Werlhof's disease

This disease does not spare anyone. It is diagnosed and in newborns, preschool and school-age children, adults are also immune from it. But as statistics show, this pathology is three times more likely to be sick than women, than the male half of humanity. So what is Werlhof's disease? Is it possible to effectively fight it? On all these and many other questions and try to find answers in this article.

Causes of Werlhof's Disease

Verlof's disease, or as it is also called thrombocytopenic purpura, is a pathological change in the human body, which is formed on the basis of the predisposition of platelets to aggregation (the process of gluing cells together, the initial stage of thrombus formation).

To date, the causes of Verlhof's disease have not been established for certain. If you unequivocally speak about the hereditary, genetic, character of the disease, then it is diagnosed much less often than the acquired form of defeat. The fermentopathic, immune or viral genesis of pathology was also considered, but this turn of the problem has not yet received any serious confirmation.

Symptoms of Verlhof's Disease

The lesion that develops in the human body leads to the clogging of the passage section of small capillaries. If this disease is suspected, the doctor may observe such symptoms of Verlhof's illness:

• Reduction in the blood of a patient's platelet count taken per unit volume of plasma. This result can be obtained through the active use of these components of blood in the formation of blood clots.
• Thrombocytopenic purpura is accompanied by ischemic disorders that affect almost all organs of the human body: the heart, gall bladder, liver, genitals, kidneys, brain and so on.
• This disease is classified as pathology, which quickly develops, affecting at the same time absolutely all age categories.
• The most common patients with thrombocytopenic purpura are patients aged 10 to 50 years.
• Pathological changes can occur "on an equal footing" (against the background of absolute health), or after having an infectious, catarrhal or intestinal pathology.
• People begin to get pain in the head.
• He feels fast fatigue.
• There is a general decrease in the tone of the body.
• The patient has a poor appetite.
• The symptom of Verlhof's illness may be nausea, in rare situations it is able to go even into a vomitive reflex.
• A fever of the wrong kind may appear.
• There is pain symptoms, localized in the chest and peritoneum.
• Almost the entire body of the patient is covered with a small amount of hemorrhages and bruises. Hemorrhages are spontaneous, manifested mainly at night.
• Visual impairment.

Gradually, with the development of the disease, the already existing symptoms of Verlhof's disease can be joined:

• Bleeding of different locations: from nasal passages, uterus, from the organs of the gastrointestinal tract, bleeding into the retina of the eye, bleeding from the gums. A little less often, but hemoptysis may join.
• Symptoms of a neuralgic nature may join:
  • Paralysis of one side of the body.
  • Ataxia is a violation of balance and coordination of voluntary movements.
  • Disorientation in space.
  • The retardation of conversation.
  • Convulsions.
  • Tremor
  • A mist appears before your eyes.
  • Sometimes acute mental disorders develop.
  • In a particularly difficult situation, the patient falls into a coma.
• But the main clinical picture of the disease, still are bleeding, originating from the mucous (especially in the case of small patients) or hemorrhages, manifested on the skin. They can arise as a consequence of bruises or injections, and for no reason whatsoever. Hemorrhages on the epidermis can have different sizes (from small points to large enough spots), but mostly they are localized from the front side of the human body, as well as the upper and lower extremities. The difference between blood manifestations in Verlhof's disease from classical vascular bleeding, which unlike the latter, these bruises can periodically merge with their neighbors. This factor is the actual evaluation in determining the diagnosis of thrombocytopenic purpura and evaluation of the nature of hemorrhagic diathesis.
• Depending on the time of hemorrhage, as in the case of vascular bruise, the shade of hemopoiesis can be either greenish-blue or dark-brown or yellow.
• In a woman of childbearing age, a hemorrhage can occur in the ovaries, which can simulate an ectopic pregnancy.
• Pathology occurs with frequent exacerbations.
• The incapacity of the patient is reduced.

