Hairy cell leukemia represents a very slowly progressing malignant disease of B lymphocytes. It belongs to the so-called non-Hodgkin’s lymphomas. Usually, this form of leukemia can be treated very well with the use of chemotherapeutic agents.
What is hairy cell leukemia?
In hairy cell leukemia, degenerate B lymphocytes are present, and their unchecked proliferation leads to gradual destruction of the bone marrow. Normal stem cells of the bone marrow are displaced in this process. As a result, fewer hematopoietic cells, which are composed of erythrocytes, leukocytes and platelets, can be formed. Anemia and weakening of the immune system result. Overall, hairy cell leukemia is very rare, with an average incidence of about three per million people. Men are four to five times more likely to develop the disease than women. The disease usually occurs in middle age. With the exception of children, however, any age can be affected. Hairy cell leukemia originates from genetically modified B lymphocytes. B lymphocytes are a special type of leukocyte. They are responsible for the formation of antibodies as part of immune reactions. The malignantly altered B lymphocytes form fringe-like plasma processes that appear like hairs. Due to this fact, the affected cells are called hair cells. Since the hair cells proliferate uncontrollably, it is referred to as hair cell leukemia. Hair cell leukemia belongs to the so-called non-Hodgkin’s lymphomas. This is a heterogeneous group of diseases characterized by the proliferation of lymphocytes. Unlike Hodgkin’s disease, these lymphomas do not form multinucleated Sternberg-Reed cells, which are characterized by the aggregation of several Hodgkin’s cells (degenerated lymphocytes). In the case of hairy cell leukemia, it is the subgroup of B-cell non-Hodgkin lymphomas. Overall, hairy cell leukemia is a very slowly progressing disease that can be very well repressed by treatment with chemotherapeutic agents. Normal life expectancy is achieved with therapy.
Causes
The cause of hairy cell leukemia is largely unknown. It is suspected that insecticides or herbicides may play a role, among other factors. The influence of glyphosate, which is used in weed control, is also being discussed. In any case, mutations in the BRAF gene on chromosome 7 have been detected. However, these are not congenital but acquired somatic mutations that may arise in the context of increased cell division rate of B lymphocytes and exposure to mutagenic substances. However, the corresponding mutations may also occur spontaneously.
Symptoms, complaints, and 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 is noticeable by low concentrations of erythrocytes, leukocytes, and platelets. 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 increased susceptibility to infection because white blood cells are the actual immune cells. Finally, the lack of platelets (thrombocytopenia) leads to an increased tendency to bleed. However, in only 10 to 20 percent of affected individuals, a marked increase in leukocytes is also observed. Other symptoms may include enlargement of the spleen (splenomegaly) and enlargement of the liver (hepatomegaly). In rarer cases, more severe symptoms such as vascular inflammation, bone changes, and B-symptomatology with fever, night sweats, and weight loss are seen. However, there are definitely asymptomatic courses at the beginning of the disease process.
Diagnosis
Blood tests are most suitable for diagnosing hairy cell leukemia. This usually detects the typical hairy cells. Furthermore, low concentrations of erythrocytes, leukocytes, and platelets are usually detected. In a few patients, however, leukocyte levels are elevated. Increased immature erythrocytes and erythrocytes of different sizes are also noticed. Normally, all erythrocytes have approximately the same size.
Complications
Hairy cell leukemia causes the patient to experience severe fatigue and general weakness. In most cases, the patient’s resilience also decreases, so that certain daily activities and sports activities can no longer be performed easily. As a result, the quality of life decreases extremely and psychological complaints and moods also occur. The affected person is also susceptible to various inflammations and infections due to hairy cell leukemia and thus becomes ill more often. The bleeding tendency is also greatly increased, so that the affected person suffers from severe bleeding even from minor injuries. In some cases, the bleeding can also occur spontaneously and without any particular reason. Everyday life is also made more difficult for the patient by hairy cell leukemia. Weight loss and severe fever continue to occur. At night there are sweats and not infrequently shortness of breath. In the worst case, the patient may die from the symptoms of hairy cell leukemia if no treatment of this disease occurs. Hairy cell leukemia is treated with the help of chemotherapy. In most cases, the course of the disease is positive if the cancer is diagnosed at an early stage. Even after treatment, the patient must undergo various examinations.
