Hairy cell leukemia

The diagnosis of cancer puts ordinary people into a stupor, a state of complete shock. With this diagnosis, the first thought is that life is over. But it is not that simple. There are many diseases related to the oncological field of medicine. Some of them, with timely medical intervention, give a person the opportunity to forget about the disease as a bad dream, while others, even with the intervention of qualified specialists, give the patient only a short period of life, a delay in death. One of the pathologies that will be discussed in more detail in this article is hairy cell leukemia - a serious disease, but fortunately quite rare.

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Causes of Hairy Cell Leukemia

The very name of the pathology "follows" from the appearance of the cellular structure, which is quite specific. In this picture of the disease, lymphocytes are subject to malignancy - cells of the lymphatic system, which are responsible for the immune status of a person.

In the case of leukemia, these structures begin to transform, changing their normal outline, and, accordingly, losing the ability to participate in immune processes. At the same time, under multiple magnification, the cell being studied appears as a shapeless structure with cytoplasmic processes extending from it, similar to hairs.

The causes of hairy cell leukemia have not been thoroughly studied by scientists working in the field of oncology to date. But as it has been established, the probability of its defeat and development largely depends on the person's gender and age. As clinical observations have shown, the statistical periods of the pathology in question correspond to approximately 50 years, while about 75% of patients diagnosed with hairy cell leukemia were representatives of the strong half of humanity. An explanation for this phenomenon has not yet been found.

The age period of diagnosis is determined not by late damage and the rate of progression, but by slow rates of development with a long asymptomatic period. That is, this disease can progress in the patient's body for many years, and he does not even suspect it.

Observations have shown that other factors can also act as catalysts for this process:

• Irradiation of the human body with a certain category of rays.
• Carcinogens, which are present in huge quantities in supermarket products today.
• Infectious diseases.

The above pathologies can presumably provoke the development of the disease in question, but reliable confirmation of this has not yet been obtained. Scientists continue to work in this direction.

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Symptoms of Hairy Cell Leukemia

Perhaps one of the main distinguishing indicators of the pathology in question is the fact that splenomegaly begins to develop against its background, which is expressed by a slow growth of the spleen volume. This is facilitated by a high level of decay of pathological leukocytes.

Based on the mechanics of the development of the pathological process, the following symptoms of hairy cell leukemia can be identified:

• The development of pathology may result in the occurrence of a nagging pain or heaviness, which is felt on the left side of the peritoneum.
• When palpating the left side, the attending physician feels an enlarged spleen.
• Anemia is observed.
• Shortness of breath.
• The patient's body is weakened and prone to infection. This symptom can be explained by the suppression of hematopoiesis, which is suppressed by leukemic cells localized in the bone marrow.
• Symptomatic manifestations of complete intoxication of the body.
• The person begins to lose weight.
• He loses his appetite.
• Signs of fever may appear.
• Increased sweat production occurs during night hours.
• A person notes a spontaneous, unexplained increase in body temperature.
• The patient begins to feel constant weakness and rapid fatigue.
• The patient's body may experience numerous bruises and bleeding for no apparent reason.
• Inflammation and soreness of the lymph nodes in the armpit, neck, groin and abdomen.

The whole difficulty in detecting this disease is that the symptoms begin to appear gradually and not immediately, so their appearance is often attributed to overwork after work, emotional exhaustion, age-related changes in the body.

Diagnosis of hairy cell leukemia

If the local therapist has even the slightest suspicion about an oncological disease, he gives the patient a referral to a more specialized specialist for examination, which is carried out in a specialized oncology clinic.

Diagnosis of hairy cell leukemia involves a series of standard procedures:

• The initial examination of the patient is carried out to establish the general condition of the patient, palpation of the abdominal area, and identification of the presence of inflamed lymph nodes in the patient.
• Getting to know his medical history and lifestyle.
• The first and one of the basic tests when making this diagnosis is a blood test, in which a medical laboratory technician can identify typical "hair cells". Another factor that is determined by this analysis and is its "calling card" differentiating the disease in question from other leukemias is pancytopenia, a pathological disorder in the patient's body, which is based on a decrease in the number of blood cells. It is pancytopenia that provokes the development of anemia in the patient and a sharp decrease in the level of protection of the body itself.
• In order to confirm the diagnosis and obtain a more complete clinical picture of the disease, a bone marrow puncture is also taken, or a small piece of the spleen is removed.
• A biopsy of the removed material is performed.
• The removed material is also sent for histology.
• Doctors also use immunophenotyping. This study is an analysis of a sample obtained from a patient (bone marrow or blood) using a high-resolution magnifying drug. Such analysis makes it possible to visually detect specific forms of a certain protein substance on the surface of cells.
• Computer axial tomography (CT) allows you to get a series of frames of the organ being examined, made from different angles. Medical images are obtained using X-ray equipment, which sends the image to a computer monitor. Immediately before the start of the examination, the health worker injects the patient with a contrast agent. This can be a pharmacological drug in the form of a solution, capsule or tablet. The use of a contrast agent allows you to get a clearer picture of the area being examined. Such an examination allows the specialist to recognize and see a change in the size of the spleen and lymph nodes.

