the splenectomy.

“In general, temperature control and warming are the first treatment choices for CAD, and rituximab or corticosteroids are effective in severe or intractable cases, although reports of cases in which splenectomy improved hemolytic anemia in CAD are few,” the authors wrote. “Herein, we report a rare case of refractory hemolytic anemia with CAD successfully managed with splenectomy.”

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The patient was a man aged 60 years who complained of fatigue. Laboratory testing revealed decreased hemoglobin levels, reticulocytosis, elevated cold agglutinin levels, splenomegaly, positive C3d, and negative immunoglobulin (Ig) G, resulting in a diagnosis of CAD. Warming and transfusions had limited effect and his splenomegaly worsened, so a splenectomy was performed.

Eighteen months after the surgery, his hemolytic anemia had improved and no further transfusions were needed. However, his cold agglutinin levels remained high, and IgG turned positive, so he was diagnosed with mixed-type autoimmune hemolytic anemia (AIHA).

Typically, splenectomy is ineffective as a treatment option for CAD because the hemolysis mainly occurs in the liver. Splenectomy is, however, effective in cases of warm-type AIHA, in which the hemolysis occurs in the spleen. This case represents a rare case of CAD with extravascular hemolysis in the spleen, possibly due to IgG action instead of IgM.

The patient also had pancytopenia in addition to hypersplenism before surgery, and the authors speculate that pancytopenia could be predictive of splenectomy as an effective therapeutic option in certain cases.

Reference

Okamoto S, Urade T, Yakushijin K, el al. Successful management of refractory autoimmune hemolytic anemia with cold agglutinin disease with splenectomy: a case report with review of literature. Kobe J Med Sci. 2023;68(1):E30-E34.