Glucocorticoid therapy successfully treats man’s CAD: Case study
Doctors took unusual approach when standard therapies didn't work
The glucocorticoid prednisolone successfully treated a man’s primary cold agglutinin disease (CAD), which is typically resistant to such therapies, a case report said.
“Occasional” primary CAD cases “have demonstrated a favorable response” to glucocorticoids, “highlighting the need for a flexible and individualized therapeutic approach,” the researchers wrote.
The case study, “A Case of Primary Cold Agglutinin-Mediated Hemolytic Anemia Successfully Treated with Steroids,” was published in Cureus.
CAD is caused by self-reactive antibodies, called cold agglutinins, that bind to red blood cells at low temperatures, activating a group of immune proteins called the complement cascade. This ultimately leads to the destruction of red blood cells (hemolysis) and CAD symptoms such as fatigue.
The disease can be classified as primary, when the cause is unknown, or secondary, when it occurs due to other conditions, like infections, cancer, or other autoimmune disorders.
Avoiding cold, immune suppressants are typical treatments
Primary CAD management involves avoiding cold, along with therapies that suppress all or part of the immune system. Enjaymo (sutimlimab-jome), the only CAD-approved therapy, works by suppressing the complement cascade. However, it is not cleared for use in all regions.
Rituximab, a medication approved for certain cancers and autoimmune diseases, is commonly used off label as a first-line therapy for CAD. It works by promoting the death of B-cells, the immune cells responsible for producing antibodies.
Glucocorticoids, a class of anti-inflammatory and immunosuppressive medications, are frequently used for several autoimmune diseases, including those related to CAD. However, in most cases, CAD does not respond to glucocorticoids.
A trio of researchers described the case of a 69-year-old man in Sri Lanka with primary CAD who was successfully treated with glucocorticoids.
The man visited a local hospital after three months of general weakness and fatigue. He was pale, showed mild jaundice (yellowing of the skin and the whites of the eyes), and had mild liver enlargement.
Blood tests revealed hemolysis-related anemia, as evidenced by low hemoglobin (the protein responsible for carrying oxygen in red blood cells) and high levels of two hemolysis markers, lactate dehydrogenase and bilirubin. He also had high numbers of immature red blood cells.
A direct Coombs test, which detects antibodies and complement proteins attached to red blood cells, was positive for both molecules. He also had high cold agglutinin levels. Further tests ruled out infections, liver disease, blood cancer, and other malignancies.
Based on all these findings, the man was diagnosed with primary CAD. He began treatment with rituximab, along with folic acid and vitamin B12 to support red blood cell production, and received a blood transfusion. However, his hemoglobin levels showed no improvement one month after completing rituximab treatment.
The man was subsequently started on the corticosteroid prednisolone, which significantly raised his hemoglobin levels after two weeks.
The case shows that “clinicians should consider alternative or adjunctive treatments when standard therapies fail to achieve adequate disease control,” the researchers wrote.
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