Alpha-Gal Syndrome: First Death Reported | Symptoms & Risks

by Grace Chen

First Fatal Case of Alpha-Gal Syndrome Linked to Red Meat Consumption Reported

A New Jersey man’s death after eating a hamburger has been linked to alpha-gal syndrome, marking the first documented fatality from the condition triggered by mammalian meat, according to research published in The Journal of Allergy and Clinical Immunology: In Practice.

The case underscores a growing concern about alpha-gal syndrome (AGS), an allergy to a sugar molecule found in most mammals, and highlights the need for increased awareness among both medical professionals and the public. The syndrome is typically triggered by the bite of the lone star tick, and symptoms can range from hives and itching to severe anaphylaxis.

A Delayed and Unexpected Reaction

The 47-year-old New Jersey resident had no significant medical history beyond recent “chigger” bites, which researchers believe were larval lone star ticks. In the summer of 2024, while camping with family, he consumed a steak – an unusual choice, as he rarely ate red meat. Approximately four hours later, he experienced abdominal discomfort, including pain, diarrhea, and vomiting, which subsided after two hours.

Despite feeling unwell, the man continued his activities the following day, even taking a five-mile walk. He confided in his son that he felt as though he was “going to die” during the initial episode but did not seek medical attention. Two weeks later, he ate a hamburger and subsequently mowed the lawn before collapsing in his bathroom. Despite a two-hour resuscitation effort by his son and paramedics, he was pronounced dead at the hospital.

“If you had heard someone had severe abdominal pain 4 hours after eating meat, and then 4 hours again after something else, you’d say, done deal. You’ve got alpha-gal,” explained researcher Thomas A.E. Platts-Mills, MD, PhD, professor of medicine in asthma, allergy and immunology at the University of Virginia School of Medicine.

Diagnostic Challenges and a Rare Presentation

The postmortem examination revealed no significant abnormalities in the man’s cardiac, respiratory, neurological, or abdominal systems. Initial investigations ruled out obvious causes of death, leading the man’s wife to seek further evaluation from Erin M. McFeely, MD, of Hackensack Meridian Health.

Blood tests revealed a positive IgE to beef and a tryptase level exceeding 2,000 ng/mL – a level considered “astronomic” and indicative of fatal anaphylaxis. Researchers also found a low level of IgE to lamb, consistent with typical alpha-gal presentation. The man also tested positive for rye grass and ragweed allergens, which researchers attributed to his diphenhydramine level.

What made this case particularly noteworthy was the presentation of symptoms. The man’s anaphylaxis primarily manifested as gastrointestinal distress, a less commonly recognized sign of AGS. “It might seem strange for an allergy to present with GI symptoms only, but this, many experts say, is probably the most common phenotype of alpha-gal syndrome,” said Sharon Forsyth, executive director of the Alpha-gal Alliance Action Fund. “It’s just really underdiagnosed.”

Growing Prevalence and Public Health Implications

The increasing prevalence of the lone star tick, coupled with a lack of public awareness, poses a significant public health challenge. The tick’s expanding range means more individuals are at risk of sensitization. “At least half the people we see have gotten their tick bites from their own yard,” Platts-Mills noted.

Experts emphasize the importance of recognizing the delayed onset of symptoms – typically 3 to 5 hours after consuming mammalian meat – and the potential for gastrointestinal symptoms to be the primary indicator of a reaction. Douglas H. Jones, MD, FAAAI, FACAAI, cofounder and CEO of the Food Allergy Support Team, advised physicians to consider AGS in patients with a history of tick bites who experience delayed symptoms after eating beef, pork, or other mammalian meat.

The Alpha-gal Alliance Action Fund is advocating for the inclusion of alpha-gal on the list of major food allergens through the Alpha-gal Allergen Inclusion Act (HR 1178), which would require its disclosure on packaged food labels. They are also pushing for alpha-gal syndrome to be a mandatory reported condition at the state level to better track its incidence.

“We really need to have our numbers counted,” Forsyth stated. “It would be great if the CDC would make it a nationally notifiable disease.”

This case serves as a stark reminder of the potentially life-threatening consequences of alpha-gal syndrome and the urgent need for increased awareness, education, and proactive measures to protect public health. The researchers emphasize that recognizing the association between meat consumption and delayed abdominal symptoms is crucial for timely diagnosis and potentially life-saving intervention.

For more information:
Thomas A.E. Platts-Mills, MD, PhD, can be reached at [email protected]. Sharon Forsyth can be reached at [email protected]. Douglas H. Jones, MD, FAAAI, FACAAI, can be reached at [email protected].

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