leukemia

Leukemia: short overview

• What is leukemia? Group of cancers of the hematopoietic system. Also called “blood cancer” or “leucosis”.
• Common forms: Acute myeloid leukemia (AML), Acute lymphoblastic leukemia (ALL), Chronic myeloid leukemia (CML), Chronic lymphocytic leukemia (CLL, actually a form of lymphoma)
• Possible symptoms: Tiredness and fatigue, reduced efficiency, rapid fatigue, blemishes, tendency to bleeding and bruising (hematoma), tendency to infections, fever of unclear origin, weight loss, night sweats etc.
• Frequency: Every year, 13,700 people in Germany contract leukemia, usually between the ages of 60 and 70. Men are slightly more affected than women. About four percent of the patients are children under the age of 15.
• Treatment options: depending on the type and stage of leukemia; e.g. Chemotherapy, tyrosine kinase inhibitors, interferons, monoclonal antibodies, radiotherapy, stem cell transplantation etc.
• Forecast: Acute leukemia is often curable if it is detected and treated in good time. In chronic leukemia, therapy can prolong the survival of many patients. Healing is possible here at most by a high-risk stem cell transplantation.

Leukemia: symptoms

Leukemia can suddenly manifest itself with symptoms and run fast. Doctors then speak of acute leukemia, In other cases, the blood cancer develops slowly and slowly. Then it is a question chronic leukemia.

Acute leukemia: symptoms

The symptoms of acute leukemia develop relatively quickly. Symptoms of both acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) include:

• reduced efficiency
• persistent fever
• nocturnal sweating
• fatigue
• weight loss
• Bone and joint pain (especially in children with ALL)

The body of the patient produces large quantities of immature white blood cells (Leukocytes). These displace the healthy blood cells, ie mature leukocytes, red blood cells (erythrocytes) and platelets (thrombocytes). This causes more leukemia signs. So does the lack of red blood cells anemia (Anemia). The victims suffer for example under:

• paleness
• palpitations
• shortness of breath
• dizziness

The lack of platelets in acute leukemia often causes one increased bleeding tendency, For example, patients often have gum or nose bleeds. If injured, it takes longer than usual for a wound to stop bleeding. In addition, the patients get increased bruises (Hematoma) – another typical sign. In case of severe deficiency of platelets (thrombocytopenia), red haemorrhages occur in the skin called petechiae.

Leukemia can also weaken infection defense. As a result, patients often suffer stubborn infections like badly healing inflammation in the oral cavity. The reason: The body of the patient has too little functioning white blood cells – and these are usually used to ward off infection. The immune system in leukemia is thus weakened overall.

Other possible leukemia symptoms are:

• painless swollen lymph nodes
• enlarged liver and spleen
• rashes
• Gingival overgrowth

Chronic leukemia: symptoms

Chronic leukemia begins insidiously. In the first months or even years many patients have no complaints. Some report only general symptoms, such as tiredness and decreased performance. These are usually not recognized as signs of leukemia. That’s why most patients do not go to the doctor. It is only at an advanced stage that chronic leukemia develops symptoms that resemble an acute course.

In the Chronic myelogenous leukemia (CML) There are three phases in which the disease becomes increasingly aggressive. This is also shown by the leukemia signs:

• Chronic phaseHere, the number of white blood cells is abnormally elevated (leukocytosis) and the spleen is enlarged (splenomegaly). The latter can cause a feeling of pressure in the left upper abdomen. Other leukemia symptoms at this stage include fatigue and decreased performance.

• Acceleration phase (transition phase): The number of leucocytes continues to increase. At the same time, the number of red blood cells and platelets decreases. Typical symptoms of CML are now skin blanching, palpitations, shortness of breath and frequent nose and gum bleeding. Night sweats and fever can also occur. The liver is increasingly enlarged.

• Blast crisis (blast burst): In this last stage of the disease, the bone marrow releases large quantities of immature precursors of blood cells (called myeloblasts and promyelocytes) into the blood. This causes symptoms similar to those of acute leukemia. Most patients die soon.

