Castleman disease: A doctor fighting for his own cure

Castleman disease
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Dr David Fajgenbaum, of Perelman School of Medicine at University of Pennsylvania, is working to find a cure for Castleman disease while battling the illness himself.

Castleman disease is a very rare immune disorder that kills around 35% of individuals withing 5 years of diagnosis. There is very little information known about what causes the disease and there is currently no known cure.

What is Castleman disease?

The rare immune disorder, Castleman disease, is an immune disorder that results in the overgrowth of cells in the lymph nodes.

In the US, around 5,000 people are diagnosed with a type of Castleman disease every year, adding to the 30,000 people living in the US affected by this condition.

Castleman disease comes in two types, unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). UCD involves a single enlarged lymph node or region of lymph nodes whereas MCD causes multiple regions of enlarged lymph nodes.

Within MCD, there are two subtypes. One of which is caused by human herpesvirus-8 (HHV-8). While the second, diopathic MCD is currently unknown.

How is Castleman disease treated?

Each type and subtype of Castleman disease is treated differently. Those affected by UCD are likely to undergo surgery to remove the affected lymph node, in addition to taking medication to minimise the lymph node or radiation therapy to kill the diseased tissue. The treatment for MCD often involves immunotherapies, corticosteroids and antiviral treatments.

Castleman disease is often treated using chemotherapy. Chemotherapy is used to slow down the overgrowing cells in the impacted lymph node.

Treating Castleman disease as both a patient and a doctor

Assistant professor of medicine at the Perelman School of Medicine at University of Pennsylvania, Dr David Fajgenbaum, was diagnosed with diopathic MCD in his third year of medical school, after noticing sudden changes in his health in 2010.

Within several week of being affected by these symptoms, Fajgenbaum’s organs began to fail, in the November of that year he was read his last rights.

Castleman disease is often misdiagnosed as cancer, this is because the lymph nodes generally tied to the condition are found in the neck, underarm, groin and collarbone.

Fajgenbaum authored a memoir discussing his life with Castleman disease. “1 in 10 Americans has a rare disease and 95 percent of those diseases don’t have a single FDA-approved drug for them,” Fajgenbaum said. “For these patients, there can seem to be very little hope.”

“We think there is a genetic component, but we are doing research to see what that component may be,” he said. “We know most Castleman patients do not have a relative with Castleman. So, it’s not a strong component, but we think there is a component.”

“We’re doing everything we can to make that hope (for a cure) become a reality,” he added. “We really do have to do more work to understand more. ‘Why does it happen? How does it happen?’ And finally, ‘How do we stop it?’”

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