Definition and causes
All blood cells develop from a stem cell , Which divides into 2 lines and forms themyeloidstem cell and thelymphoidstem cell (the latter form a part of the white blood cells). The myeloid stem cell develops roughly in 3 different lines, resulting in the formation of red blood cells, a part of the white blood cells (granulocytes) and platelets (see the blood in general ). During this development are the cells in different precursors, where there may be errors.
The CML is a characteristic kromosomfejl with the presence of the so-calledPhiladelphia chromosome, Which gives an uncontrolled growth of a specific cell in the bone marrow. This leukæmicelle is a prelude to the formation of granulocytes, a subgroup of the white blood cells, and are therefore formed a mass of cells of this type, which can both be found in the blood and bone marrow.
The reason why there is this error in the chromosome, is not known, but it results in the celleforstadiet to granulocytten will overproduceret, while the cell natural cell death prevented. This leads to more cells, which live longer than normal, and they will typically camps in the spleen.
There are about. 75 new cases per. years in Denmark. CML diagnosed frequently aged 45-60 years, and men are affected more by the disease than women.
The disease is divided into 2 phases:
- The chronic phaseis a few years, and there are clear cell in the blood and bone marrow. There is often very scarce symptoms in this period, and the only signs can be persistent fatigue.
- The accelerated phaseWhere you start to develop symptoms in the form of:
- Signs of anaemia (anaemia), such as fatigue, pallor and dizziness.
- The trend for bleeding (due thrombocytopenia ) Which, inter alia, emerges as nosebleeds and bruising.
- Infections with fever and malaise.
- Bone pain.
- In severe cases, you can feel the spleen dive off the left side just below the ribs.
Precautions and diagnosis
Developer Monday the above symptoms should seek medical that will take a blood sample. This test will show an increased number of granulocytes, and an increased number of the cells in forstadiet before the development of granulocytes. There may be a knoglemarvsundersøgelse , Which will show increased number of cells.
The diagnosis is made frequently in the chronic phase, which has experienced a prolonged fatigue, combined with a very large bulge (enlarged spleen) on the left side.
The treatment of CML is a specialistopgave, which take place at the major hospitals. A bone marrow (KMT) is still the most preferred procedure of the younger people in the 20-30 years of age. Before the KMT provides treatment chemotherapy , Which eliminates blood cells in bone marrow, blood and spleen. It is best to start treatment as soon as the diagnosis is provided.
New technology has paved the way for the production of a substance (Glivec), which blocks the formation of this specific leukæmicelle. This substance works positively to over half of the sick, but since it is a substance which has reached him during the last few years, know we do not langtidsudsigten for the effect.
In the accelerated phase dealt also with blood against anaemia and antibiotics for infections.
Younger people, where the chemotherapy works best, and which have made a bone marrow without complications, has the best forecast. Elderly, which may clear a bone marrow (KMT), has also reasonable forecast, while older people who only receive chemotherapy, has a poor prognosis.