When should one go to the doctor?
Because 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 affected person suffers from permanent fatigue and lassitude. This complaint cannot be compensated by sufficient sleep. General weakness also occurs, so that the resilience of the affected person also decreases considerably due to hairy cell leukemia. A doctor should also be consulted if there is an increased tendency to bleed. Those affected suffer from severe bleeding even from simple and small cuts, and this does not stop easily. Likewise, an increased susceptibility to infections can also indicate hairy cell leukemia. In this case, inflammations and infections occur more frequently. Furthermore, a night sweat or a strong weight loss points to hair 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 and 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 the further proliferation of cells. The purine analogues are incorporated into the DNA of the cell nucleus instead of purines. They thus prevent further cell division. Prior to therapy with purine analogues, interferons are used to strengthen the immune system. Overall, this therapy can completely destroy the diseased cells and defeat the cancer. However, the development 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 are also well controllable again with the help of chemotherapy. In the classical therapy, the patient used to receive a seven-day 14-hour continuous infusion. Today, treatment lasts five days with a daily 2-hour infusion. As mentioned earlier, over 90 percent of patients respond to this treatment. At the same time, no signs of recurrence are found in over 80 percent of those affected, even after more than five years. In the special variant of hairy cell leukemia with an increase in the leukocyte count, monoclonal antibodies are also administered in addition to treatment with interferons and purine analogues. Here, too, treatment is usually successful. In the past, the spleen was often removed with very good remission results. Because of the excellent prognosis with chemotherapy, splenectomy is usually no longer performed today.
Outlook and prognosis
The outlook after the diagnosis of hairy cell leukemia can be considered good. Several long-term studies have been conducted by scientists. According to these, 70 percent of all sufferers can continue their lives normally after treatment.The decisive factor for a positive outcome is that individuals respond to the treatment. For this to happen, the typical signs of hairy cell leukemia must subside permanently and not only partially. Hairy cell leukemia variant (HZL-V) must be distinguished from this. It does not respond to many therapies. If patients suffer from it, it must be statistically assumed that they will live only a short time. After initial disease and complete resolution of symptoms, it is important to diagnose relapses as soon 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, after the initial resolution of symptoms, the disease never returns in many affected individuals. Blood and ultrasound examinations should be performed regularly. In the beginning, body samples should be examined every four weeks, and later at least every six months. A tight network of preventive care promotes full reintegration into daily life and minimizes the risk of recurrence.
Prevention
There are currently no recommendations for preventive measures against hairy cell leukemia.
Follow-up care
In most cases of hairy cell leukemia, patients do not have any particular options for follow-up care because the disease cannot be fully treated. Lifelong therapy may be given, although the patient’s life expectancy is still greatly reduced by this disease. In general, early diagnosis of hairy cell leukemia always has a positive effect on the further course of the disease, so that the focus in this disease is on early detection. The treatment itself is usually carried out with the help of various drugs. The patient should always pay attention to the correct dosage and also to regular intake. Likewise, regular and ongoing checks by a doctor are very important to properly control the symptoms of the disease. Even after the symptoms have subsided, regular examinations by a doctor should still be carried out. Sufferers need the help and loving care and support of family and close friends in their fight against cancer due to hairy cell leukemia. This can also alleviate depression or other psychological upsets. Often, contact with other patients has a positive effect on the course of hairy cell leukemia.
What you can do 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. However, since chemotherapy is not without side effects, patients can take a number of measures to alleviate the accompanying symptoms and improve their chances of recovery. In principle, 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 plenty of fresh fruit and vegetables as well as sufficient fluid intake in the form of still mineral waters or unsweetened herbal teas. These also balance the acid-base balance. An existing hyperacidity – as most people in the Western world have – would promote inflammatory processes in the body. In addition, exercise – preferably in fresh air – stimulates the metabolism and lymph flow. This allows the body to detoxify more easily and reduces stress. Stress reduction should also be taken into account: small breaks and mindfulness exercises can be incorporated into the daily routine very well. Likewise, sufficient sleep has a stabilizing effect on the immune system. To further support the body, a temporary micronutrient therapy in the form of dietary supplements can also be helpful. These provide the body with important minerals and increase the body’s defenses. Treatment with acupuncture or acupressure can help against symptoms such as nausea and vomiting.