Verlhof disease in children

As though adults did not try, but completely to protect the kid from impacts and abrasions it does not turn out. Not yet a firm gait, imperfect coordination combined with innate mobility can cause a baby to fall even on level ground. And as a consequence of the "flight" - a bruise on the delicate skin of the child. However, sometimes hemorrhages begin to appear on the body of the baby, it would seem, for no apparent reason. This really should alert the parents and become an excuse for going to the pediatrician for advice. After all, this symptomatology may be the first symptom that begins to manifest Werlhof's disease in children.

It is this disease that pediatricians endure in the first place in terms of the frequency of affection of the child's organism in the field of genetic pathologies associated with the hemopoietic system in children. The frequency of diagnosis in children of thrombocytopenic purpura is one to two cases per hundred thousand healthy children. Primary adherence to a certain sex in children does not reveal this disease.

The reasons for the appearance of this pathology in small patients are still unknown. Calling the root cause of the disease, doctors divided in opinion. Some believe that it has a genetic character. Development of pathology associated with the inferiority of the blood cell, the cause of which is a mutation. Others talk about the immune trace of a disease that develops due to a drop in the level of the protective forces of the child's body. Still others are willing to argue that the mechanism that triggers this disease is in the presence of both of these causes at the same time.

The first signs of the disease can manifest themselves after a short period of time after the drug therapy, severe hypothermia or overheating, vaccination, as a consequence of the transferred infectious diseases, which were caused by pathogenic flora (bacteria or viruses). Pathogenetic ways of progressing of this disease are several:

1. There is a confluence of certain factors that trigger the mechanism of synthesis of specific antibodies, which become antagonists for their own platelets. The process itself proceeds as the formation of an antibody-antigen tandem on the blood cell wall. This formation leads to cell death.
2. In parallel with this, there is a failure in the process of formation and development of platelets in the organs of hematopoiesis. That is, the newly formed antigen not only destroys the "adult" blood cell, but also introduces a change in the normal process of development of the megakaryocyte germ.
3. Such a violation of the process of hematopoiesis leads to the fact that platelets live less than they should. In a healthy body, this component of blood lives from ten to twelve days, whereas in the case of Verlhof's disease this interval can be reduced to one or two days.

As a consequence, the quantitative component of the platelets significantly decreases in the child's body, which invariably affects the functional insufficiency of the organism to normal work. Platelets are the food of the body. Reduction in the level of this component of blood provokes a decrease in the endothelial layer of the blood capillaries. This is the trigger for the emergence of sudden prolonged and profuse bleeding.

If such a patient is not provided with timely medical care, the result can be very lamentable - a fatal outcome.

There are cases when Verlhof's disease is first diagnosed to a woman during the period when she is carrying her baby. Predominantly, women with this diagnosis are quite normal to bear the child, and the birth takes place in the side of the norm. The blood loss in the process of obstetric care, with the diagnosis of thrombocytopenic purpura, shows volumes that are perfectly acceptable and do not constitute a threat to the life of the mother in childbirth. While an artificial termination of pregnancy can provoke much more bleeding. An exception is only the acute form of the course of the disease.

Proceeding from the above, it can be stated that the diagnosis of a pregnant Verlhof disease does not serve as a contraindication to the further bearing and birth of a baby. Indication for the artificial termination of pregnancy can serve as frequent and sufficiently abundant bleeding.

Diagnosis of Verlhof disease

To make the correct diagnosis, the doctor must have sufficient experience and qualifications. Diagnosis of Verlhof's disease includes:

