Leukemia • Symptoms of blood cancer

Acute leukaemias can occur at any age and are characterized by the sudden appearance of violent symptoms. Chronic leukemia occurs primarily in adults and usually has a gradual course.

Compared to other types of cancer, leukaemias are relatively rare. In Germany, about 2.7 percent of tumors in women and 3.1 percent of tumors in men are a type of leukemia. In total, around 14,000 people in Germany develop blood cancer every year.

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What is leukemia?

The term leukemia stands for a whole group of cancers, all of which have their origin in the cells of the hematopoietic system. Blood cells of all types are derived from a common hematopoietic stem cell in the bone marrow. The blood cells go through numerous preliminary stages until maturity and in each of these development phases they can degenerate, i.e. become malignant.

There are four main types of leukemia:

• acute myeloid leukemia (AML)

• acute lymphoblastic leukemia (ALL)

• chronic myeloid leukemia (CML)

• chronic lymphoblastic leukemia (CLL)

The maturation process of the white blood cells (leukocytes) is disturbed in the different types of leukemia because certain control mechanisms of growth have failed. Instead of mature, functional white blood cells, immature and therefore not fully functional leukocytes are formed, which are then released into the bloodstream.

In addition to the lack of function, these immature blood cells have the property of multiplying very quickly and in an uncontrolled manner. The normal blood formation in the bone marrow is increasingly displaced by the large amounts of inoperable or reduced blood cells and healthy white and red blood cells and platelets can no longer be produced to the necessary extent

Acute and chronic leukaemias differ

Depending on what type of leukocytes the leukemia cells come from, a distinction is made between two types of leukemia. Myeloid leukaemias arise from precursor cells of the granulocytes, lymphatic leukaemias develop from precursor cells of the lymphocytes. There are acute and chronic forms of both types. As a rule, the symptoms of the acute forms develop very quickly, the symptoms are very severe and treatment must be started immediately. Chronic leukaemias, on the other hand, are generally slowly progressing diseases with few symptoms that often go unnoticed over a long period of time.

What are the symptoms of leukemia?

The signs of leukemia may initially resemble those of other harmless diseases. Those affected often feel tired, worn out and reduced in performance. Other very unspecific symptoms are pallor, fever and susceptibility to infection. There are also complaints such as unwanted weight loss, lymph node swelling, skin changes or upper abdominal pain. These then usually lead to targeted diagnostics.

The typical symptoms of leukemia are:

• unclear fever

• night sweats

• Loss of appetite and unwanted weight loss

• frequent infections because the immune system is not working properly

• Signs of anemia such as fatigue and reduced performance, pallor, dizziness, rapid heartbeat and shortness of breath

• Lymph node swelling, especially on the neck, armpits and groin, usually not painful

• Skin changes such as petechiae (tiny, punctiform bleeding), especially on the arms and legs, rash, gum proliferation

• Bleeding tendency (gums, nosebleeds, bruising). Injuries bleed for an unusually long time

• Abdominal pain due to enlargement of the liver and spleen

• Shortness of breath, even with light physical exertion

• Bone pain due to the spread of leukemia cells in the bone marrow

• Headache, dizziness, feeling disorders or paralysis due to an attack on the central nervous system by leukemia cells

Causes and risk factors for blood cancer

There are certain risk factors that increase the risk of developing leukemia. So far, however, it has not been possible to find a reliable cause for the development of blood cancer and none of the known risk factors can be proven for a large proportion of leukemia diseases.

Risk factors include:

• Assessment: If malignant diseases have occurred frequently within a family, the risk of developing leukemia is also increased. This indicates a certain inherited predisposition to blood cancer. The fact that certain genetic diseases increase the risk of leukemia also suggests a role in the disposition. For example, people with Down syndrome are 20 times more likely to develop acute myeloid leukemia (AML).

• acquired chromosome changes: Changes in the chromosomes (carriers of the genetic material) can often be detected in the leukemia cells. The best known is the so-called Philadelphia chromosome, which can be found in a large percentage of the changed leukocytes in chronic myeloid leukemia (CML). Exchanging gene segments results in a genetic defect that turns a healthy cell into a leukemia cell.

• radioactive beams: The high-energy radiation from radioactive material damages in particular the genome of cells that have a high division rate. These cells also include the bone marrow cells that are responsible for blood formation.

• chemical substances: Direct contact with various chemical substances such as benzene and other organic solvents, insecticides (insecticides) and herbicides (pesticides) increases the risk of developing leukemia. The use of drugs for cancer treatment such as cytostatics and immunosuppressants impair the function of the bone marrow and can promote leukemia in the long term.