The Chronic lymphocytic leukemia (CLL) is also progressing slowly. That’s why the term “leukemia” is in their name. Actually, this is not a blood cancer, but a special form of lymphoma (malignant lymphoma).

Overview of leukemia forms

The four main forms of leukemia are:

Leukemia Shape	Remarks
Acute myeloid leukemia (AML)	– starts quite suddenly and progresses quickly – most common acute leukemia – About half of the patients are older than 70 years
Chronic myeloid leukemia (CML)	– slow, gradual course (except in the last stage: blast crisis) – mean age of onset at 50 to 60 years – very rare in children
Acute Lymphocytic Leukemia (ALL)	– starts quite suddenly and progresses quickly – most common of all leukemia forms – v.a. in children (ALL is the most common type of cancer in children); adult patients mostly older than 80 years
Chronic lymphocytic leukemia (CLL)	– slow, gradual course – most common leukemia in adults; mean age of onset at 70 to 75 years – is not one of the “true” leukemias, but to lymph node cancer (malignant lymphoma)

There are other types of leukemia, but they are very rare. An example is hairy cell leukemia.

Related to the leukemia are the so-called myelodysplastic syndromes (MDS), These are also chronic diseases of the bone marrow, are formed in the insufficiently functioning blood cells. The symptoms are similar to Chronic Myelogenous Leukemia. But they are initially less pronounced. In approximately 25 to 30 percent of patients, the myelodysplastic syndrome sooner or later produces a full-blown leukemia, namely acute myeloid leukemia.

Myeloid leukemia

Myeloid leukemias are caused by the so-called myeloid precursor cells in the bone marrow. From these progenitor cells normally develop healthy red blood cells, platelets and granulocytes and monocytes. The last two are subgroups of white blood cells.

However, when myeloid precursor cells degenerate and begin to grow uncontrollably, myeloid leukemia develops. Depending on their course, doctors distinguish the Acute Myeloid Leukemia (AML) and the Chronic Myeloid Leukemia (CML), Both types of blood cancer are especially common in adults. The AML is significantly more common than the CML.

Read more about the two forms of myeloid blood cancer in the article Myeloid leukemia.

Lymphatic leukemia

Lymphatic leukemias are based on blood cell precursors other than myeloid blood cancer: the so-called lymphatic progenitor cells degenerate here. Out of them against the lymphocytes. This subgroup of white blood cells is very important for the specific (specific) defense against foreign substances and pathogens (specific immune defense).

Again, one speaks of depending on the disease of Acute Lymphatic Leukemia (ALL) or Chronic Lymphocytic Leukemia (CLM), ALL is the most common form of blood cancer in children and adolescents. By contrast, CLL typically occurs in adulthood. It is referred to as “leukemia” (blood cancer) only because of its course. Actually, the CLL is a form of lymph node cancer – it is one of the so-called non-Hodgkin’s lymphoma.

More about these two cancers can be found in the article Lymphatic Leukemia.

Hairy cell leukemia

Hairy cell leukemia (or hairy cell leukemia) is a very rare cancer. For them, the same applies as for the chronic lymphocytic leukemia: The name part “leukemia” only indicates that the disease is like blood cancer. However, it is attributed to lymph node cancer (more precisely: non-Hodgkin’s lymphoma).

The name part “hair cells” comes from the fact that the cancerous cells have hairy extensions.

Hairy cell leukemia occurs only in adulthood. Men fall significantly more often than women. The chronic disease is not very aggressive. Most patients have a normal life expectancy.

You can read all about this cancer in the article Hairy cell leukemia.

Leukemia in children

Above all, leukemia is a disease of adults: they account for about 96 percent of all patients. When childhood leukemia develops, it is almost always acute lymphoblastic leukemia (ALL). Second is acute myeloid leukemia (AML). Chronic leukemia is very rare in children.

If acute childhood blood cancer is detected and treated early, the chances of recovery are good. In comparison, acute leukemia in adults is more of a poor prognosis.

You can find out all about blood cancer in children in the article on leukemia in children.

Leukemia: treatment

The leukemia treatment is individually adapted to each patient. Various factors play a role here. In addition to the age and general state of health of the patient, this is above all the course of the disease (acute or chronic).

