CLL leukemia occurs in older adults; tends to progress slowly

Dear Dr. Roach: I am a 75-yearold 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 that at this time I show no symptoms of this disease; if symptoms do show up in time, there are very good treatments to combat the problems, but there is no cure.

Should I insist that my doctor begin treatments now so that I will not suffer more later on?

B.C.M.

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 years. 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 the cancer cells (that’s the FISH test) helps to predict how fast the cancer will progress. Fortunately, the del (13q) mutation is a very lowrisk mutation, and people with this mutation in their CLL have very little or no increase in risk of death overall. Further, well-done 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. Your doctor is doing his or her best to give advice that keeps in mind both your quality and your length of life. Don’t override your doctor’s advice lightly. Dear Dr. Roach: I have recently 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. IgG4 is a type of immunoglobulin, an antibody. In IgG4-related disease, the antibodies are directed against various tissues of the body, 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. The diagnosis often is missed, as this is a newly recognized condition. It is probably much more common than previously thought. It may resemble lupus or sarcoidosis. The diagnosis is made after a biopsy with special staining of the specimen. This means the 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 that your eye muscle and salivary-gland biopsies were related to this presentation of the disease. Enlarged lymph nodes and weight loss are common in this disease.

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. This disease is not yet well characterized. Many autoimmune diseases run in families, so it is possible that your daughter may have it as well.

Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to To Your Good Health @med.cornell.edu