Hairy cell leukemia is a very slowly progressing malignant disease of B lymphocytes. It is one of the so-called non-Hodgkin lymphomas. As a rule, this form of leukemia can be treated very well with the use of chemotherapeutic agents.
What is hairy cell leukemia?
In hairy cell leukemia there are degenerated B lymphocytes whose uninhibited proliferation leads to the gradual destruction of the bone marrow. Normal bone marrow stem cells are displaced. As a result, fewer blood-forming cells, which are made up of erythrocytes, leukocytes and thrombocytes, can be formed overall. Anemia and a weakening of the immune system occur.
Overall, hairy cell leukemia is very rare, with an average frequency of about three per million people. Men get sick four to five times more often than women. The disease usually occurs in middle age. However, with the exception of children, any age can be affected. Hairy cell leukemia is caused by genetically modified B lymphocytes.
The B lymphocytes are a special form of leukocytes. They are responsible for the formation of antibodies as part of immune reactions. The malignantly altered B lymphocytes form fringe-like plasma extensions that appear like hair. Due to this fact, the affected cells are called hair cells. Because the hair cells multiply uncontrollably, it is referred to as hairy cell leukemia.
Hairy cell leukemia is one of the so-called non-Hodgkin lymphomas. This is a heterogeneous group of diseases characterized by the proliferation of lymphocytes. In contrast to Hodgkin’s disease, these lymphomas do not form multinucleated Sternberg-Reed cells, which are characterized by the accumulation of several Hodgkin cells (degenerate lymphocytes).
Hairy cell leukemia is a subgroup of B-cell non-Hodgkin lymphomas. Overall, hairy cell leukemia is a very slowly progressing disease that can be very well suppressed by treatment with chemotherapeutic agents. With therapy, a normal life expectancy is achieved.
The cause of hairy cell leukemia is largely unknown. It is suspected, among other things, that insecticides or herbicides could play a role. The influence of glyphosate, which is used to control weeds, is also discussed.
In any case, mutations in the BRAF gene on chromosome 7 were detected. However, these are not congenital but acquired somatic mutations that can arise as part of the increased cell division rate of the B lymphocytes and exposure to mutagenic substances. However, the corresponding mutations can also occur spontaneously.
Symptoms, Ailments & Signs
Classic hairy cell leukemia is characterized by a reduction in the number of all blood-forming cells (pancytopenia) as a result of the slow destruction of the bone marrow. This becomes noticeable through low concentrations of erythrocytes, leukocytes and thrombocytes. The lack of erythrocytes (red blood cells) leads to anemia with fatigue, pallor and general weakness.
The low concentration of leukocytes (white blood cells) is also called leukopenia. It causes an increased susceptibility to infections because the white blood cells are the actual immune cells. Finally, the lack of thrombocytes ( thrombopenia ) leads to an increased tendency to bleed. In only 10 to 20 percent of those affected, however, a strong increase in leukocytes is observed.
Further symptoms can be an enlargement of the spleen ( splenomegaly ) or an enlargement of the liver ( hepatomegaly ). In rarer cases, more severe symptoms such as vascular inflammation, bone changes and B symptoms with fever, night sweats and weight loss appear. However, there are also asymptomatic forms at the beginning of the disease process.
Blood tests are the best way to diagnose hairy cell leukemia. The typical hair cells are usually detected. Furthermore, concentrations of erythrocytes, leukocytes and thrombocytes are usually found to be too low.
In a few patients, however, the leukocyte count is elevated. There is also an increase in immature and differently sized erythrocytes. Normally, all erythrocytes are about the same size.
Hairy cell leukemia causes the patient to be extremely tired and generally weak. In most cases, the patient’s resilience also decreases, so that certain daily activities and sports activities can no longer be carried out easily. As a result, the quality of life decreases extremely and it also leads to psychological complaints and upsets.
The affected person is also susceptible to various inflammations and infections due to hairy cell leukemia and thus becomes ill more often. The tendency to bleed is also greatly increased, so that the affected person suffers from severe bleeding even with minor injuries. In some cases, bleeding can occur spontaneously and for no particular reason.
Everyday life is also made more difficult for the patient by hairy cell leukemia. Weight loss and high fever continue. Sweating breaks out at night and, not infrequently, shortness of breath. In the worst case, the patient can die from the symptoms of hairy cell leukemia if this disease is not treated.
Hairy cell leukemia is treated with chemotherapy. In most cases, the course of the disease is positive if the cancer is diagnosed at an early stage. Even after the treatment, the patient has to undergo various examinations.
When should you go to the doctor?
Since hairy cell leukemia can lead to serious and even life-threatening symptoms, this disease should always be examined and treated by a doctor. As a rule, the doctor should be consulted if the person concerned suffers from permanent tiredness and exhaustion.
This complaint cannot be offset by adequate sleep. General weakness also occurs, so that the resilience of the person affected by the hairy cell leukemia decreases significantly. A doctor should also be consulted if there is an increased tendency to bleed.