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Laboratory diagnostics of hairy cell leukemia

As mentioned earlier, a very informative method for determining the disease is laboratory diagnostics of hairy cell leukemia.

Once admitted to the oncology clinic, the patient must undergo a series of laboratory tests.

• A general blood test allows the treating physician - oncologist to obtain the following indicators:
  • Numerical components of platelets, red and white blood cells.
  • This analysis allows you to obtain the level of hemoglobin, which is a direct indicator of anemia and other symptoms.
  • Erythrocyte sedimentation rate (ESR).

This analysis is taken by taking material from a vein using a medical syringe. After the blood is taken, it is sent to the laboratory, where, using the necessary equipment, it is possible to make a count. This study is the basis for diagnosing other diverse diseases.

• Peripheral blood smear examination. This analysis procedure allows testing blood material for the presence of modified cells, counting their number, and also conducting an "inventory" of formed elements of the blood.
• Laboratory research methods also include the removal of material for biopsy. The removal procedure is also called aspiration. This procedure is performed using a long hollow needle. To obtain a sample of bone marrow, it is inserted into the pelvic or sternum bone. After that, the obtained sample is transferred to a cytologist, who, using a microscope, conducts the research itself. The purpose of the research is to detect factors indicating the presence of cancerous neoplasms in the patient's body.

Treatment of hairy cell leukemia

One of the main methods included in the antitumor therapy protocol are chemotherapy drugs. Treatment of hairy cell leukemia is based on a number of factors that determine the chosen therapeutic method:

• The quantitative ratio of healthy and abnormal cells present in the bone marrow and blood.
• Size parameters of the spleen and the level of their deviation from the norm.
• The presence of leukemia in the patient's medical history, and also the answer to the question of whether this is a primary diagnosis or a relapse.

Not long ago, and in many cases now, oncologists use splenectomy (surgical intervention to remove the spleen) when treating cytopenia against the background of the disease in question. This procedure allows you to normalize the cellular formula of the blood. But as therapeutic observations have shown, constantly conducted with patients who have undergone this procedure, its effectiveness is observed for eight months.

It is advisable that treatment be started when one or two pathological symptoms appear, that is, when the disease has not yet acquired more extensive and severe consequences.

But today a medicine has been found that shows quite good encouraging results when used in therapeutic therapy. The new generation drug cladribine allows achieving long-term remission in eight cases out of ten. Sometimes one therapeutic course is enough for the patient to forget about the problem for three years.

A particularly lasting effect is achieved if this drug is prescribed to the patient after a course of interferon alpha or pentostatin has been completed.

The question of whether it is possible to finally get rid of hairy cell leukemia remains open to this day. Samples taken and examination of "former" patients showed that even after a long time, the patient has "traces" of residual disease.

The antitumor (antinectoplasmic) drug cladribine is administered intravenously to the patient. The patient is given a drip. The rate at which the drug enters the body should be low enough. This procedure is prescribed as a two-hour or 24-hour infusion.

The dosage and duration of the treatment course for each patient is prescribed strictly individually, after the oncologist has analyzed the clinical picture of the disease and the patient’s condition at the time of prescription.

Pharmacologists recommend a starting dose of the drug in the amount of 0.09 to 0.1 mg per kilogram of the patient's weight. There is currently no data on the effectiveness of higher doses of cladribine. The average duration of the treatment course is seven days.

Immediately before the procedure, the drug is diluted with a 9% sodium chloride solution, which is taken in volumes from half to one liter.

If the doctor has prescribed a 24-hour administration of the drug, a 0.9% bacteriostatic sodium chloride solution is usually used to dilute it, which contains benzyl alcohol, which serves as a preservative for the administered solution. In this case, this chemical compound is necessary so that the drug does not lose its pharmacological value throughout the infusion.

