Treating common infections early may be life-saving in PNH: Report

Varicella-zoster virus proved fatal for young man with PNH in China

Marisa Wexler MS avatar

by Marisa Wexler MS |

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Varicella-zoster virus — which causes chickenpox and shingles — can lead to fatal infections in people with paroxysmal nocturnal hemoglobinuria (PNH) who are on immune-suppressing treatment, a case report highlights.

Researchers urge clinicians to be aware of signs of possible infection in a report, “Disseminated varicella-zoster virus infection in an aplastic anemia- paroxysmal nocturnal hemoglobinuria syndrome patient: A case report,” published in the journal Frontiers in Cellular and Infection Microbiology

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Immunosuppressants raise risk of infections

People with PNH are often treated with therapies that suppress the activity of the immune system, which can increase the risk of infections, since the immune system is normally responsible for defending the body against infectious threats. In these situations, appropriate monitoring to identify and treat infections early is critical.

“Patients treated with steroids and immunosuppressants are prone to various infections,” the researchers wrote. “Therefore, it is necessary to dynamically evaluate the benefits and risks of using steroid drugs or immunosuppressive drugs based on the patient’s situation in a timely manner.”

In this report, scientists in China described the case of a young man, 26, who died as a result of a severe infection with the varicella-zoster virus.

In July 2022, the patient had gone to the hospital after noticing that he was bleeding at the gums while brushing his teeth. Blood tests and a bone marrow biopsy were performed, ultimately confirming a diagnosis of PNH and aplastic anemia, another bone marrow disorder that commonly co-occurs with PNH.

The patient was started on immune-suppressing therapies, namely cyclosporine and corticosteroids, as well as traditional Chinese medicine. His condition was stable for a few months, but then in November, he was again admitted to the hospital due to a persistent, stabbing pain around his waist. He was also found to have limb swelling and a rash on his genitals.

Shortly after being admitted to the hospital, he developed an intense fever that was resistant to treatment, accompanied by rapid breathing and heart rate, and his abdominal pain persisted despite treatment. Clinicians at this point suspected he had some kind of infection, so further diagnostic tests were ordered and an initial line of anti-infection treatment was given.

The next day, the patient fell unconscious and had difficulty breathing. At that point, the rash was evident across his entire body. He went into cardiac arrest; clinicians managed to revive him and then keep him in a coma on life support for a few hours, but ultimately the patient died.

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Varicella-zoster infection deemed likely cause of patient’s death

The day after the patient died, diagnostic tests came back positive for varicella-zoster infection, which clinicians concluded was the most likely cause of death.

Upon reviewing the patient’s clinical history after his death, clinicians noted that, at the time of his original hospitalization, a few reddish spots had been identified on his skin. At the time, these were dismissed as being small bleed-related blemishes arising from the examination, but, in retrospect, researchers said these might have been a sign of varicella-zoster infection.

Noting that these infections can be extremely serious, the researchers urged clinicians caring for people with PNH to be on the lookout for signs of possible infection so that proper treatment can be given as early as possible.

“If [the infection] is not identified in time, it may delay the treatment opportunity, make the condition worse, and cause serious adverse prognosis. Therefore, clinicians need to pay attention to it,” the researchers wrote.