Even with simple and small cuts, those affected suffer from heavy bleeding that does not stop easily. An increased susceptibility to infections can also indicate hairy cell leukemia. Inflammation and infections are more common. Night sweats or severe weight loss also indicate hairy cell leukemia and should also be examined. As a rule, the diagnosis can be made by a general practitioner or a pediatrician. Further treatment is then carried out by a specialist.
Treatment & Therapy
Hairy cell leukemia can be treated very well with chemotherapy. In more than 90 percent of cases, there is a complete or at least partial remission of the diseased cells. So-called cytostatics based on purine analogues are used as chemotherapeutic agents. Cytostatics prevent further cell proliferation. The purine analogues are incorporated into the DNA of the cell nucleus instead of purines. They thus prevent further cell division.
Before therapy with purine analogues, treatment with interferons is carried out to strengthen the immune system. Overall, this therapy can completely destroy the diseased cells and defeat the cancer. However, the formation of recurrences is possible if not all cancer cells have been killed. Due to the slow growth, these recurrences can occur even after several years.
But the recurrences can also be easily controlled again with the help of chemotherapy. In the classic therapy, the patient used to receive a seven-day, 14-hour continuous infusion. Today the treatment lasts five days with a daily 2-hour infusion. As previously mentioned, over 90 percent of patients respond to this treatment. In more than 80 percent of those affected, there are no signs of a recurrence even after more than five years.
In the special variant of hairy cell leukemia with an increase in the number of leukocytes, monoclonal antibodies are also administered in addition to treatment with interferons and purine analogues. Here, too, the treatment is usually successful. In the past, the spleen was often removed with very good remission results. Due to the excellent prognosis with chemotherapy, a splenectomy is usually no longer performed today.
Outlook & Forecast
The prospects after the diagnosis of hairy cell leukemia can be rated as good. Several long-term studies have been conducted by scientists. According to this, 70 percent of all patients can continue their lives normally after treatment. The key to a positive outcome is that people respond to treatment.
The typical signs of hairy cell leukemia must subside permanently and not just partially. The hairy cell leukemia variant (HZL-V) must be distinguished from this. She doesn’t respond to many therapies. Statistically speaking, patients suffering from it must be assumed to have a short lifespan.
After an initial illness and the symptoms have completely subsided, it is important to diagnose recurrences as quickly as possible. Since hairy cell leukemia is chronic, it can break out again and again. There is no usable data on which periods are associated with excessive risk. Rather, the disease never returns once the symptoms have gone away for many of those affected.
Blood and ultrasound tests must be carried out regularly. In the beginning, body samples should be examined every four weeks, later at least every six months. A tight network of preventive care promotes complete reintegration into everyday life and minimizes the risk of illness recurring.
There are currently no recommendations for preventive measures against hairy cell leukemia.
In most cases, patients with hairy cell leukemia do not have any special aftercare options because the disease cannot be completely treated. Lifelong therapy can take place, although the patient’s life expectancy is greatly reduced by this disease. In general, an early diagnosis of hairy cell leukemia always has a positive effect on the further course of the disease, so that the focus of this disease is on early detection.
The treatment itself is usually carried out with the help of various medications. The patient should always pay attention to the correct dosage and regular intake. Regular and ongoing check-ups by a doctor are also very important in order to properly control the symptoms of the disease.
Even after the symptoms have subsided, regular examinations by a doctor should still be carried out. Those affected need the help and loving care and support of family and close friends in the fight against cancer caused by hairy cell leukemia. It can also relieve depression or other mental upsets. Contact with other patients often has a positive effect on the course of hairy cell leukemia.
You can do that yourself
A diagnosed hairy cell leukemia is not a disease for self-treatment. The therapy should be accompanied by an oncologist. The first choice in this case is usually chemotherapy. Since this is not without side effects, those affected can take a number of measures to alleviate the accompanying symptoms and improve the chances of recovery.
Basically, the immune system should be strengthened, since the body is susceptible to infectious diseases during cancer. A well-functioning immune system requires a balanced diet rich in vital substances with lots of fresh fruit and vegetables and sufficient liquid intake in the form of still mineral water or unsweetened herbal teas.
They also balance the acid-base balance. Existing hyperacidity – as most people in the western world have – would promote the inflammatory processes in the body. In addition, exercise – preferably in the fresh air – stimulates the metabolism and the lymph flow. This makes it easier for the body to detoxify and reduces stress.
Stress reduction should also be considered: small breaks and mindfulness exercises can be easily integrated into the daily routine. Sufficient sleep also has a stabilizing effect on the immune system.
In order to continue to support the body, a time-limited micronutrient therapy in the form of dietary supplements can also be helpful. These provide the body with important minerals and increase the immune system. Treatment with acupuncture or acupressure can help against symptoms such as nausea and vomiting.