Hairy cell leukemia

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

[1], [2], [3], [4], [5], [6],

Causes of hairy cell leukemia

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

In the case of the onset 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 a multiple increase, the cell under investigation appears as a formless structure with cytoplasmic outgrowths that resemble cytoplasm, resembling hairs.

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

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

As the observations showed, other factors can become a catalyst of this process:

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

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

[7], [8]

Symptoms of hairy cell leukemia

Perhaps one of the main distinguishing features of the pathology under consideration is the fact that splenomegaly develops against its background, which is expressed by the slow growth of spleen volumes. This is facilitated by a high level of disruption of pathological leukocytes.

Based on the mechanics of the development of the pathological process, we can identify such symptoms of hairy cell leukemia:

• The development of pathology may respond with the emergence of traumatic pain or severity, which is determined from the left side of the peritoneum.
• When palpation of the left side, the treating doctor senses the enlarged size of the spleen.
• There is anemia.
• The respite.
• The patient's body is weakened and prone to infection. Such a symptom can be explained by the oppression of hemopoiesis, which is suppressed by leukemia cells localized in the bone marrow.
• Symptomatic manifestations of complete intoxication of the body.
• A person begins to lose weight.
• His appetite is gone.
• There may be signs of fever.
• At night, there is an increased production of sweat.
• Man ascertains a spontaneous unexplained increase in body temperature.
• The patient begins to feel a constant weakness, rapid fatigue.
• On the body of the patient can be seen without cause numerous bruises and bleeding.
• Inflammation and soreness of lymph nodes in the armpit, in the 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 district therapist had even the slightest suspicion about an oncological disease, he gives the patient a referral to a more narrow-minded specialist for examination, which is carried out in a specialized oncological clinic.

Diagnosis of hairy cell leukemia is a series of standard measures:

• A primary examination of the patient is carried out to establish the general condition of the patient, palpation of the abdominal region, revealing the presence of inflamed lymph nodes in the patient.
• Acquaintance with his anamnesis and way of life.
• The first and one of the basic tests in the formulation of this diagnosis is a blood test, in which a lab technician can identify typical "hair cells". Another factor that is determined by this analysis and is its "visiting card" differentiating the disease 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 patient's anemia and a sharp decrease in the level of protection of the body.
• 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 withdrawn.
• A biopsy of the seized material is performed.
• The seized material is also being sent for histology.
• Physicians 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 an analysis makes it possible to visually detect the specific forms of a specific protein substance on the cell surface.
• Computerized axial tomography (CT), allows you to obtain a number of frames of the organ under investigation, produced at different angles. Medical images help to get X-ray equipment, which sends the image to a computer monitor. Immediately before the start of the study, the health worker introduces a contrast agent to the patient. This may be a pharmacological preparation in the form of a solution, capsule or tablet. The use of contrast medium allows obtaining a clearer picture of the investigated zone. Such a survey allows a specialist to recognize and see a change in the larger dimension of the size of the spleen and lymph nodes.

[9], [10], [11]

Laboratory diagnosis of hairy cell leukemia

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

Once in the oncology clinic, the patient needs to undergo a series of laboratory tests.

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

This analysis is taken by taking a material from the vein using a medical syringe. After the seizure of blood, she enters the laboratory, where, with the necessary equipment, and can calculate. This study is the basis of diagnosis and other diverse diseases.

• Study of the smear of peripheral blood. This procedure of analysis makes it possible to test the blood material for the presence of modified cells in it, counting the number of cells, and also "inventorying" the blood elements.
• To laboratory methods of research carry removal of a material for carrying out of a biopsy. The procedure for withdrawal is also called aspiration. This procedure is carried out using a long hollow needle. To obtain a sample of the bone marrow, it is injected into the bone of the pelvis or sternum. After that the received sample is transferred to the cytologist who, using a microscope, carries out the study itself. The purpose of the study is to find factors that indicate the presence of cancerous tumors in the body.

Treatment of hairy cell leukemia

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

• The quantitative ratio of healthy and mutated cells that are present in the bone marrow and blood.
• Dimensional parameters of the spleen and the level of their deviation from the norm.
• The presence in the anamnesis of a patient with leukemia, as well as the answer to a question, is its primary diagnosis or relapse.

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

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

But to date, a medicinal product has been found, which shows, when applied in medical therapy, quite good promising results. A new generation of cladribine, which makes it possible to achieve a long-term remission in eight cases out of ten. There is enough one therapeutic course for the patient to forget about the problem of the year by three.

A particularly persistent effect is obtained if this drug is prescribed to a patient already after receiving a course of interferon alfa or pentostatin.