Acute leukemia: treatment

As soon as possible after the diagnosis “Acute leukemia” patients with a chemotherapy kick off. It is considered to be the most important therapy for acute blood cancer. The patient receives special drugs, so-called cytostatics (chemotherapeutic agents). They prevent cancer cells (and other rapidly dividing cells) from growing. The damaged cells can not multiply any further. They are then recognized by the body’s own control mechanisms and targeted dismantled.

Most are the Cytostatics as an infusion administered directly into a vein (as an infusion), but occasionally also as tablets ingested. They can be given individually or in combination as well as in different dosages. This allows chemotherapy to be individually adapted to each patient. The treatment also takes place in cycles: The patient receives the cytostatics one day or several days in a row. This is followed by a treatment break (days to months) before a new cycle is started. Most cancer patients receive an average of four to six such chemotherapy cycles.

Basically, acute leukemia therapy takes place in three phases, which can last for months and years:

1. induction therapy: Patients here receive strong chemotherapy that aims to eliminate as much as possible of all cancer cells and alleviate the most severe symptoms. The treatment is usually carried out in hospital.
2. consolidation therapy: It is designed to “solidify” the success of induction therapy. Many patients receive adapted chemotherapy to eliminate any remaining cancer cells.
3. maintenance treatment: The aim here is to stabilize the success of treatment and to prevent relapse (recurrence). The maintenance therapy can be designed very differently from patient to patient. Often, cytostatics are given in tablet form for at least one year.

Induction therapy can be so successful that virtually no cancer cells can be detected in the patient’s blood and bone marrow. Doctors then speak of a remission. It does not mean that the leukemia is cured. It may still have survived individual cancer cells. Therefore, further therapy steps (consolidation therapy) are necessary.

After the maintenance therapy closes the aftercare on: Blood and bone marrow of the patient are examined regularly. If it comes to a relapse, the cancer cells can be detected early in this way. In addition, aftercare is about treating any side effects and long-term effects of previous chemotherapy regimens.

Further therapy options

Sometimes one isstem cell also part of the leukemia treatment. The stem cells are the “mother cells” that make up all the blood cells in the bone marrow (for life). Prior to transplantation, high-dose chemotherapy (and possible whole body irradiation) destroys virtually all of the patient’s bone marrow and (hopefully) all cancer cells. Thereafter, the patient is given healthy stem cells as in a transfusion. The cells settle in the medullary cavities of the bones and produce new, healthy blood cells.

In most leukemia, stem cells are transmitted from a healthy donor (Allogeneic stem cell transplantation). Rarely, it is the patient’s own stem cells that were taken from him before bone marrow destruction (Autologous stem cell transplantation). The method of treatment is particularly useful when other treatments (especially chemotherapy) are not effective or the patient relapses.

Many patients with acute lymphoblastic leukemia (ALL) receive one in addition to chemo radiotherapy, On the one hand, the head is irradiated as a precaution, as the cancer cells more frequently attack the brain. On the other hand, radiation can be used to specifically treat malignantly altered lymph nodes (for example in the chest area).

Chronic leukemia: treatment

The Chronic Myeloid Leukemia (CML) is usually discovered in the chronically stable disease phase (see above). The doctor then prescribes mostly so-called tyrosine kinase inhibitor (like imatinib). These drugs are very effective against blood cancer cells: they inhibit growth signals in the cells. This can stop the disease for many years. The tyrosine kinase inhibitors are taken as a tablet, usually for life.

At the same time, the blood and bone marrow of the patients are regularly checked. If, for example, the blood levels or the patient’s condition deteriorate, this indicates that the CML is moving into the next phase (acceleration phase). The doctor then changes the drug treatment: he prescribes other tyrosine kinase inhibitors. Thus, in many patients, the disease can be traced back to a chronically stable phase.

If that does not succeed, one may come Allogeneic stem cell transplantation in question – ie the transplantation of healthy, blood-forming stem cells of a donor. So far, this is the only form of therapy that has the potential to completely cure Chronic Myeloid Leukemia. However, it is very risky. Therefore, careful consideration and potential risks of treatment are carefully considered in each patient.