• Analysis of the clinical picture of the disease.
• Detection of anamnesis of the patient.
• Inspection of a specialist patient. Symptom pinch. With thrombocytopenic purpura, with pinching, a bruise immediately forms under the skin.
• Endothelial tests are mandatory.
  • The cuff sample makes it possible to evaluate the character of the norm of the state of small capillaries (elasticity, elasticity and the level of fragility). To conduct this study, an ordinary cuff is used, which is used to measure blood pressure. Preliminary, the level of blood pressure is normal. Then, after a certain time, the cuff is applied again and the blood pressure is adjusted to values above the norm by 10-15 units of mm Hg. Art. With the disease under consideration, petechia begins to manifest itself almost immediately - many small hemorrhages form. Such a picture is a positive result of thrombocytopenic purple tests.
  • A hammer-like sign is similar to a pinch, but instead of a pinch a hammer is used.
  • A can and a sample test or a Waldman test. This technique allows to determine the presence of a hyperergic process in the body of a patient directly affecting the system of blood vessels and hematopoiesis (this is a turbulent course of the pathological process, which, even in time and adequate therapy, leads to the patient's death). The procedure is performed using a medical bank (hence the name of the study), which is put to the patient. Preferably, the jar is attached slightly below the collarbone. With the help of it, irritations of the epidural capillaries are achieved. It lasts about twenty minutes, from which a test of a blood smear is taken from the zone on which the bank was standing, and the level of monocytes is determined. Control is the number of these same structures, but determined before carrying out this stimulation. Such a study is done more than once, and periodically throughout the course of the disease. With pathogenicity of the ongoing process, the number of monocytes can increase several times.
• A general blood test is mandatory. At its carrying out it is possible to reveal a thrombocytopenia - the basic sign of illness of Verlhof. When conducting a laboratory study using magnifying equipment, you can perfectly consider the giant dimensions of platelets.
• Assigns and the study of blood for its coagulability. This analysis shows an extension of bleeding time. The pathology is judged by the degree of decrease in the volume of the plasma clot with a standard content of platelets (retraction of the blood clot).
• A bone marrow puncture is also performed. This study, with thrombocytopenic purpura, allows us to state a sharp increase in the megakaryoblast, promegakaryocyte and megakaryocyte levels in the patient's body. In the normal state, the platelets are loosened from the megakaryocyte nucleus, relieving them of the cytoplasm, which causes the structural formation of the megakaryocyte to be broken down into several components, after which they are disposed of by phagocytosis (the process of active capture and absorption of living organisms (bacteria, fungi, etc.)). And inanimate particles of unicellular organisms). In the case of a disease, the procedure for loosening platelets rapidly decreases its activity.
• When diagnosing Verlhof disease, a complete examination of the patient is performed, in which the enlargement of the spleen is sometimes ascertained.
• If the patient has extensive bleeding, then all the symptoms of posthemorrhagic hypochromic anemia are noted.
• Sometimes there is moderate leukocytosis.
• Against the background of the above, adrenal insufficiency may develop, as well as the activation of the progression of pathological processes in other organs of the organism, proceeding along the ishimic path.
• In the chronic course of the disease, hemorrhagic crises are periodically observed, accompanied by deep anemia and excessive blood outflows that affect large areas of the human body. If such a process affects the brain area, then the probability of death of the patient is high.

Treatment of Verlhof's Disease

The method of therapy for this disease largely depends on the stage of pathology and its flow patterns. If the case is neglected, surgical treatment may be necessary, which is expressed in the removal of the spleen. The very clinical treatment of Verlhof's disease includes the introduction of a protocol for the treatment of corticosteroid hormones and immunosuppressants.

For example, such hormones as, honey mead, prednisolone, decartine n20, prednisolone hemisuccinate, salt-decorin n250, prednisol.

Prednisolone - the hormone of the adrenal cortex - the way of its application and dosage is prescribed exclusively individually, depending on the patient's condition and the overall clinical picture of the disease. The starting daily dosage is 20-30 mg, which corresponds to four to six tablets, with a more severe form of pathological changes, the calculation of the daily dosage can be based on the formula of 1 mg of the drug per kilogram of the patient's weight divided into four to six receptions. In case of a particularly acute pathology, such an amount of the drug may not be sufficient, then after five to seven days, the recommended starting dosage can be doubled. The effect of these drugs primarily removes hemorrhagic syndrome, after its arrest, the mechanism of exposure allows you to gradually increase the number of platelets in the patient's blood.

The duration of treatment directly depends on obtaining a therapeutic effect and is carried out until the patient's complete recovery.

