Werlhoff's disease

This disease spares no one. It is diagnosed in newborns, preschool and school-age children, and adults are not immune from it either. But as statistics show, women suffer from this pathology three times more often than the male half of humanity. So what is Werlhof's disease? Can it be effectively treated? We will try to find answers to all these and many other questions in this article.

Causes of Werlhof's disease

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

To date, it has not been possible to establish the exact causes of Werlhof's disease. If we speak unequivocally about the hereditary, genetic nature of the disease, then it is diagnosed much less often than the acquired form of the lesion. Enzyme-pathic, immune or viral genesis of the pathology have also been considered, but even this turn of the problem has not yet received serious confirmation.

Symptoms of Werlhof's disease

The lesion, developing in the human body, leads to blockage of the passage section of small capillaries. If this disease is suspected, the doctor may observe the following symptoms of Werlhof's disease:

• A decrease in the amount of platelets in the patient's blood taken per unit of plasma volume. This result can be obtained due to the active use of these blood components in the formation of blood clots.
• Thrombocytopenic purpura is accompanied by ischemic disorders that affect almost all organs of the human body: the heart, gallbladder, liver, genitals, kidneys, brain, and so on.
• This disease is classified as a pathology that develops quite quickly, affecting absolutely all age categories.
• The most common patients affected by thrombocytopenic purpura are those aged 10 to 50 years.
• Pathological changes can arise “out of the blue” (against the background of absolute health), or after an infectious, cold or intestinal pathology.
• The person begins to experience pain in the head area.
• He feels quickly tired.
• A general decrease in the body's tone is felt.
• The patient has a poor appetite.
• Nausea may be a symptom of Werlhof's disease, and in rare situations it can even develop into a gag reflex.
• A fever of an irregular type may appear.
• Pain symptoms occur, localized in the chest and peritoneum.
• Almost the entire body of the patient is covered with small hemorrhages and bruises. Hemorrhages are spontaneous, appearing mainly at night.
• Visual impairment.

Gradually, as the disease progresses, the following may join the already existing symptoms of Werlhof's disease:

• Bleeding of various localizations: from the nasal passages, uterus, from the gastrointestinal tract, hemorrhage in the retina, bleeding from the gums. Somewhat less often, hemoptysis may join.
• Symptoms of a neuralgic nature may also occur:
  • Paralysis of one side of the body.
  • Ataxia is a disorder of balance and coordination of voluntary movements.
  • Disorientation in space.
  • Slowness of conversation.
  • Cramps.
  • Tremor
  • A fog appears before my eyes.
  • Sometimes acute mental disorders develop.
  • In a particularly severe situation, the patient falls into a comatose state.
• But the main clinical picture of the disease is still bleeding from the mucous membranes (especially in small patients) or hemorrhages manifested on the skin. They can occur as a result of bruises or injections, and without any reason. Hemorrhages on the epidermis can have different sizes (from small dots to fairly large spots), but they are mainly localized on the front side of the human body, as well as the upper and lower extremities. The difference between hemorrhages in Werlhof's disease and classic vascular bleeding is that, unlike the latter, these bruises can periodically merge with their neighbors. This factor is an actual assessment in determining the diagnosis of thrombocytopenic purpura and assessing the nature of hemorrhagic diathesis.
• Depending on the duration of the hemorrhage, as in the case of a vascular bruise, the shade of the hemorrhage spots can be either greenish-blue or dark brown or yellow.
• In a woman of childbearing age, bleeding may occur in the ovaries, which can simulate an ectopic pregnancy.
• The pathology occurs with frequent exacerbations.
• The patient's ability to work decreases.

Werlhof's disease in children

No matter how hard adults try, they won't be able to completely protect their baby from blows and abrasions. A still unsteady gait, imperfect coordination combined with innate mobility can lead the baby to fall even on level ground. And as a result of the "flight" - a bruise on the baby's delicate skin. However, sometimes hemorrhages begin to appear on the baby's body, seemingly for no apparent reason. This should really alert parents and become a reason to go to a pediatrician for a consultation. After all, such symptoms can be the first symptom that Werlhof's disease begins to manifest in children.

