Lymphatic leukemia

and Martina Feichter, medical editor and biologist

Martina Feichter studied biology with an elective subject pharmacy in Innsbruck and also immersed herself in the world of medicinal plants. From there it was not far to other medical topics that still captivate her to this day. She trained as a journalist at the Axel Springer Academy in Hamburg and has been working for since 2007 - first as an editor and since 2012 as a freelance writer.

More about the experts All content is checked by medical journalists.

The term "lymphocytic leukemia" encompasses two forms of cancer in which certain blood cells multiply uncontrollably: acute lymphoblastic leukemia (ALL) and chronic lymphocytic leukemia (CLL). ALL is the most common leukemia disease in childhood, while CLL is almost exclusively found in older people. Read more about lymphocytic leukemia!

ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. C91

This is how lymphatic leukemia develops

"Lymphatic leukemia" is what doctors call cancers that originate from the so-called lymphatic precursor cells of blood formation:

All blood cells (red and white blood cells and platelets) have a common origin - the blood stem cells in the bone marrow. Two types of progenitor cells develop from these stem cells: lymphatic and myeloid progenitor cells. The lymphocytes develop from the former in several steps. This subgroup of white blood cells (leukocytes) is an important part of the immune system. All other white and red blood cells and platelets arise from the myeloid progenitor cells.

In lymphocytic leukemia, the formation of lymphocytes is disturbed: large numbers of immature lymphocytes are created that multiply in an uncontrolled manner. As a result, they increasingly push back the mature, healthy blood cells. That means: There is less and less of the other subgroups of the white blood cells. There is also a shortage of red blood cells and platelets.

Depending on the course of the disease, doctors differentiate:

  • Acute lymphoblastic leukemia (ALL) starts quite suddenly and progresses rapidly.
  • Chronic lymphocytic leukemia (CLL), on the other hand, develops insidiously and slowly.

Acute lymphocytic leukemia (ALL)

Acute lymphoblastic leukemia is the most common type of leukemia in children. About 80 percent of all children with leukemia have ALL. Sprouts under the age of five are most often affected. ALL is rather rare in adults. The disease occurs here more often in people over 80 years of age.

Overall, acute lymphoblastic leukemia is very rare: in Germany, around 1 in 100,000 people develop this form of blood cancer. People with trisomy 21 (Down syndrome), among others, have an increased risk of the disease.

Symptoms of ALL

Acute lymphoblastic leukemia manifests itself with various symptoms that usually develop within days: The increasing shortage of red blood cells causes anemia. The patients therefore have noticeably pale skin and mucous membranes and a rapid heartbeat. They are also less efficient and tire quickly. Sometimes there is also shortness of breath and dizziness.

Because the cancer cells also displace the blood platelets, bleeding (such as bleeding from the gums and nose) develops. Patients also get bruises (bruises) easily. Often one also observes punctiform hemorrhages in the skin and mucous membranes. Doctors refer to them as petechiae.

Other common symptoms of ALL are fever, poor appetite and a tendency to become infected. Many patients also have enlarged lymph nodes and an enlarged spleen (splenomegaly).

If the cancer cells have attacked the central nervous system (brain and spinal cord), it can lead to headaches, vomiting, indifference and nerve failure as well as paralysis.

Diagnosis of ALL

All of the symptoms mentioned also occur in many other diseases. So they are not a clear sign of acute lymphoblastic leukemia. In any case, you should see a doctor as soon as possible if you experience such symptoms. He will first collect the medical history by having the symptoms described in detail. For example, he asks about previous or underlying illnesses and any cases of cancer in the family.

This is followed by a physical exam. It is intended to provide information about the general condition of the patient.

Blood tests and a bone marrow puncture are especially important if acute lymphoblastic leukemia (or any other form of leukemia) is suspected. In the latter case, the doctor takes a sample of the bone marrow and has it examined in detail in the laboratory. With this, an ALL can be reliably proven. In addition, it can be determined which subtype of acute lymphatic leukemia the patient suffers from (such as Common ALL, Pro-B ALL, Cortical T ALL etc.). These sub-forms differ in terms of course and prognosis. They also respond differently to different therapies.

In addition, there are usually further examinations, for example EKG, imaging procedures (such as X-rays, ultrasound) and an examination of the nerve water (lumbar puncture). They are used either to better assess the patient's physical condition or to check the spread of cancer cells in the body. You can read more about this under Leukemia: Diagnosis and examinations.

Therapy of ALL

People with acute leukemia (like ALL) need treatment as soon as possible. The aim is to ensure that the disease regresses completely (remission).

How the exact therapy planning looks in each individual case depends on the age of the patient and the exact subtype of ALL. As a rule, patients receive intensive chemotherapy in several cycles. It should eliminate all cancer cells in the body as possible. This is usually followed by further chemotherapies, but these are less intensive. They serve to fight any remaining cancer cells and to avoid a relapse.

Other treatment approaches for acute lymphoblastic leukemia are stem cell transplantation and radiation therapy. In stem cell transplantation, blood stem cells are transferred to the patient. This should produce new, healthy blood cells. Radiation therapy in ALL is primarily used to prevent or treat cancer of the brain.

