With no symptoms, best hold off on CLL treatment
Dear Doctor: I am a 75-year-old white female who recently was diagnosed with chronic lymphocytic leukemia. My doctor, a hematologist, completed a FISH blood test, which showed it to be the type of leukemia associated with an abnormal chromosome called del(13q). I am in relatively good health, and my doctor says I show no symptoms of this disease; if symptoms do show up in time, there are very good treatments, but there is no cure.
Should I insist my doctor begin treatments now so that I will not suffer more later on? — B.C.M.
A CLL is one of the most common leukemias (a type of blood cancer). It occurs mostly in older adults, with the average age at onset being about 70. While it progresses very slowly in most people, it is usually not curable.
CLL differs greatly from person to person, in terms of its aggressiveness. Checking the DNA mutations of cancer cells (the FISH test) helps to predict how fast the cancer will progress. Fortunately, the del(13q) mutation is a very low risk mutation, and people with this mutation in their CLL have very little or no increase in risk of death overall. Further, trials comparing people who are treated early in the disease show no overall benefit in survival, compared with people who waited to get treatment until they developed symptoms.
So, I agree with your doctor. Because treatments do cause some side effects, if you started treatment now, you would be guaranteeing yourself a worse quality of life right away, without any guarantee of making things better later. Don’t override your doctor’s advice lightly.
Dear Doctor: I have been diagnosed with IgG4-related disease. Can it be genetic? My daughter has many unexplained symptoms that I worry may be related to this condition. I have required biopsies of an eye muscle, the salivary gland and lung lesions, among others. What can you tell me about this disease? — J.E.
A IgG4 is a type of immunoglobulin, an antibody. In IgG4related disease, the antibodies are directed against various tissues, and the disease manifestations depend on what organs are affected. It is thought to have components of allergy as well as autoimmunity.
The condition is seen more frequently in men than in women, and in middle age. This is a newly recognized condition, so the diagnosis is often missed. It is probably much more common than previously thought. It may resemble lupus or sarcoidosis. Diagnosis is made after a biopsy with special staining of the specimen, so diagnosis is more likely when it’s suspected to begin with.
It can cause an autoimmune pancreatitis, or may present as a tumour. I suspect your eye-muscle and salivary-gland biopsies were related to this presentation. Enlarged lymph nodes and weight loss are common.
Treatment is initially with glucocorticoid steroids, such as prednisone. If the diagnosis is correct, people start getting better within two to four weeks. Some experts use other medications to try to reduce the use of steroids, which have many side effects if used long term.
As far as heritability goes, I can’t find a definitive answer. The disease is not yet well characterized. Many autoimmune diseases run in families, so it is possible your daughter may have it.