Andrea Lobo, —

The rare case of a young woman with cold agglutinin disease (CAD) secondary to chickenpox, also known as varicella, was described in a recent report in India. While corticosteroids, a type of steroid, are usually not recommended for CAD secondary to an infection, the rapid and sustained red blood cell destruction, or hemolysis, seen in the woman prompted such treatment, to which she responded well. This rare case suggests that a “short course of steroids may be beneficial” in CAD-secondary to chickenpox if severe hemolysis does not lessen in the following days, the researchers wrote. The case report, “Cold antibody autoimmune hemolytic anemia following varicella infection,” was published in the Indian Journal of Pathology & Microbiology. Autoimmune hemolytic anemia (AIHA) ia a group of diseases in which the body produces antibodies that wrongly target and destroy red blood cells. AIHA can be mainly classified as CAD or warm AIHA, depending on whether the antibodies bind more easily to red blood cells at lower or higher temperatures. CAD accounts for 25% of all AIHA cases, and is driven by a type of self-reactive antibodies, called cold agglutinins, that bind to red blood cells at low temperatures. This binding promotes the formation of red blood cell clumps — a process called agglutination — and their subsequent destruction, resulting in anemia. Most cases of CAD are secondary to other diseases, such as cancer, autoimmune conditions, or infections. “Very few cases of AIHA following varicella infection have been reported,” the researchers wrote, and most cases in adults have been associated with warm-activated antibodies. CAD developed after case of chickenpox. Now, researchers in India have described the case of a 24-year-old woman who developed CAD following chickenpox, which is caused by the varicella-zoster virus. She was admitted to the hospital due to reddened and raised areas of skin on the face, trunk, and extremities that evolved to vesicles and crusting — typical of chickenpox — five days before admission. She also developed a fever and yellowish pigmentation of the eyes. Laboratory tests showed low levels of hemoglobin, the protein of red blood cells that carries oxygen throughout the body, and high levels of bilirubin and lactate dehydrogenase (LDH), two markers of hemolysis. The woman also had higher-than-normal counts of immune cells, particularly neutrophils, indicative of an underlying condition such as an infection. She was given antibiotics and other standard medications to fight the infection. Further blood examinations showed the presence of red blood cell agglutinates and neutrophilic erythrophagocytosis, a process in which red blood cells are engulfed, or eaten, by neutrophils. Tests for malaria, dengue, and hepatitis viruses — which can cause hemolysis — were negative. A direct Coombs test was positive for cold agglutinins, which together with the absence of other self-reactive antibodies, indicated a diagnosis of CAD secondary to varicella infection. On the second day after admission, her hemoglobin levels dropped further and she was transfused with three warmed packs of red blood cells. After an initial rise in hemoglobin levels, there was a decrease, together with an increase in bilirubin, suggesting ongoing hemolysis. On day four, red blood cell agglutination and erythrophagocytosis were still observed, and LDH levels remained elevated. As such, she was started on immunosuppressive prednisolone, a corticosteroid. At day six, hemoglobin began to show a consistent rise, with a corresponding reduction in bilirubin levels. Throughout the hospital stay, the woman has stable and did not require further blood transfusions. Prednisolone treatment was maintained for one week, and rapidly reduced over 10 days, and she was discharged 12 days after admission. One week later, she had no symptoms, showed normal hemoglobin, bilirubin, and LDH levels, and tested negative for cold agglutinins. No signs of red blood cell agglutination or erythrophagocytosis were observed. “Although her hemoglobin improved and hemolysis was stalled, it is difficult to associate this response to steroids based on a single case report,” the researchers wrote. “Steroids have been tried in the past with success in warm antibody AIHA following chicken pox, while the cases of cold antibody AIHA resolved spontaneously over a period of 1 week.” This case suggests that “short course of steroids may have a role in cold antibody AIHA following chickenpox for control of severe ongoing hemolysis and restoration of rapidly falling hemoglobin levels particularly when illness shows no abatement in the first few days,” the team wrote. They emphasized, however, that further studies are needed to clarify the beneficial effects of steroids in these cases, as well as the risk factors for chickenpox-associated CAD.

Andrea Lobo, —

Articles by Andrea Lobo

Woman diagnosed with CAD after having chickenpox: Case report

Rare Disease Day 2023

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