Paul Allen and Non-Hodgkin Lymphoma: A Cancer of the Immune System

Two lymphomas, thirty-five years apart

BBC News reported that Paul Allen left Microsoft in 1983 after his first diagnosis, which was Hodgkin lymphoma. He recovered and spent decades as an investor, sports owner, and philanthropist through his firm Vulcan.

NPR reported that in early October 2018 he announced on Twitter that the non-Hodgkin lymphoma he had been treated for in 2009 had returned, and that he intended to fight it. He died in Seattle on Monday, October 15, 2018, at 65, from complications of non-Hodgkin lymphoma. Vulcan announced his death.

Two distinct lymphomas across one life is not an oddity of celebrity. It is a pattern documented in the medical literature, and it is where this explanation starts.

Lymphoma is not one disease

Lymphocytes are white blood cells that fight infection, in two main varieties called B cells and T cells. They circulate through lymph nodes, the spleen, and the bone marrow. Lymphoma is cancer of those cells.

The National Cancer Institute splits lymphoma into two families. Hodgkin lymphoma is defined by a specific abnormal cell seen under the microscope, the Reed-Sternberg cell. Non-Hodgkin lymphoma is everything else, and "everything else" means dozens of separate diseases.

The current count for the United States is the American Cancer Society's 2026 projection: 79,320 new cases of non-Hodgkin lymphoma and 19,970 deaths. SEER, NCI's cancer surveillance program, posts that pair beside its own measured rate of 18.7 new cases per 100,000 people per year. The median age at diagnosis is 68.

The division that decides what treatment means

NCI sorts non-Hodgkin lymphoma into two prognostic groups, and the logic runs backwards from what most people expect.

Indolent lymphoma grows slowly. NCI gives a median survival of around 20 years, while noting it is typically not curable once advanced. Follicular lymphoma is the most common indolent type, making up about 20 percent of all non-Hodgkin lymphoma.

Aggressive lymphoma grows fast, carries a worse short-term outlook, and yet NCI states that "more than 70% of patients with aggressive NHL can be cured."

So the slow disease is usually controlled rather than eliminated, and the fast one is often cured outright. Which group applies is the first thing to establish, because it changes what the word treatment is aiming at.

That is also why NCI's patient guidance lists "watchful waiting" among the options. For some indolent lymphomas, starting drugs early has not been shown to help.

Surviving Hodgkin lymphoma has a long tail

NCI is direct about what follows successful Hodgkin treatment. "Patients who complete therapy for HL are at risk of developing long-term side effects, ranging from direct damage to organ function or the immune system to second malignancies."

The timing is specific. NCI states that for the first 15 years after treatment, Hodgkin lymphoma itself is the main cause of death. By 15 to 20 years out, deaths from a second cancer, cardiovascular disease, or lung scarring exceed deaths from the original lymphoma.

NCI cites a study of 4,919 patients treated between 1965 and 2000 with a median follow-up of over 20 years, finding an excess of 123 deaths per 10,000 person-years compared with the general population. Lung and breast cancer are among the most common second solid tumors, and about 75 percent of them arise within the old radiation fields.

None of that describes what happened to any particular person, and this page makes no claim about Allen's case. It is a reason that anyone treated for Hodgkin lymphoma should stay in lifelong follow-up, and should keep a written record of exactly which drugs and which radiation fields were used.

When to get checked

• A swollen lymph node that has not shrunk after four weeks
• Any swollen node above the collarbone, at any age
• Swollen nodes in more than one region at once
• Fever above 100.4°F for weeks with no infection found
• Night sweats that soak nightclothes or bedding
• Losing over a tenth of your body weight in six months
• Widespread itching with no rash, or unexplained bone pain

NCI groups fever, drenching night sweats, and weight loss together as B symptoms. It defines them exactly: "unexplained weight loss (more than 10% of body weight in the 6 months before diagnosis). Unexplained fever with temperatures above 38°C. Drenching and recurrent night sweats." That temperature is 100.4°F.

Getting the diagnosis right

The subtype determines the treatment, so the sample has to be good enough to name it. NCI warns that "outside biopsy specimens should be carefully reviewed by a hematopathologist," a pathologist specializing in blood and lymph tissue.

NCI also compares methods, citing French data where up to 40 percent of diagnoses came from core needle biopsy and 60 percent from excisional biopsy, with excisional giving greater diagnostic certainty. Excisional means the whole node is removed.

The broader workup includes a complete blood count, blood chemistry, lactate dehydrogenase, hepatitis B and C and HIV testing, CT and PET scans, bone marrow sampling, and genetic analysis of the node. Staging uses the Lugano classification, which replaced the older Ann Arbor system.

Treatment, and what happens at relapse

NCI lists radiation therapy, chemotherapy, immunotherapy, targeted therapy, watchful waiting, surgery, and stem cell transplant. Rituximab, an antibody aimed at a marker on B cells, is a standard part of many regimens.

For disease that returns, NCI states that "consolidation therapy for relapsed disease after reinduction therapy using autologous stem cell transplant (SCT) or allogeneic SCT can be considered." NCI explains that transplants "restore blood stem cells in people who have had theirs destroyed by the high doses of chemotherapy or radiation therapy." Autologous means the patient's own cells. Allogeneic means a donor's, which adds an anti-cancer effect and also the risk of graft-versus-host disease.

What the survival figures show

SEER puts five-year relative survival for non-Hodgkin lymphoma at 74.3 percent, from cases diagnosed between 2016 through 2022. By stage it lists 87.6 percent for stage I, 79.7 percent for stage II, 74.0 percent for stage III, and 63.6 percent for stage IV.

That shallow decline is unusual. In most cancers, distant spread collapses the number. Lymphoma responds to drugs that circulate everywhere, so stage IV disease still carries a five-year figure above 60 percent.

Incidence has been falling about 0.6 percent a year, and death rates about 2.4 percent a year. All of these are group figures, and none describes an individual patient.

Sources

• https://www.bbc.co.uk/news/world-us-canada-45871379
• https://www.npr.org/2018/10/15/657640313/microsoft-co-founder-paul-allen-dies-at-65
• https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
• https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq
• https://www.cancer.gov/types/lymphoma/hp/adult-hodgkin-treatment-pdq
• https://www.cancer.gov/about-cancer/treatment/types/stem-cell-transplant
• https://seer.cancer.gov/statfacts/html/nhl.html
• https://www.cancer.org/research/cancer-facts-statistics.html