Contraindicated this drug with persistent retention of high blood pressure figures, diabetes mellitus, nephritis, psychosis of various genesis, ulcerous manifestations of the mucosa walls of the stomach and duodenum, in the case of the postoperative period, with the open form of tuberculosis. And also during pregnancy and if there is an increased sensitivity to the constituent components of the drug and the retirement age of the patient.

Strictly to cancel prednisolone should not be, its cancellation is carried out gradually. In parallel with the decrease in the number of hormones taken adrenal cortex, the patient is assigned delagil, chloroquine, chloroquine, hingamine phosphate.

Delagil - medicines for the treatment of protozoal infections is administered to the body orally after eating 0.5 g twice a week, then once a week, but on the same day. If necessary (at the discretion of the treating doctor), the schedule of therapy can be prescribed for 0.25-0.5 g once a day.

Contraindications to the use of this medication include dysfunction of the liver and kidneys, severe changes in the heart rate, with porphyrinuria in the patient's history, with oppression of hematopoiesis in the bone marrow, as well as with increased intolerance of the constituents of the drug.

If, however, when the hormones of the adrenal cortex are abolished, there is a sharp exacerbation of the disease, the physician returns the dosage to the original values.

If after four months of therapy fails to achieve a stable effectiveness of therapy, the doctor - hematologist or treating therapist, most likely, will prescribe an operation to remove the spleen. This therapeutic course in 80% of cases allows you to bring the patient to a full recovery.

The excision of the organ is covered by hormone therapy. Immediately before the operation, the doctor prescribes the amount of prednisolone administered more than the patient took up to this point.

If the patient is diagnosed with Verlhof disease, with what, an autoimmune form of manifestation, even the removal of the spleen is not always capable of leading a person to a complete cure. In this case, bleeding is stopped, but thrombocytopenia does not go away.

If the removal of the spleen did not solve the problem, the physician prescribes immunosuppressants (for example, aspress, imarant, azathioprine, azanine, azamun). In parallel with them in the protocol of treatment are introduced and drugs belonging to the group of glucocorticoids.

Immunosuppressive drugs azathioprine is prescribed for one to two months at a rate of 4 mg per kilogram of the patient's weight. Then, the dosage of the drug gradually begins to decrease to 3 - 2 mg per kilogram of body weight of the patient.

It is not recommended to prescribe this drug in case of severe liver pathology, leukopenia or in case of hypersensitivity of the patient's body to the constituent components of the drug.

The antineoplastic agent cyclophosphamide is referred to as alkylating compounds and prescribed 0.2-0.4 g daily. The whole course requires six to eight grams of the drug. The drug can be administered as an intravenous, intramuscular, intraperitoneal or intrapleural. The method of entering the patient's body is appointed by the treating doctor in each case individually.

Contraindications to the use of this drug include pathological changes in the kidneys, bone marrow hypoplasia, pronounced cachexia and anemia, oncological pathology in the terminal stage, increased intolerance to the components of the drug, leukopenia and / or thrombocytopenia, as well as the period of gestation of a toddler and feeding him with pectoral milk.

Vincristine - a herbal medicine that exerts an antitumor effect, is prescribed by a hematologist, 1 to 2 mg per square meter of the patient's body, taken once a week. The duration of the course of treatment is from one and half to two months.

A side effect of immunosuppressants are their mutagenic characteristics, therefore preparations of this group are not desirable to prescribe to babies, whose organism is still growing and developing. They do not apply to thrombocytopenia, which has an autoimmune character, before the operation to remove the spleen. In the pre-operative period, such medications do not show the necessary result, they are most effective in the postoperative period.

It is not effective in the case of Verlhof's disease and blood transfusion due to the fact that the process of melting platelets (thrombocytolysis) has not yet been eliminated.

When carrying out therapeutic treatment of Verlhof's disease, do not take medications in parallel that are capable of affecting platelet aggregation. To such drugs can be attributed kurantil, carbenicillin, aspirin, caffeine, brufen, butadione and various barbiturates.