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

Antitumor (antiblast) drug Cladribine is injected into the patient's body intravenously. The patient is given a dropper. The rate of drug intake should be low enough. This procedure is prescribed as a two-hour or 24-hour infusion.

Dosage and duration of the treatment course for each patient is appointed exclusively individually, after the analysis by the oncologist of the clinical picture of the disease, the state of the patient's body at the time of appointment.

Pharmacologists recommend a starting dose of the drug in the amount of 0.09 to 0.1 mg, taken per kilogram of the patient's weight. Data on the effectiveness of higher doses of cladribine injection are not available today. The average duration of the treatment course is seven days.

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

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

Contraindication to the use of this drug can be attributed to individual intolerance to the patient's body of one or more of its components, renal dysfunction (except for mild form), liver dysfunction (except for mild form). Cladribine should not also be administered together with myelosuppressive drugs, during pregnancy and lactation, as well as children and adolescents who are under 16 years of age. With great caution, enter if there is a secondary infection in the patient's body or when the bone marrow function is suppressed.

Acceptance of the drug in question gives encouraging results. Patients, about 95%, who have received treatment with cladribine, show a survival rate of about nine years.

Pentostatin. This drug is injected intravenously continuously for the intended length of time. The medication is prescribed in a dosage of 4 mg per patient body surface area, recalculated by m ². The procedure is carried out once every two weeks for three to six months. This drug allows not only to normalize the blood formula, but also lead to the disappearance of pathologically altered cellular structures localized in the bone marrow.

Introduced to the patient with a diagnosis of hairy cell leukemia and interferon. The antiviral drug interferon is intended to protect the weakened organism from invasion of the pathogenic pathogenic microflora.

This drug enters the patient through the nasal passages or orally. One procedure takes 10 ml of water for injection, slightly warmed to a temperature of 37 ° C and three ampoules of the drug (for inhalation). Like drops in the nose, the composition of one ampoule is diluted with 2 ml of warm water. In each nasal drill is instilled in 0.25 ml, which corresponds to five drops of solution.

The procedure is carried out with an interval of one to two hours, no less than five approaches per day. Duration of treatment two - three days.

Also, in the protocol of treatment of such a patient, drugs are usually introduced that are designed to normalize the hormonal background of a person and raise his immune status.

Prevention of hairy cell leukemia

As already noted above, modern medicine does not know 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 the conduct of a healthy lifestyle, minimizing stressful situations, avoiding bad habits, and excluding hypodynamia from one's life. That is, those milestones that can keep the immune status of a person at a sufficiently high level and significantly reduce the risk for the occurrence of any disease.

Prognosis of hairy cell leukemia

The outcome of the therapeutic treatment of the disease under consideration depends on many factors:

• This is the stage of the disease, at the time of treatment.
• The level of sensitivity of the modified cells and the patient's organism to the drugs administered.

The prognosis of hairy cell leukemia has a favorable outcome. This disease, as a rule, has a low rate of progression. Therefore, if it was diagnosed not at a very late stage of development, a person, with the support of his attending doctor, is able to live reasonably well and for a long time.

Approximately 95% of patients who received the diagnosis in question are fairly well residing for 10 years or more. Although it should be discounted that being a rare disease, doctors do not always have full information and the data given is based on an analysis of the clinical picture of a small number of people.

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

If the relapse occurs, after a short period of time, the treating physician prescribes another protocol of treatment, if the remission has lasted for a long time, then during the second offensive of relapse, the doctor prescribes to his patient a similar therapeutic course.

Survival in hairy cell leukemia

For any serious illness, doctors have a so-called criterion - survival of patients. He is especially relevant in such a field of medicine as oncology. As the latest statistics show, when getting a full-fledged treatment, the survival rate for hairy cell leukemia has a fairly high percentage. In about 10% of patients treated, physicians report 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 such data that the introduction of a new generation of cladribine in the protocol of treatment gives encouraging results. Patients, about 90 to 100%, who underwent cladribine treatment, show a complete remission, with approximately 97 of 100 living more than five years. The average survival rate of such people is about nine years.

There are data on the life expectancy of patients and if they refuse treatment. Since the diagnosis is established, this period varies from five to ten years. Such a spread in time 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 enough pathology (according to statistics, one patient for 150 thousand people a year), patients with this disease regularly appear in oncology clinics at a reception at the oncology - hematologist. At the same time, doctors note that this pathology has become more active over the past few years, the 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). Afflicts and that this disease is a little "younger". Cases of its detection in persons younger than 40 years have become more frequent. This is an occasion to reflect and take more care of your health, and even with a slight deviation from the norm, it will be superfluous to consult a qualified specialist.