In some patients, the cancer cells show a certain genetic change (Philadelphia chromosome). It is from this chromosome that the body produces an abnormal variant of the enzyme tyrosine kinase. This type of enzyme stimulates the growth of leukemia cells. The patients therefore sometimes receive so-called tyrosine kinase inhibitors (such as imatinib). They inhibit the enzyme in question.

Read more about the therapy options for blood cancer under Leukemia: Treatment.

Prognosis of ALL

In the last few decades, the number of patients with ALL who could be cured has increased, and the chances of recovery are usually good, especially in children. Five years after diagnosis, around 70 percent of adult patients and 95 percent of affected children are still alive if properly treated. After ten years, survival rates are around 33 percent in adults and 70 percent in children.

In individual cases, however, the prognosis of acute lymphoblastic leukemia depends on many different factors. These include the exact form of ALL, the stage of the disease at the time of diagnosis, and the age and general condition of the patient.

Chronic lymphocytic leukemia (CLL)

Chronic lymphocytic leukemia is a slowly progressing leukemia disease; it usually develops over many years and can persist for a long time without causing noticeable symptoms.

Caution: Despite its name, chronic lymphocytic leukemia is no longer counted as a leukemia ("blood cancer"), but is a form of lymph gland cancer (more precisely: non-Hodgkin lymphoma).

Chronic lymphocytic leukemia is the most common form of leukemia in western industrialized countries. In Germany, around 3,000 men and 2,000 women get it every year. These are mostly older patients: the mean age of onset is between 70 and 75 years. Chronic leukemia like CLL is very rare in children.

Symptoms and Diagnosis

Chronic lymphocytic leukemia usually causes no symptoms for a long time. Sometimes unspecific complaints such as decreased performance, tiredness and loss of appetite occur. As the disease progresses, painlessly swollen lymph nodes and an enlarged liver and spleen develop. Some people develop a fever, sweat at night, and are prone to infections and blue spots (bruises). There are also signs of anemia (paleness of the skin and mucous membranes, easy fatigue, dizziness, etc.).

Read more about the signs and the course of chronic leukemia under Leukemia: Symptoms.

The doctor often discovers chronic lymphocytic leukemia by chance, for example because the number of white blood cells is noticeably high. In addition to precise blood tests, taking a medical history (anamnesis) and a physical examination are the main diagnostic steps if CLL is suspected.

In some cases it is necessary to take a tissue sample (biopsy) from the lymph nodes and analyze it in the laboratory. In this way it can be determined whether and how far the disease has spread. For the same reason, an ultrasound scan of the abdomen can be performed, for example. In some cases, a bone marrow examination is also useful.

You can read more about the different examinations under Leukemia: examinations and diagnosis.

Therapy of CLL

Many people with CLL do not feel sick and have no symptoms for years because the disease is slow to progress. Then usually no therapy is necessary: ​​Instead, the doctors wait and only carry out regular check-ups ("watch and wait").

If the blood values ​​worsen or the cancer causes symptoms, therapy is initiated. What this looks like depends on the general condition of the patient, their kidney function and any previous illnesses.

So-called chemoimmunotherapy (or immunochemotherapy) is then often initiated. That is, the patients receive chemotherapy in combination with immunotherapy:

The cancer drugs (cytostatics) used in chemotherapy are given as tablets or infusions. In addition, patients receive so-called monoclonal antibodies as part of the immunotherapy. These are special, artificially produced antibodies that specifically recognize the patient's cancer cells and can bind to them. Then the immune system can take action specifically against the malignant cells.

In some patients, chronic lymphocytic leukemia is treated with chemotherapy or immunotherapy alone. Additional radiation therapy or surgery is rarely necessary. This can be the case, for example, when lymph nodes are infected by cancer cells and cause complications.

In some CLL patients, the cancer cells develop certain genetic changes over time (see above: Therapy for ALL). Those affected no longer respond as well to standard chemotherapy and immunotherapy. Then treatment with so-called tyrosine kinase inhibitors (such as ibrutinib) may be more effective.

If the first cancer treatment is unsuccessful or the cancer regresses, a stem cell transplant may be an option in some cases. First, high-dose chemotherapy kills the entire bone marrow and (hopefully) all cancer cells. Then blood stem cells from a donor are transferred to the patient, from which new, healthy blood cells emerge. Stem cell transplantation is a very stressful and risky treatment. It is therefore only suitable for young or fit patients. And with them, too, the benefits and risks of the treatment must be carefully weighed against each other in advance.

Prognosis of CLL

Chronic lymphocytic leukemia (CLL) is the "most benign" form of leukemia. The disease usually progresses slowly and often causes no or hardly any symptoms for years. If treatment is necessary, it can usually suppress the CLL and slow its progression. According to the current state of knowledge, only risky stem cell transplants offer a chance of a cure.

How long patients with chronic lymphocytic leukemia survive depends to a large extent on the stage of the disease and the genetic makeup of the cancer cells (shape and structure of the chromosomes, possible genetic changes, etc.). Any previous and accompanying illnesses as well as the general condition of the patient also influence the prognosis.

It is particularly dangerous for patients that their weakened immune system makes them susceptible to infections. Uncontrollable infections are therefore also the most common cause of death in people with chronic lymphocytic leukemia (or another form of leukemia).

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