• Smoking: It is estimated that ten percent of all leukemia cases are caused by cigarette smoking. The risk of developing acute myeloid leukemia (AML) is said to be increased by 40 percent in active smokers and by 25 percent in ex-smokers.

• Age: With increasing age, the risk of developing chronic form of leukemia or acute myeloid leukemia increases.

How can you detect leukemia?

With a thorough medical history, the current complaints, history and possible risk factors are asked. A physical exam may indicate enlargement of the lymph nodes, spleen, and liver.

This is followed by a blood test that can make statements as to whether white blood cells are malignantly changed and which subgroups of leukocytes are affected by this change. Using modern technologies, cells can be assessed in terms of their appearance and changes in the genome can be identified. In addition, the blood count provides information about the general condition of the person concerned and about the functions of individual organs.

In addition, if leukemia is suspected, a sample of the bone marrow is taken from the pelvic bone or sternum (bone marrow biopsy). The bone marrow is examined for its cellular and fine tissue properties. Statements on the prognosis of the disease can also be made.

Further examinations serve to precisely determine the spread of the disease. For this purpose, ultrasound examinations, x-rays, magnetic resonance imaging (MRI), computer tomography (CT), lumbar punctures (examination of the spinal fluid) and lymph node biopsies are carried out.

How can you treat leukemia?

The treatment of blood cancer depends on the general condition of the person affected and the type of leukemia. The main treatment for all acute leukaemias is chemotherapy. By using cell growth-inhibiting drugs (cytostatics), the leukemia cells in the whole body are to be completely destroyed or at least largely suppressed.

Acute leukaemias are treated in several phases. First, the so-called induction therapy is administered, which destroys the degenerated blood cells. This is followed by consolidation therapy and, in the case of ALL, also maintenance therapy. Both are intended to prevent changed cells from developing again in the bone marrow.

In the case of chronic leukaemias in particular, chemotherapy can be supplemented with antibody therapies, interferon therapies and radiation.

Therapy of leukemia with stem cells

Another option for treating leukemia is stem cell transplantation. The affected patient's bone marrow should be replaced by healthy stem cells. These come from a suitable donor or less often from the patient himself.

Affected people can donate stem cells themselves, this is called autologous stem cell transplantation. However, this is only possible if the cancer cells in the bone marrow have been temporarily pushed back by treatment. Since the benefit of an autologous transplant for the various forms of leukemia is not clearly established, leukemia patients today mostly receive stem cells from a healthy relative or a foreign donor. This is called allogeneic stem cell transplantation. In order to avoid rejection reactions, the tissue characteristics of the donor and recipient must be as identical as possible.

There are three ways to obtain blood stem cells for an allogeneic stem cell transplant:

• Bone marrow stem cells: If the blood stem cells are removed from the bone marrow of the pelvic bone, general anesthesia and hospitalization are required for the donor.

• Peripheral blood stem cells: A special form of blood sampling is necessary to use peripheral blood from the arm vein. For the donor, pretreatment with a growth factor that lasts several days is necessary so that as many blood stem cells as possible from the bone marrow migrate into the blood.

• Umbilical cord blood stem cells: Another source of stem cells is umbilical cord blood from healthy newborns. It contains many blood stem cells, is easy to obtain and can be used if there is less agreement between the commercial characteristics.

A stem cell transplant is an option for those with acute leukemia and chronic myeloid leukemia if the usual treatment has not brought the desired results or if the risk of relapse is very high.

Course and prognosis in blood cancer

Thanks to modern therapies, the chances of recovery and survival for people with leukemia have increased significantly. Appropriate treatments can prolong survival even with advanced diseases. However, the success of treatment strongly depends on when and in which stage of the disease the leukemia was diagnosed and the treatment started. Even if diagnosed early, the course of the disease cannot be predicted exactly.

The chronic forms of leukemia are curable in the rarest of cases, the best healing prospects exist through a stem cell transplant. Since the cancer cells in chronic blood cancer generally only proliferate slowly and the symptoms only gradually get worse, the progression of chronic leukemia can usually be slowed down. The necessary therapy is less aggressive than with acute forms, but must be continued for a long time.

A cure is possible for acute forms of leukemia. The earlier the therapy begins and the younger the affected, the greater the life expectancy. If untreated, the sick usually live for an average of only three months. In all cases, a recurrence of the disease (relapse) significantly worsens the chances of recovery.

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