Rare case of CAD secondary to lupus reported in young woman

Other AIHA types have also been associated with the autoimmune disease

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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A young woman in Colombia developed cold agglutinin disease (CAD) secondary to systemic lupus erythematosus (SLE), an autoimmune disease often called lupus, a study reports.

While other types of autoimmune hemolytic anemia (AIHA) — rare autoimmune diseases marked by self-reactive antibodies attacking and destroying red blood cells — have been reported in lupus patients, CAD has rarely been associated with it.

“As a result of the rare association between these two entities, our report is significant,” the researchers wrote in “Autoimmune Hemolytic Anemia Caused by Cold Agglutinin Antibodies in Systemic Lupus erythematosus—a Rare Association: Case Report,” which was published in the Journal of Blood Medicine.

AIHA can be classified as CAD, warm AIHA, and mixed AIHA, depending on whether the antibodies bind more easily to red blood cells at lower temperatures — known as cold agglutinins — or higher temperatures. The mixed form has both antibody types.

CAD is said to be primary when its cause is unknown or secondary when it occurs with an underlying condition, generally a blood cancer, an infection, or another autoimmune disease. About 1 in 10 lupus patients develop AIHA and most are associated with so-called warm antibodies, “but mixed AIHA has also been reported,” the researchers wrote. “AIHA due to cold agglutinins associated with SLE is extremely rare.”

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CAD secondary to lupus

Researchers in Colombia described the case of CAD secondary to lupus in a 22-year-old woman.

The woman went to the emergency room because she had been feeling unwell for the previous 10 days. Her symptoms included fever, headache, muscle pain, a mild rash that went away on its own, diarrhea, and occasional vomiting.

A physical examination revealed swollen glands in the neck, swollen knuckles, and lower limb swelling — consistent with lupus — and a pale appearance and yellowish eyes, which are symptoms of AIHA. A blood cancer or infection was ruled out and she was diagnosed with lupus. Further testing showed lupus nephritis, a common kidney complication of lupus.

Blood testing also revealed signs of AIHA — a low percentage of red blood cells and low levels of hemoglobin, the protein in red blood cells that carries oxygen around the body. The presence of cold agglutinins confirmed a diagnosis of CAD.

Given that her white blood cell and blood-clotting platelet levels were low, the woman was moved to the intensive care unit where she was started on the corticosteroid dexamethasone and the antimalarial hydroxychloroquine to treat her lupus.

In secondary CAD, “the treatment focuses on the management of the underlying disease,” the researchers wrote. In this case, that resulted in clinical improvements and all three blood cell counts normalizing “without the need for blood transfusions.”

The woman had no other complications and was sent home with treatment that included the corticosteroid prednisone, hydroxychloroquine, and the immunosuppressive agent mycophenolate mofetil. She had no new relapses on follow-up.

“Our patient responded strongly to management with dexamethasone, hydroxychloroquine and mycophenolate,” the researchers wrote. “This case illustrates the importance of a strong clinical suspicion of AIHA due to cold agglutinins associated with SLE when faced with similar clinical symptoms in order to achieve a timely diagnosis and provide optimal therapy.”