At any stage of the disease, a patient’s condition may deteriorate significantly within a short period of time. Then doctors speak of a blast crisis. Those affected receive an intensive treatment, as in the case of acute leukemia chemotherapy, So you try to repress the signs of the disease as quickly as possible. If the patient’s condition has improved and stabilized, a stem cell transplantation may be useful.

Some patients with CML will be with interferons treated. These are messenger substances with which the cells of the immune system communicate with each other. They can inhibit the growth of cancer cells. However, interferons – as well as chemotherapy – are usually less effective in CML than the tyrosine kinase inhibitors described above.

However, this is not always the case: tyrosine kinase inhibitors work best in patients whose cancer cells have the so-called “Philadelphia chromosome”. This is called a characteristically altered chromosome 22. It can be detected in more than 90 percent of all CML patients. The remaining patients do not have the altered chromosome. In these cases treatment with tyrosine kinase inhibitors often does not work so well. Then it may be necessary to change the therapy and to use, for example, interferons.

The Chronic lymphocytic leukemia (CLL) does not require treatment for a long time in many patients. Only when the blood levels worsen or when symptoms develop in the advanced stage, medics initiate a therapy – adapted to each individual patient.

For example, many sufferers receive one chemotherapy plus so-called Monoclonal antibodies (Immunochemotherapy or chemoimmunotherapy): The artificially produced antibodies bind specifically to the cancer cells and thereby mark them for the immune system. Both therapies are occasionally used individually.

If the cancer cells have certain genetic changes, treatment with TKI make sense. These drugs block a pathologically altered enzyme that promotes the growth of cancer cells.

When other treatments do not work or you later relapse, doctors sometimes have one stem cell by: The CLL patients are transferred after healthy chemotherapy healthy, blood-forming stem cells of a donor (allogeneic stem cell transplantation). This risky treatment is only suitable for young or very fit patients.

Accompanying measures (supportive therapy)

In addition to the leukemia treatment by means of chemotherapy, radiotherapy & Co., supportive measures are also very important. They serve, for example, to reduce symptoms of the disease and consequences of the treatment. This can greatly improve the well-being and quality of life of patients.

For example Nausea and vomiting common and very unpleasant side effects of chemotherapy for leukemia (and other cancers). They can be relieved with special medications (antiemetics).

The increased susceptibility to infections is also a serious problem with leukemia. Both the disease itself and chemotherapy weaken the immune system. It can then fight off pathogens worse. This favors infections, which then also can be very difficult. Sometimes they even become life threatening! That’s why a careful hygiene and one possible low-germ environment very important for leukemia patients. Many receive as well antibioticsto prevent or treat bacterial infections. Also against fungal infections, there are special agents, so-called antifungals.

Also, other complaints can often be specifically treated, for example, anemia (anemia) means blood transfusion and pain with appropriate painkillers.

Leukemia: causes and risk factors

The causes of the different forms of blood cancer have not yet been clarified. However, experts have identified several risk factors that promote the development of leukemia. These include:

Genetic predisposition: The risk of leukemia is slightly elevated if cancer has occurred more often in one’s own family. In addition, certain genetic diseases make them more susceptible to blood cancer. For example, people with trisomy 21 (Down syndrome) are 20 times more likely to have acute myeloid leukemia (AML) than people without this genetic modification.

Age: The development of acute myeloid leukemia (AML) is influenced by age: the disease risk increases with the years of life. The same applies to Chronic Myeloid Leukemia (CML) and Chronic Lymphocytic Leukemia (CLL). In contrast, acute lymphoblastic leukemia (ALL) occurs mainly in childhood.

Smoke: Smoke accounts for about ten percent of all leukemia cases, researchers estimate. For example, in active smokers, the risk of acute myeloid leukemia (AML) is 40 percent higher than in people who have never smoked. In former smokers, the risk of disease is still increased by 25 percent.

Ionizing rays: By this one understands different energetic rays, for example radioactive rays. They damage the genetic material – especially in those body cells that often divide. These include the blood-forming cells in the bone marrow. As a result, leukemia can develop. The higher the radiation dose that is applied to the body, the greater the leukemia risk.