Contraindications to the use of this drug include individual intolerance by the patient's body to one or more of its components, kidney dysfunction (except for mild forms), liver dysfunction (except for mild forms). Also, cladribine should not be prescribed together with myelosuppressive drugs, during pregnancy and breastfeeding, as well as to children and adolescents who are under 16 years of age. With great caution, administer if the patient has a secondary infection or if bone marrow function is suppressed.

The drug under consideration gives encouraging results. Patients, about 95%, who have undergone a course of treatment with cladribine show a survival rate of about nine years.

Pentostatin. This medication is administered by intravenous injection continuously for a specified period of time. The medication is prescribed in a dosage of 4 mg per patient's body surface area, recalculated per m2 ^. The procedure is performed once every two weeks for three to six months. This medication not only normalizes the blood formula, but also causes the disappearance of pathologically altered cellular structures localized in the bone marrow.

Interferon is administered to a patient diagnosed with hairy cell leukemia. The antiviral drug interferon is designed to protect the body weakened by the disease from the invasion of pathogenic microflora.

This drug enters the patient's body through the nasal passages or orally. One procedure requires 10 ml of water for injection, slightly warmed to a temperature of 37 ° C and three ampoules of the drug (for inhalation). As nasal drops, the composition of one ampoule is diluted in 2 ml of warm water. 0.25 ml is instilled into each nasal passage, which corresponds to five drops of the solution.

The procedure is carried out at intervals of one to two hours, no less than five approaches per day. The duration of treatment is two to three days.

Also, the treatment protocol for such a patient usually includes medications that are designed to normalize a person’s hormonal background and raise his immune status.

Prevention of hairy cell leukemia

As noted above, modern medicine does not have knowledge of the causes and sources that can provoke the development of the disease in question. Therefore, the prevention of hairy cell leukemia is based only on maintaining a healthy lifestyle, minimizing stressful situations, giving up bad habits, eliminating physical inactivity from your life. That is, those milestones that can keep a person's immune status at a sufficiently high level and significantly reduce the risk of any disease.

Prognosis of hairy cell leukemia

The outcome of therapeutic treatment of the disease in question depends on many factors:

• This is also the stage of the disease at the start of treatment.
• The level of sensitivity of modified cells and the patient's body itself to the administered drugs.

The prognosis for hairy cell leukemia is generally favorable. This disease usually has a slow progression rate. Therefore, if it is diagnosed not at a very late stage of development, a person, with the support of his or her attending physician, is able to live a fairly high-quality life for a long period of time.

Approximately 95% of patients who receive the diagnosis under consideration live quite normally for 10 years or more. Although it should be allowed for the fact that being a rare disease, doctors do not always have full information and the data provided are based on the analysis of the clinical picture of a small number of people.

If the disease returns, the patient is prescribed repeated treatment, which gives another temporary remission. The result of repeated therapy is as follows: after a five-year period, the disease returns in 24-33% of patients, after a decade this percentage increases to 42-48%.

If a relapse occurs after a short period of time, the attending physician prescribes a different treatment protocol, but if remission lasted for a long time, then upon the second occurrence of a relapse, the doctor prescribes a similar therapeutic course for his patient.

Survival in hairy cell leukemia

In any serious disease, doctors have a so-called criterion - patient survival. It is especially relevant in such a field of medicine as oncology. As the latest statistics show, with full treatment, survival in hairy cell leukemia has a fairly high percentage. In about 10% of treated patients, doctors note complete remission, in 70% of cases, oncologists leading this patient observe partial remission, which significantly improved the course and prognosis of hairy cell leukemia.

There are also data that the introduction of the new generation drug cladribine into the treatment protocol gives encouraging results. Patients, about 90 to 100%, who have undergone a course of treatment with cladribine show complete remission, while approximately 97 out of 100 live more than five years. The average survival of such people is about nine years.

There are data on the life expectancy of patients even if they refuse treatment. From the moment of diagnosis, this period varies from five to ten years. Such a time range is due to various parameters: the patient's condition at the time of diagnosis, the degree of neglect of the disease, the rate of its progression and the clinical picture of pathological changes.

Despite the fact that hairy cell leukemia is a rare pathology (according to statistics, one patient per 150 thousand people per year), patients with this disease regularly appear in oncology clinics for an appointment with an oncologist-hematologist. At the same time, doctors note that this pathology has become more active over the past few years, cases of this diagnosis have become more frequent (according to rough estimates of specialists, the number of cases of detection of the disease has increased by a quarter). It is also disappointing that this disease has become somewhat "younger". Cases of its detection in people under 40 have become more frequent. This is a reason to think and pay more attention to your health, and even with a slight deviation from the norm, it would not be superfluous to undergo a consultation with a qualified specialist.