It is this disease that pediatricians consider to be the most common cause of damage to the child's body in the area of genetic pathologies associated with the hematopoietic system in children. The frequency of diagnosing thrombocytopenic purpura in children is one to two cases per hundred thousand healthy children. This disease does not reveal a predominant commitment to a certain sex in children.

The reasons for the appearance of the pathology in question in young patients are still unknown. When naming the root cause of the disease, doctors are divided in their opinion. Some believe that it is genetic in nature. The development of pathology associated with the inferiority of a blood cell, the cause of which is a mutation. Others talk about the immune trace of the disease, developing due to a drop in the level of the child's body's defenses. Still others are ready to argue that the mechanism that triggers this disease is the presence of both of these causes at the same time.

The first signs of the disease can appear after a short period of time after drug therapy, severe hypothermia or overheating, vaccination, as a result of infectious diseases caused by pathogenic flora (bacteria or viruses). There are several pathogenetic ways of progression of this disease:

1. A confluence of certain factors occurs, which triggers the mechanism of synthesis of special antibodies that become antagonists for one's own platelets. The process itself occurs as the formation of an antibody-antigen tandem on the wall of a blood cell. This formation leads to cell death.
2. In parallel with this, a failure occurs in the process of formation and development of thrombocytes in the organs of hematopoiesis. That is, the newly formed antigen not only destroys the already "adult" blood cell, but also introduces a change in the normal process of development of the megakaryocytic sprout.
3. This disruption of the hematopoiesis process leads to thrombocytes living less than they should. In a healthy organism, this component of the blood lives from ten to twelve days, whereas in Werlhof's disease this period can be reduced to one or two days.

As a result, the quantitative component of platelets in the child's body significantly decreases, which invariably affects the functional inability of the body to function normally. Platelets are the body's nutrition. A decrease in the level of this component of the blood provokes a decrease in the endothelial layer of the blood capillaries. This is the impetus for the appearance of sudden, prolonged and profuse bleeding.

If such a patient does not receive timely medical care, the result may be very tragic – fatal.

There are cases when Werlhof's disease is first diagnosed to a woman during the period when she is carrying her baby. Mostly, women with such a diagnosis carry a child quite normally, and childbirth is within the normal range. Blood loss during childbirth, with a diagnosis of thrombocytopenic purpura, shows volumes that are quite acceptable and do not pose a threat to the life of the woman in labor. Whereas artificial termination of pregnancy can provoke much larger volumes of bleeding. The only exception is the acute form of the disease.

Based on the above, it can be stated that the diagnosis of Werlhof's disease in a pregnant woman does not serve as a contraindication to further bearing and giving birth to a baby. Frequent and fairly heavy bleeding may serve as an indication for artificial termination of pregnancy.

