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A disease commonly thought of as bone disease this condition is actually a blood disorder, and as such, it falls under the purview of the specialty of Hematology. It is definitely a malignancy, and if allowed to go untreated, it is universally fatal within three to five years from the time of diagnosis.
The basic problem in this disease is that the specific blood cells that normally produce antibodies to fight bacteria transform into malignant cells and grow uncontrollably. Since these blood cells, named plasmacytes, reside naturally in the spaces of the bone marrow, they grow and displace the regular blood producing cells. Plasma cells also produce a substance that actually weakens and destroys bones and the unfortunate patient can thus develop fragile bones that easily fracture. In addition, these same plasma cells produce an overabundance of antibodies (remember, producing antibodies is their original purpose in nature) which in turn thicken the blood, clog the kidneys and reduce the ability of the body's immune system. Since normal bone marrow is replaced with these malignant plasma cells, the patient has anemia, bleeding tendencies and susceptibility to infection. Untreated, patients often die of infection or bleeding. However, prior to their demise they suffer broken bones, kidney failure (requiring dialysis) and frequent pneumonia.
What causes the normal plasma cell to become malignant is unknown, but generally it is felt that a certain virus integrates itself in the plasma cell or in the surrounding marrow, which incorporates itself into the DNA and causes transformation to malignancy. There is no known prevention of this disease.
As sad and tragic as this disease can be, it has given modern medicine a forum to advance innovative and exciting treatments. Some of these treatments have "spilled over" to other disease treatments, demonstrating that advance in the field of one disease can translate into treating other conditions.
For many years Myeloma was treated with an oral chemotherapy pill plus high doses of steroids. Through the years, doctors have attempted using much stronger combinations of chemotherapy. But this more aggressive approach does not seem to add significant benefits.
In younger patients, those who can tolerate a very aggressive approach, attempts are made at even using extremely high doses of chemicals and radiation followed by transplanting back the patient's own bone marrow cells. But even this super aggressive approach does not provide curability and the approach remains controversial.
Amazingly, the best treatment today for this very serious disease is a relatively well tolerated regimen of Zometa Thalidomide, Steroids and a new medicine named Velcade. None of these four medicines are considered chemotherapy! Zometa is a bone strengthening medicine that is infused once a month, and since its introduction about
7 years ago patients have become almost totally free of spontaneous bone fractures.
Steroids are natural hormones that are given in very high doses and in intermittent intervals such that patients can tolerate these regimen well. Thalidomide has actually been developed back in the 1950's to help with nausea of pregnancy. Because it caused horrible birth defects, it was taken off the market, but was reintroduced in the 1970's to treat resistant Leprosy (of all things)! In the 1990's scientist began using it in myeloma - with surprisingly good results. The combination of Thalidomide and steroids causes up to 80 percent remissions. Because of bothersome nerve damage, the use of thalidomide is limited, but this year our pharmaceutical colleagues brought to the market a variant form of Thalidomide that is better tolerated and is more potent (Revlimid).
Finally, about three years ago we saw the introduction of a new class of medicine, Velcade, that is a biological agent which specifically targets the reproduction of the malignant plasma cells and that has amazing results of putting most patients into a substantial remission.
Today, research is focused on combining the various medicines that we have in our arsenal so as to achieve more effective ands durable remissions.
I am often asked how can I deal with such a sad specialty. I decided to write this article about myeloma as an example of how exciting and intellectually challenging my specialty actually is. Here is a disease that was not only deadly, but caused horrible suffering prior to a patient's demise. Today, the medical community has been blessed with the knowledge of new tools and options to help our patients suffer much less, live much healthier and significantly longer! The challenge for the doctor is to learn and integrate these new treatment options, and the excitement is to watch the patient actually respond well. The real exhilaration, however, is the knowledge that we are getting closer to an actual cure for this horrible disease, a cure than I can reasonably expect within the span of my own career!
ARON B. BICK, MD
SYNERGY HEMATOLOGY ONCOLOGY
MEDICAL ASSOCIATES, INC
5901 WEST OLYMPIC BLVD #420
LOS ANGELES, CA. 90036
323 525 1111