If a woman has a history of thrombocytopenic purpura, then such a patient should not be prescribed such procedures as curettage of the uterine cavity, nor should she resort to pressure rewinding (tamponade).

The protocol of treatment, often includes other hemostatic techniques (local or general action). It can be dicycin, adroxone, estrogens, as well as a hemostatic sponge.

One of the best hemostatic drugs - dicinone - is prescribed by the attending physician at a rate of 10 to 20 mg per kilogram of the patient's weight divided by three to four doses. Mainly 0.25-0.5 g is administered once. For therapeutic necessity, the dosage of the drug can be increased to 0.75 g, taken three to four times throughout the day.

Contraindications of dicinone include hemorrhage, manifested on the basis of available anticoagulants, individual intolerance of the constituents of the drug, as well as if the patient has a history of thromboembolism or thrombosis.

In the event that thrombocytopenic purpura is diagnosed in a pregnant woman, hormone therapy does not have any significant impact on the development of the embryo, but when lactating, the newborn baby should be transferred to artificial feeding. It is necessary that the child does not receive antibodies from the mother's milk, which during this period develops her body.

Removal of the spleen to a pregnant woman is made only when the intended use of surgery is significantly higher for the woman's body than the consequences of postoperative rehabilitation for her unborn child.

Prevention of Verlhof's Disease

Based on the fact that the causes of the disease in question are not established in advance, there is no clear mechanism of these transformations, the primary prevention of Verlhof's disease has been very poorly developed to date. Here you can only include general prevention of morbidity:

• Prevention of infectious and inflammatory diseases of any genesis.
• Prevention of human body damage by various viruses.
• Do not abuse your stay in direct sunlight.
• Carefully follow the occurrence of allergic reactions of the body to any irritation.
• Very carefully refers to the reception of pharmacological preparations, clearly maintaining the way of their administration and dosage.
• Avoid both strong overheating and substantial subcooling of the body.
• Eliminate bad habits and adjust the diet.

Secondary prophylaxis of Verlhof disease is caused by the adoption of adequate measures to prevent the recurrence of thrombocytopenic purpura. In the light of this pathology, it is possible to give such recommendations:

• From his diet, the patient will have to remove acute food, spices, seasonings and vinegar.
• Physical stress should be gentle. Children, with the diagnosis in question, are exempt from physical education and sports.
• Such patients are not recommended for the therapy of other pathologies to use nonsteroidal anti-inflammatory drugs.
• Contraindicated in such patients and physiotherapy procedures. For example, such as UHF (impact on the affected area of the body or organ with high frequencies of the electromagnetic field) or various warming up (for example, UFOs).

Prognosis of Verlhof disease

The prognosis of Verlhof's disease is rather ambiguous and largely depends on the form of manifestation of the disease. That is, the pathology is in the acute phase of manifestation or has passed to the chronic stage, it has not an immune or, on the contrary, an immune character.

If the pathology of a megakaryocyte germ of an immunological nature is diagnosed, or the operation to remove the spleen did not bring the expected result, in such a situation the prognosis is very negative. Most likely, it will not be possible to avoid a fatal outcome for the patient.

The patient with the diagnosis under consideration is under the constant supervision of a specialist, and he is on a dispensary record. Periodically, every two to three months, such a patient undergoes a follow-up examination, using hemorrhagic tests. Even the appearance of a small bleeding for this patient is an indication for the appointment of drugs of hemostatic action.

It is believed that the disease in question is completely incurable, but with the right approach to therapy, this pathology has a favorable prognosis.

The most precious thing a person has is his health and, accordingly, his life. Perhaps, it is not always possible to avoid certain diseases, but it depends on the person how soon this pathology will be recognized and adequate measures taken that will not allow the body to cause significant damage. This theory can not be better treated for such a disease as Verlhof's disease. In her case, the earlier you score an alarm, turning to a specialist, the more effectively he will be able to help you. In this situation, not only further health, but also life directly depends on how soon measures were taken to stop the pathology in question.