Diagnosis of Werlhof's disease

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

• Analysis of the clinical picture of the disease.
• Identifying the patient's medical history.
• Examination of the patient by a specialist. Symptom of pinching. In thrombocytopenic purpura, when pinched, a bruise immediately forms under the skin.
• Endothelial tests are mandatory.
  • The cuff test allows you to assess the nature of the norm of the state of small capillaries (elasticity, flexibility and fragility level). To conduct this study, an ordinary cuff is used, which is used to measure blood pressure. The level of blood pressure is preliminarily determined as normal. Then, after a certain time, the cuff is applied again and the blood pressure is brought to values above the norm by 10 - 15 units of mm Hg. With the disease in question, petechiae begins to appear almost immediately - many small hemorrhages are formed. Such a picture is a positive result of tests for thrombocytopenic purpura.
  • The hammer sign is similar to the plucking sign, but a hammer is used instead of a pinch.
  • Cupping and prick tests or Waldman's test. This method allows to determine the presence of a hyperergic process in the patient's body, directly affecting the system of blood vessels and hematopoiesis (this is a rapid course of the pathological process, which, even despite timely and adequate therapy, leads to the death of the patient). The procedure is carried out using a medical can (hence the name of the study), which is placed on the patient. The can is mainly attached slightly below the collarbone. With its help, irritation of the epidural capillaries is achieved. It is held for about twenty minutes, after which a blood smear is taken from the area where the can was standing and the level of monocytes is determined. The control is the number of these same structures, but determined before this irritation. Such a study is done not once, but 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. When it is performed, thrombocytopenia can be detected - the main symptom of Werlhof's disease. When conducting a laboratory study using magnifying equipment, the giant size of thrombocytes can be clearly seen.
• A blood coagulation test is also prescribed. This analysis shows an increase in bleeding time. The pathology is judged by the degree of decrease in the volume of the plasma clot with a standard platelet content (retraction of the blood clot).
• Bone marrow puncture is also performed. This study, in thrombocytopenic purpura, allows us to state a sharp increase in the level of megakaryoblasts, promegakaryocytes and megakaryocytes in the patient's body. In this case, in a normal state, platelets are pinched off from the nucleus of megakaryocytes, ridding them of cytoplasm, which provokes the structural formation of the megakaryocyte to dismember into several components, after which they are utilized by phagocytosis (the process of active capture and absorption of living organisms (bacteria, fungi, etc.) and non-living particles by unicellular organisms). In case of illness, the procedure of platelet pinching rapidly reduces its activity.
• When diagnosing Werlhof's disease, a complete examination of the patient is carried out, during which an enlarged spleen is sometimes noted.
• If the patient experiences extensive bleeding, then all the symptoms of posthemorrhagic hypochromic anemia are subsequently noted.
• Moderate leukocytosis is sometimes observed.
• Against the background of the above, adrenal insufficiency may develop, as well as activation of the progression of pathological processes in other organs of the body, proceeding along the ischemic pathway.
• In chronic cases of the disease, hemorrhagic crises are periodically observed, accompanied by deep anemia and excessive blood effusions, which affect large areas of the human body. If such a process affects the brain area, then the probability of the patient's death is high.

Treatment of Werlhof's disease

The method of therapy for this disease largely depends on the stage of the pathology and the characteristics of its course. In advanced cases, surgical treatment may be required, which involves removing the spleen. The clinical treatment of Werlhof's disease itself includes the introduction of corticosteroid hormones and immunosuppressants into the treatment protocol.

For example, hormones such as medopred, prednisolone, decortin n20, prednisolone hemisuccinate, solu-decortin n250, prednisol.

Prednisolone is an adrenal cortex hormone - its method of administration and dosage are prescribed strictly 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 daily dosage can be calculated according to the formula 1 mg of the drug per kilogram of the patient's weight, divided into four to six doses. In particularly acute pathology, this amount of the drug may be insufficient, then after five to seven days, the recommended starting dosage can be doubled. The action of these drugs primarily relieves hemorrhagic syndrome, after its relief, the mechanism of action allows to gradually increase the number of platelets in the patient's blood.

The duration of treatment directly depends on the achievement of the therapeutic effect and is carried out until the patient has fully recovered.

This medicine is contraindicated in case of persistent high blood pressure, diabetes, nephritis, psychosis of various genesis, ulcerative manifestations of the mucous membrane of the stomach and duodenum, in the postoperative period, in the open form of tuberculosis. And also during pregnancy and if there is increased sensitivity to the components of the medicine and the patient's retirement age.

Prednisolone should not be discontinued abruptly, it is discontinued gradually. In parallel with the reduction in the amount of adrenal cortex hormones taken, the patient is prescribed delagyl, chloroquine, chloroquine, phosphate hingamine.

Delagil - a medicinal product for the treatment of protozoal infections is administered orally, after meals, 0.5 g twice a week, then once a week, but on the same day. If necessary (at the discretion of the attending physician), the therapy schedule can be scheduled at 0.25 - 0.5 g once a day.

Contraindications for the use of this drug include dysfunction of the liver and kidneys, severe changes in heart rhythm, a history of porphyrinuria in the patient, suppression of hematopoiesis in the bone marrow, as well as increased intolerance to the components of the drug.

If, when discontinuing adrenal cortex hormones, a sharp exacerbation of the disease is observed, the attending physician returns the dosage of this drug to the original values.