Radiation therapy for cancer also uses such ionizing radiation. Not only can they kill the cancer cells as desired, but they can also damage the genome in healthy cells. In rare cases, patients develop a radiation-related second cancer.

X-rays are also ionizing. However, experts assume that an occasional X-ray examination can not trigger leukemia. Nevertheless, you should only make x-rays if absolutely necessary. Because the damage caused by the rays in the body, can add up in the course of life.

Chemical substances: Different chemicals can increase the leukemia risk. These include, for example, benzene and other organic solvents. Also insecticides (insecticides) and pesticides (herbicides) are suspected to promote blood cancer.

This connection is certain for certain drugs that are actually used to treat cancer (such as cytostatics): they can promote the development of leukemia in the long term. Therefore, before they are used, physicians carefully weigh the benefits and risks of such drugs.

virus: Certain viruses (HTL viruses I and II) are involved in the development of a very rare type of leukemia. People in the Japanese area are mainly affected by this so-called human T-cell leukemia. With us this blood cancer variant is extremely rare.

All other forms of leukemia (AML, CML, ALL, CLL, etc.), according to the current state of knowledge, arise without any involvement of viruses or other pathogens.

Leukemia: examinations and diagnosis

While chronic leukemia usually remains symptom-free for a long time, acute forms start relatively suddenly and progress rapidly. Symptoms such as reduced performance, paleness, rapid heartbeat, frequent nosebleeds or persistent fever, however, also occur in many other and sometimes harmless diseases. That’s why they are not always taken equally seriously. However, such complaints are always suspected to be leukemia. Therefore you should definitely go to the doctor.

The first contact for suspected blood cancer is the family doctor. If necessary, he will refer the patient to a specialist, for example to a specialist in blood and cancer (haematologist or oncologist).

Conversation and physical examination

The doctor will be the first medical history to raise (anamnesis). To do this, he asks how the patient generally feels, what the symptoms are and how long they have lasted. It may also be important to provide information on any other illnesses that currently exist or have occurred previously. In addition, the doctor asks, for example, if the patient receives any medications and if family cancers are known.

This is followed by a thorough physical examination, Among other things, the doctor will listen to the lungs and heart, measure the blood pressure and scan the liver, spleen and lymph nodes. The results help the doctor to better assess the general condition of the patient.

blood test

Important for suspected leukemia or a related disease is the blood test. It will be one small blood picture and a Differential blood count created. The small blood count indicates, among other things, the number of white blood cells (total number), the red blood cells and the platelets. For the differential blood picture, the various subgroups of white blood cells are measured individually. In addition, under the microscope, the appearance of the blood cells can be assessed.

Pathologically altered blood counts, such as an increase in white blood cells and a lack of red blood cells, may be an important indicator of leukemia. However, they can also cause many other diseases.

Besides the blood cells are in the lab too other blood parameters such as kidney and liver values. These values ​​indicate how well these two organs work. If leukemia is confirmed in the further course and the kidney and / or liver values ​​of the patient are poor, this must be taken into account in the treatment planning.

The lab also checks if there are any signs of infection with bacteria, viruses or fungi in the blood. These germs could also be responsible for some ailments, such as increased levels of white blood cells, fever and fatigue.

Bone marrow

Any suspected leukemia requires careful examination of the patient’s bone marrow. For this, the doctor takes a bone marrow sample with local anesthesia with a special needle, usually from the pelvic bone (bone marrow puncture). The number and appearance of the bone marrow cells are examined in the laboratory. Typical changes can clearly detect leukemia. Sometimes even the form of the disease can be determined. In addition, the cells can be examined for changes in their genome (for example, the “Philadelphia chromosome” in chronic myeloid leukemia).

Adults and older children usually receive local anesthesia before bone marrow removal. For smaller children, a short anesthetic may be useful. The whole puncture usually takes only about 15 minutes and can be performed on an outpatient basis.

Further investigations

If the diagnosis of leukemia is confirmed, further investigations are often necessary. They are to show whether other body regions and organs are affected by the cancer cells. The general condition of the patient can also be better estimated with such examinations. This is important for therapy planning.

