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Plasma cell leukemia is a rare and typically very aggressive form of blood cancer. It’s often related closely to multiple myeloma, and sometimes presents as a secondary form of this disease. More often, though, it occurs all on its own, which is usually known as a “primary” diagnosis. It’s most common in adults between the ages of 50-60, and usually affects about the same number of men as women each year. The disease progresses by causing abnormalities in the plasma cells, which aggregate together and cause bone lesions. The aggregations usually also actually attack the bone, and can cause a range of systemic failures throughout the body. Once diagnosed, patients usually only have a few months to live, though a lot of this can depend on the type of disease and whether it is related to myeloma; myeloma cases are usually the most serious. Sometimes chemotherapy and other aggressive techniques can be used to fight the cancer, but at best this adds only about a year to a patient’s life expectancy. The most common treatment is usually to make the patient as comfortable as possible.
Understanding Leukemia Generally
The term “leukemia” is used to describe a range of cancers affecting the blood and blood tissues in humans and many animals. It can occur primarily in the blood itself, normally in white blood cells, or in bone marrow or lymphatic tissue, both of which play an important role in the formation of new blood. Many forms of the disease are highly treatable, and with the proper care and medication can stay in remission for years at a time. Others are more serious and more likely to be fatal. Plasma cell leukemia, often simply abbreviated to PCL in the medical community, is usually one of these.
Progression and Specifics
Plasma cells are a type of white blood cell that produce antibodies and play a very important role in the body’s immune response. One of the hallmarks of PCL is a mutation in the form and regeneration of plasma cells that causes them to grow much faster than normal, and that prompts them to attack not foreign entities, as they would in a healthy person, but rather to attack healthy tissues and other cells. They also tend to cause clusters in the bones and create lesions that can lead to fractures.
The bone lesions also interfere with the body's ability to produce normal cells to fight infection and carry oxygen through the body. When the abnormal cells attack the bone, it can lead to increased calcium levels, which can cause a strain on the kidneys, as well as confusion and weakness. The increased calcium levels are usually the result of calcium from the diseased bone entering the bloodstream as the bone dissolves. Most of the time the disease progresses very quickly and the plasma mutations impact virtually every organ and tissue in the body.
Fatigue and frequent infections are two of the most common early symptoms, though neither of these is usually serious enough to warrant blood testing for leukemia, at least not at first. Most patients don’t receive a diagnosis until their problems become debilitating or cause significant interferences with their lives, and most of the time at this point the disease has spread to many different parts of the body and is all but unstoppable.
In most cases fatigue happens because the plasma clusters and mutations prevent red blood cells from traveling their normal routes, which usually leads to decreased oxygenation. Blood oxygen is critical for things like muscle movement and strength, and low levels often lead to exhaustion and lethargy.
Infections, such as shingles, skin infections, pneumonia, and bladder and kidney infections, are common in individuals with PCL. These infections develop because the abnormal cells prevent the body from producing antibodies. With the immune system compromised, these infections tend to become rampant.
Overlap with Myeloma
A related blood cancer known as myeloma often looks similar, at least in terms of major symptoms and progression. In fact, a number of cases of PCL develop as complications of multiple myeloma, usually in its latest phases. Myelomas begin as mutated plasma cells in the bone marrow that squeeze out healthy plasma cells, rather than attacking them or forming harmful lesions; the crowding also tends to weaken the bones from within and can cause protein build-ups that can damage the kidney, among other organs.
In general, PCL is more aggressive and has poorer survival rates than myeloma in its various forms. Individuals with PCL tend to have a greater frequency of organ involvement, a greater number of tumors, high blood calcium levels, and lower platelet counts. The biggest exception is PCL that develops out of a previously diagnosed myeloma; in these cases the condition typically leads to a quick death, especially if the patient has already undergone the most aggressive treatments available.
The prognosis for PCL in any of its forms isn’t usually very good. Treatment typically includes aggressive chemotherapy, as well as stem cell transplants. Side effects of this treatment include weakness, anemia, and nausea. Aggressive chemotherapy regimens, using a combination of drugs, can increase the survival rates from two to six months to 18 to 20 months. Stem cell transplants may provide a two to three year disease-free survival window, but few patients live longer than a year once they’ve received a diagnosis.