Definition & Facts
Kahler's disease is a bone marrow cancer, affecting the plasma cells within the bone. Plasma cells are B cells which have responded to an infection in the body and then matured. Once B cells become plasma cells, they are responsible for making antibodies which allow the body to attack and kill future germs.
Plasma cells can grow out of control, becoming cancerous and forming tumors. When an individual has one plasma cell tumor, they have what is referred to as an isolated plasmacytoma. If, however, they have more than one plasma cell tumor, they have Kahler's disease.
A rare form of cancer, multiple myeloma affects about 1 out of 143 people in their lifetime. The American Cancer Society estimates for the United States in 2016 that 30,330 new cases will be diagnosed and 12,650 deaths are expected to occur.
Symptoms & Complaints
- Bone pain, especially back pain, hip pain or skull pain
- Bone weakness, osteoporosis, and increased susceptibility to bone fractures
Low blood counts can also occur and include:
- Reduced red blood cells (anemia) due to plasma cell overgrowth crowding out normal blood cells. Anemia can cause weakness, fatigue, and paleness.
- Reduced white blood cells (leukopenia) due to plasma cell crowding out normal white cells, rendering them unable to produce new antibodies. Leukopenia creates difficulty fighting infections.
- Reduced blood platelet counts (thrombocytopenia) can cause serious bruising and bleeding from only minor cuts and scrapes.
High calcium levels in the blood (hypercalcaemia) may cause
- Extreme thirst and dehydration
- Excessive urination
- Kidney problems and acute kidney failure
- Severe constipation
- Abdominal pain
- Loss of appetite
- Coma (if high enough)
A number of problems with the central nervous system and peripheral nervous system can ensue. If bones or the spine become weakened, spinal cord compression may result in sudden, severe back pain; numbness or muscle weakness, especially in the legs; and paralysis. Protein toxicity can lead to nerve damage resulting in weakness or numbness as well.
Kidney problems that can result from Kahler's disease include:
Multiple myeloma can cause infections, increased susceptibility to infections, prolonged duration of infections, slow response to infection treatment, strong susceptibility to pneumonia, and light chain amyloidosis.
Heart problems include irregular heartbeat (cardiac arrhythmia), enlarged heart (cardiomegaly), shortness of breath, fluid buildup in legs and feet (edema), enlarged liver (hepatomegaly), and enlarged spleen (splenomegaly).
Skin changes can also ensue and include color or texture changes, intense bleeding/bruising, “Raccoon eyes” or the darkening of the skin around the eye sockets.
Although it is still unclear exactly what determines if an individual with develop multiple myeloma, some common links have been discovered as well as certain risk factors to the development of the disease. One of these links is the finding of abnormalities among the dendritic cells. These abnormalities may cause too much plasma cell growth hormone to be released, resulting in the excessive production of the IL-6 hormone.
Another potential cause is known as translocation. This is believed to be the cause of about half of myeloma cases. Translocation occurs when part of one chromosome switches with part of another chromosome in myeloma cells. This can cause oncogenes, the genes that promote cell division, to be turned on, thereby spreading the cancer cells. It has been noted that a common finding in myeloma cells is that parts of chromosome 13 are missing.
Kahler's disease seems to be most common in those ages 65 and older, is slightly more common in men than women, and occurs twice as commonly among African Americans than white Americans. An individual with a parent or sibling with the disease is approximately four times as likely to get it as well. Obesity or being overweight is a risk factor for developing the disease.
Diagnosis & Tests
A complete blood count is a laboratory test that measures the levels of red cells, white cells, and blood platelets, and is used to determine if levels are off. A quantitative test may also be used to measure the blood levels of different antibodies.
Additionally, a test known as electrophoresis measures immunoglobulins to determine if any are monoclonal immunoglobulin (also known as M protein,) meaning there are several copies of the same antibody. This protein may be found in blood or urine.
Treatment & Therapy
Treatment begins once myeloma has been found and staged by a doctor. Often multiple treatment approaches are used in conjunction with one another. Depending on what stage the myeloma is at, treatment may include:
- Intravenous immunoglobulin (IVIG). Because individuals with multiple myeloma are at an increased risk for infection, IVIG treatment may be used to raise antibody levels and prevent infections by injecting the patient with donor antibodies.
- Chemotherapy & other drugs. Chemotherapy involves using a combination of several drugs to destroy or gain control over the cancerous cells.
- Bisphosphonates. Because individuals with Kahler's disease suffer from bone weakening and damage, drugs which can slow down the process of bone weakening may be given intravenously, usually once a month. Two of these drugs are pamidronic acid and zoledronic acid.
- Radiation. This treatment involves using focused high energy X-rays to destroy cancer cells. The most common type is external beam radiation therapy, in which radiation is aimed at cancer from a machine outside the body. Radiation can also be used to destroy damaged bone areas that aren’t otherwise responding to chemotherapy.
- Surgery. Although rare in the treatment of multiple myeloma, emergency surgery may be necessary if spinal cord compression causes paralysis. Surgery may also be elected to attach metal plates or rods that can support weak bones or fractures.
- Stem cell transplant. With this treatment the patient first receives a high dose of chemotherapy to kill the cells in the bone marrow, which are then replaced with new, healthy cells in one of two ways. The first, known as autologous stem-cell transplantation, uses the patient’s own cells. This process will need to be repeated, as it is not a cure, and the myeloma will return. The second, known as allotransplantation, uses donor cells. This process is much riskier, but may fight the cancer better. This is not a standard treatment approach for Kahler's disease but may be used in clinical trials.
- Plasmapheresis. A catheter either in the neck, collarbone, or groin, is hooked up to a machine which vacuums the blood into it. Plasma is filtered and blood cells are returned to the patients with saltwater or donor plasma and the abnormal plasma are discarded. The procedure does not kill the myeloma, but lowers bad protein levels and eases symptoms.
Prevention & Prophylaxis