What is multiple myeloma?
Kahler's disease, also called multiple myeloma (MM) is a malignant proliferation of a certain kind of white blood cells: plasma cells. Plasma cells make a contribution in the fight against infectious diseases. For immunoglobulins (blood proteins) to. The incidence of multiple myeloma 4.3 per 100,000 (figures from the u.s., 2000). Multiple myeloma comes in people with darker skin tones twice as common as in white people, and slightly more often in men than in women. The first documented case of multiple myeloma in 1844 was described by Samuel Solly. It concerned the 39-year-old Sarah Newbury; She was suffering from fatigue, bone pain and bone fractures. Kahler's disease is named after the Austrian physician Otto Kahler.
History
History of discoveries and treatments related to multiple myeloma:
- 1844 Description of the first case by Solly, treatment with rhubarb and orange peel.
- 1845 Abnormal protein, later Bence Jones protein called present in the urine. Treatment with steel and quinine (T. Watson).
- 1895 Description of plasma cells.
- 1928 Description of the first large series multiple myeloma patients.
- 1938 Identification of protein-peak in serum.
- 1947 Treatment with urethane (N. Alwall).
- 1956 Characterization of the "light chains" (also called free-chains).
- 1958 Treatment with melphalan (N. Blokhin).
- 1962 Treatment with corticosteroids (S Maas)
- 1975 Classification of the gradations of multiple myeloma using the Durie-Salmon system.
- 1983 autologous (from the patient) bone marrow transplantation (TJ McElwain and RL Powers).
- 1999 Treatment with thalidomide (S. Singhal and B. Barlogie).
- 2002 Treatment with bortezomib (R.Z. Orlowski).
- 2002 lenalidomide (PG Richardson and KC Anderson).
- 2005 Classification of the gradations of multiple myeloma by using the International Staging System.
- 2005 Cytogenetic format.
- 2011 Treatment with carfilzomib
- 2013 Treatment with daratumumab (HuMax-CD38)
Multiple myeloma symptoms
These haematological disease, responsible for about 10% of all hematologic cancers is characterized by ingestion of the bone marrow by malignant plasma cells. These malignant plasma cells (a white blood cell that secretes immunoglobulins or antibodies) of producing in excess of a given type of immunoglobulin, or parts of it (the so-called free or light chains). If it is this excess of one type of immunoglobulin found in the serum and / or urine, is referred to as a monoclonal gammopathy. The presence of free or light chains (kappa or lambda) in urine is called Bence-Jones proteinuria. So, this is a type of protein in the urine.
Take the bone marrow if malignant plasma cells allows the formation of red blood cells, platelets and white blood cells and neutrophils in jeopardy. This allows than respectively anemia, bruising and infections occur. However, the first symptoms are often bone pain caused by so-called Osteolytic lesions. The malignant plasma cells produce namely OAF (Osteoclast Activating Factor), a protein that promotes bone destruction. By this bone destruction can raised blood calcium levels (hypercalcemia), which may result in nausea, decreased appetite, neurological abnormalities, itchy eyes and constipation. In the long run, nierbeschadigingen can also occur as a result of increased protein and calcium excretions. As a result, the bones become brittle and painful. Also pathological bone fracture and compression of the spinal cord can occur in these patients. Kahler's disease are many faulty proteins (immunoglobulins) produced, for example, which can precipitate in the kidneys. Renal impairment can also occur at the illness of Kahler. If also amyloid (part of the defective proteins) is dropped off, one can speak of the disease Amyloidosis.
Multiple myeloma diagnosis
The diagnosis is made by means of a bone marrow examination (puncture at the height of the sternum or your hip), the quantity of the immunoglobulins in the serum / urine (protein electrophoresis with immunofixation), the presence of a proliferation of plasma cells and / or the presence of bone lesions of the skeleton. The latter can be determined by a series of multiple myeloma, wherein there radiographs are taken of the long bones, the vertebrae and pelvis.
Multiple myeloma treatment
The most commonly used treatments for multiple myeloma include:
- chemotherapy (treatment with cell division inhibitory drugs), usually melphalan
- radiation (radiotherapy)
- supportive therapy
The best results (in patients younger than 65 years) are achieved with a heavy course of chemotherapy followed by autologous stem cell transplantation (involves own cells). In special cases chosen for allogeneic stem cell transplantation (cells from another person, usually a family member). The treatment may consist of a first autologous transplantation and a few months later by an allogeneic transplant. Or allogeneic stem cell transplantation is of great value in the treatment of multiple myeloma, is still the subject of research.
The treatment consists of supportive antibiotics, blood products, corticoids, allopurinol and bisphosphonates (to counter the bone loss).
Symptomatic multiple myeloma treated with chemotherapy (+ radiotherapy). There are various chemotherapeutics regimes depending on the age of the patient and degree to which the disease has progressed. After 4-6 cycles of chemotherapy is usually reached a stable phase. Sometimes the stable phase is maintained with medication (eg thalidomide or alpha interferon). Many patients, however, have a relapse (relapse), the median survival in 2003 was about 4½ years. Thanks to modern methods of treatment patients for many years (more than 10 years) remain disease free.