Study finds that immunotherapy substantially increases survival of people with lymphomatoid granulomatosis (10X lifespan increase (2 to now 20 years))

Medical Xpress / National Cancer Institute / The Lancet Haematology ^ | April 4, 2023 | Christopher Melani et al

[ConservativeMind]

Results from a clinical trial show that people with low-grade lymphomatoid granulomatosis who are treated with interferon alfa-2b, a type of immunotherapy, can live for decades after diagnosis. Lymphomatoid granulomatosis is a rare precancerous condition triggered by Epstein-Barr virus infection. Left untreated, the disease can progress to a high-grade form, which has a poorer prognosis and can quickly turn into an aggressive and fatal B-cell lymphoma.

In the phase 2 trial, patients treated with interferon alfa-2b lived for a median of about 20 years. By contrast, past studies reported a median survival of less than two years for people with lymphomatoid granulomatosis.

The findings suggest that immunotherapy can prevent the progression of low-grade disease to high-grade disease.

Lymphomatoid granulomatosis causes an overproduction of white blood cells known as B lymphocytes. Patients typically have lesions in the lungs, central nervous system, skin, liver, and kidneys. Symptoms can include cough, shortness of breath, fever, weight loss, and fatigue. Chemotherapy is currently the standard treatment for people with high-grade disease, but there is no standard treatment for low-grade disease.

Researchers have been studying lymphomatoid granulomatosis since the 1980s. In the 1990s, Wyndham Wilson, M.D., Ph.D. hypothesized that low-grade disease results from a defective immune response to the Epstein-Barr virus and could therefore be treated with immunotherapy, whereas high-grade disease requires chemotherapy to curb uncontrolled cell growth.

He and his colleagues treated four people with low-grade lymphomatoid granulomatosis with interferon alfa-2b over a 5-year period, and the treatment eradicated all signs of the disease in three of those patients, known as a complete remission. That study laid the foundation for the phase 2 trial of interferon alfa-2b in lymphomatoid granulomatosis, which has taken 30 years to complete because of the rarity of the disease and the challenges of recruiting enough patients for the study.

[ConservativeMind]

An available treatment grants decades more life to those diagnosed with an Epstein-Barr-related illness.

[ConservativeMind]

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