A Rare Case of Severe Cold Autoimmune Hemolytic Anemia in a Hemochromatosis Carrier
Justin H Franco1, Priya K Jindal1, Sarah Jaggernauth1
1Department of Internal Medicine, University of Toledo College of Medicine and Life Sciences, Toledo, Ohio, 43606, USA, utoledo.edu.
Cold autoimmune hemolytic anemia (cAIHA) is a rare form of anemia characterized by antibody-mediated red blood cell destruction at low temperatures (i.e., below 98.6°F or 37°C). Patients with severe cAIHA exhibit hemoglobin levels under 8 g/dL. Affected patients present with hepatosplenomegaly, hypotension, tachycardia, and an increased risk of death with a 5-year survival rate of 63.5%. Treatment with immunosuppressants and lifestyle modifications can improve cAIHA symptoms. However, the presence of a concurrent disease process can worsen clinical outcomes. In our report, we discuss the clinical management of acute decompensated cirrhosis secondary to heterozygous hemochromatosis complicated by cAIHA. Hemochromatosis is characterized by increased iron deposition that results in organ dysfunction. On admission, the patient presented with anemia, weight loss, and ascites. Although heterozygous hemochromatosis is typically asymptomatic, the presence of cAIHA appears to have contributed to the patient's liver dysfunction. Requiring different treatment regimens, the case underscores the difficulty of managing multiple rare hematological conditions.
Cold autoimmune hemolytic anemia (cAIHA) is a rare form of anemia characterized by antibody-mediated red blood cell destruction at low temperatures (i.e., below 98.6°F or 37°C). Patients with severe cAIHA exhibit hemoglobin levels under 8 g/dL. Affected patients present with hepatosplenomegaly, hypotension, tachycardia, and an increased risk of death with a 5-year survival rate of 63.5%. Treatment with immunosuppressants and lifestyle modifications can improve cAIHA symptoms. However, the presence of a concurrent disease process can worsen clinical outcomes. In our report, we discuss the clinical management of acute decompensated cirrhosis secondary to heterozygous hemochromatosis complicated by cAIHA. Hemochromatosis is characterized by increased iron deposition that results in organ dysfunction. On admission, the patient presented with anemia, weight loss, and ascites. Although heterozygous hemochromatosis is typically asymptomatic, the presence of cAIHA appears to have contributed to the patient's liver dysfunction. Requiring different treatment regimens, the case underscores the difficulty of managing multiple rare hematological conditions.
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