So you can, for example, internal organs (spleen, liver, etc.) by means of Ultrasonic investigate. Eventually will be a Computed tomography (CT) made. This imaging technique is also useful for assessing the bones. This is important if the doctor suspects that the cancer cells have spread not only in the bone marrow but also in the bone itself. Sometimes it will become one Magnetic Resonance Imaging (MRI) or one scintigraphy carried out.

In acute lymphoid leukemia (ALL), as well as some subtypes of acute myeloid leukemia (AML), cancer cells sometimes infiltrate the brain or meninges. Possible signs include headaches and nerve failures such as blurred vision and paralysis. Then one can Sample of cerebrospinal fluid taken (lumbar puncture) and analyzed in the laboratory. An MRI can also be helpful in detecting a cancerous attack of the brain.

Leukemia: disease course and prognosis

Many people with leukemia today have a much better chance of survival than many years or decades ago. Modern therapies can often improve the chances of recovery. If the cancer is already too advanced, however, treatment can at least alleviate the symptoms of many patients and prolong their survival.

In individual cases, the prognosis for leukemia depends on various factors. First, the type of cancer and the stage of the disease at the time of diagnosis. Another factor influencing the prognosis is how well the patient responds to the therapy. Other factors that influence life expectancy and chances of recovery in leukemia are the patient’s age and general condition as well as possible comorbidities.

Leukemia: Healing chances

“Is leukemia curable?” Many patients and their families ask themselves this question. In principle, that applies to acute leukemia Healing is possible. The earlier the disease is discovered and treated, the greater the chances of recovery. This is especially true for younger patients:

Without treatment, most patients only survive the diagnosis of acute leukemia for about three months. With treatment, 95 percent of children and 70 percent of adults still live with acute lymphoblastic leukemia (ALL) five years after diagnosis. In acute myeloid leukemia (AML), the 5-year survival rate is 40 to 50 percent in patients under 60 years and 20 percent in the 60+ age group.

Even if the cancer can be pushed back, it can later come to a relapse (recurrence), even after months and years. Especially with an early relapse decrease the chances of recovery. Leukämie-Patienten müssen dann erneut behandelt werden. Manchmal wählen Ärzte dabei eine aggressivere Therapie oder andere Behandlungsmethoden.

Bei einerchronischen Leukämie vermehren sich die Krebszellen langsamer als bei akuten Krebsformen (Ausnahme: Blastenkrise bei CML) – und das meist jahrelang. Deshalb ist die Behandlung in der Regel weniger intensiv, muss dafür aber langfristig fortgesetzt werden. Heilen lässt sich eine chronische Leukämie im Allgemeinen zwar nicht (diese Chance besteht höchstens bei der riskanten Stammzelltransplantation). Bei vielen Patienten kann die Therapie aber die Symptome mildern und das Fortschreiten der chronischen Leukämie bremsen.

Weiterführende Informationen

Buchempfehlungen

Chronische Leukämien: Rat und Hilfe für Betroffene und Angehörige (Rat & Hilfe) (Hermann Delbrück, Kohlhammer, 2008)

Leitlinien

• S3-Leitlinie “Diagnostik, Therapie und Nachsorge für Patienten mit einer chronischen lymphatischen Leukämie” der Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e.V. (AWMF), Deutschen Krebsgesellschaft e.V. und Deutschen Krebshilfe (2017)
• Leitlinie “Akute lymphoblastische Leukämie – ALL – im Kindesalter” der Gesellschaft für Pädiatrische Onkologie und Hämatologie (2016)
• Leitlinie “Chronische Myeloische Leukämie” der Fachgesellschaft zur Diagnostik und Therapie hämatologischer und onkologischer Erkrankungen (2013)
• Leitlinie “Akute Myeloische Leukämie” der Fachgesellschaft zur Diagnostik und Therapie hämatologischer und onkologischer Erkrankungen (2018)

Selbsthilfegruppen

MDS-NET Deutschland e.V. (www.mds-net-de.org)

Leukämie-Lymphom-Hilfe S.E.L.P. e.V. (www.selp.de)

Leukämie-Phoenix (www.leukaemie-phoenix.de)