Chronic myeloid leukaemia (CML)

Synonyms in a broader sense

Leukemia, white blood cancer, Philadelphia chromosome

Definition

CML (chronic myeloid lekemia) shows a chronic, i.e. rather slowly progressing course of the disease. This leads to the degeneration of a stem cell, which is a precursor of granulocytes in particular, i.e. cells that are important for the defence against mainly bacteria.

Frequency

There are 3/100000 new cases per year. Especially people around 60 years are affected. The average age of the disease is 65 years.

In principle, all age groups can fall ill, but children are only rarely affected. In Germany, for example, only about 1-2 children per 1 million inhabitants fall ill with chronic myeloid leukaemia every year. Due to the rarity of the disease in childhood, it is therefore not surprising that there are currently few meaningful studies and data on this topic.

However, there are indications that childhood CML may be more likely to develop more aggressively. Under certain circumstances, the disease may even represent an independent clinical picture in children. Therapeutically, it is possible to formulate about the same goals as for adult sufferers.

However, since children are much more susceptible and sensitive to the side effects of modern therapies, therapeutic decisions must be considered very carefully. Often children need decades of treatment. An important goal is therefore often to avoid a life-long therapy with the modern tyrosine kinase inhibitors. Current studies are also looking at ways of “stopping therapy”.

Causes

At present, it is relatively unclear why this disease occurs, but irradiation (as in a nuclear reactor accident) or certain substances (benzene) are factors that could lead to the disease. However, in about 90% of cases the Philadelphia chromosome is found, which is the result of a chromosomal mutation. This involves a reciprocal translocation of fragments of chromosomes 9 and 22.

Symptoms

Three phases are typical for this disease: Stable phase: Often only a performance kink is visible, as is weight loss. There are relatively untypical symptoms in this phase. Transition phase: A rapid enlargement of the spleen (splenomegaly) often occurs, which can lead to abdominal pain, for example.

Fever is also relatively common here. The loss of weight and the performance kink can increase. Blasts: The so-called blasts are early precursors of granulocytes.

In this phase the body is flooded with the degenerated cells. The symptoms therefore increase rapidly. Anemia and increased bleeding (due to the displacement of platelet formation) can also occur.

You can tell from these symptoms whether you may have leukaemia and should have this checked by your doctor. In contrast to acute forms of leukaemia, chronic leukaemias can go unnoticed for many years. Especially in the early stages, there are, if at all, rather unspecific symptoms such as chronic fatigue, fever or unintentional weight loss.

Therefore, it is not so easy to detect CML without further ado. Only in the two advanced stages (acceleration phase and blast crisis) do sufferers suffer from more serious symptoms. The family doctor often recognizes changes in the blood count by chance.

These include, for example, a strong increase in white blood cells (leukocytosis) in various forms. In addition, leukaemia cells, the so-called “blasts”, can often be detected in the blood. An ultrasound examination of the abdomen often reveals a spleen, sometimes massively enlarged.

If the indications of chronic myeloid leukaemia become more pronounced, there is usually a detailed clarification in hospital. Here special examinations of the blood and bone marrow can be carried out. You can find general information on this topic at: How to recognize leukemia?