Diffuse large B-cell lymphoma with chronic inflammation

Diffuse large B-cell lymphoma with chronic inflammation (DLBCL-CI) is a very rare type of blood cancer that develops in the setting of long-standing (chronic) inflammation. It is also sometimes called pyothorax-associated lymphoma (PAL) when it occurs in the chest cavity, which is its most well-known form. This cancer arises when B-cells (a type of white blood cell) grow out of control in areas of the body where there has been ongoing inflammation for many years, often 10 years or more. The most classic scenario involves patients who had an artificial pneumothorax (a collapsed lung procedure) as treatment for tuberculosis decades earlier, leading to chronic inflammation in the space around the lung (pleural cavity). However, it can also develop in other body sites with long-term inflammation, such as around metallic implants, in chronic bone infections, or in other body cavities with persistent fluid collections. The disease is strongly associated with Epstein-Barr virus (EBV) infection of the tumor cells. The chronic inflammation creates a local environment where the immune system cannot properly control EBV-infected B-cells, allowing them to eventually become cancerous. Symptoms depend on where the tumor develops but often include chest pain, back pain, fever, weight loss, night sweats, and sometimes a mass or swelling in the affected area. Because it typically arises in enclosed body spaces, it may grow quite large before being detected. Treatment generally follows approaches used for other types of diffuse large B-cell lymphoma, including combination chemotherapy regimens such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Surgery may also play a role, especially when the tumor is localized. The prognosis can be variable, and outcomes depend on the stage at diagnosis, the patient's overall health, and how well the cancer responds to treatment.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.What stage is my lymphoma, and has it spread beyond the original site?,What treatment plan do you recommend, and what are the expected side effects?,Is surgery an option in my case, or will I need chemotherapy alone?,How will you monitor whether the treatment is working?,Are there any clinical trials that might be appropriate for my specific type of lymphoma?,What is the expected timeline for treatment, and when might I start feeling better?,What should I watch for that would require me to seek emergency care during treatment?

Common questions about Diffuse large B-cell lymphoma with chronic inflammation

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