If after four months of therapy it is not possible to achieve stable effectiveness of therapy, the doctor - hematologist or attending physician, most likely, will prescribe an operation to remove the spleen. Such a therapeutic course in 80% of cases allows the patient to achieve complete recovery.

The organ excision is performed under the cover of hormonal therapy. Immediately before the operation, the doctor prescribes a larger amount of prednisolone than the patient has taken up to that point.

If a patient is diagnosed with Werlhof's disease, and in its autoimmune form, even removal of the spleen is not always able to bring a person to a complete cure. In this case, bleeding is stopped, but thrombocytopenia does not go away.

If the removal of the spleen does not solve the problem, the attending physician prescribes immunosuppressants (for example, azapress, imuran, azathioprine, azanine, azamun) to the patient. In parallel with them, medications belonging to the glucocorticoid group are also introduced into the treatment protocol.

Immunosuppressant 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 the patient's body weight.

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 components of the drug.

The antineoplastic drug cyclophosphamide is classified as an alkylating compound and is prescribed at 0.2–0.4 g daily. The entire course requires six to eight grams of the drug. The drug can be administered intravenously, intramuscularly, intraperitoneally, or intrapleurally. The method of administration to the patient is prescribed by the attending physician in each individual case.

Contraindications for the use of this drug include pathological changes in the kidneys, bone marrow hypoplasia, severe cachexia and anemia, terminal oncological pathology, increased intolerance to the components of the drug, leukopenia and/or thrombocytopenia, as well as the period of pregnancy and breastfeeding.

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

A side effect of immunosuppressants is their mutagenic characteristics, so it is not advisable to prescribe drugs of this group to children whose body is still growing and developing. They are also not used for thrombocytopenia of an autoimmune nature, before surgery to remove the spleen. In the preoperative period, such drugs do not show the necessary result, they are most effective in the postoperative period.

Blood transfusion is also not effective in Werlhof's disease because the process of platelet melting (thrombocytolysis) has not yet been eliminated.

When conducting therapeutic treatment of Werlhof's disease, one should not simultaneously take medications that can affect platelet aggregation. Such drugs include curantil, carbenicillin, aspirin, caffeine, brufen, butadion and various barbiturates.

If a woman has a history of thrombocytopenic purpura, then such a patient should not be prescribed procedures such as curettage of the uterine cavity, and one should not resort to pressing rewound (tamponade).

The treatment protocol often includes other hemostatic methods (local or general action). These may be dicynone, adroxone, estrogens, as well as a hemostatic sponge.

One of the best hemostatic pharmaceuticals - dicynone - is prescribed by the attending physician at the rate of 10 - 20 mg per kilogram of the patient's weight, divided into three or four doses. Mostly 0.25 - 0.5 g is administered at one time. If therapeutically necessary, the dosage of the drug can be increased to 0.75 g, taken three or four times a day.

Contraindications to Dicynone include hemorrhage, which occurs due to existing anticoagulants, individual intolerance to the components of the drug, and also if the patient has a history of thromboembolism or thrombosis.

If thrombocytopenic purpura is diagnosed in a pregnant woman, the hormone therapy does not have any significant effect on the development of the embryo, but during lactation, it is advisable to transfer the newborn baby to artificial feeding. This is necessary so that the child does not receive antibodies with the mother's milk, which her body is producing at this time.

Removal of the spleen of a pregnant woman is performed only when the expected benefit of the surgical intervention is significantly higher for the woman’s body than the consequences of postoperative rehabilitation for her future child.

Prevention of Werlhof's disease

Based on the fact that the causes of the disease in question have not been fully established, and a clear mechanism for these transformations has not been identified, primary prevention of Werlhof's disease has been very poorly developed to date. This can only include general prevention of the disease:

• Prevention of infectious and inflammatory diseases of any genesis.
• Preventing the human body from becoming infected with various viruses.
• Avoid excessive exposure to direct sunlight.
• Carefully monitor the appearance of allergic reactions of the body to any irritations.
• Be very careful when taking pharmacological drugs, strictly adhering to the method of administration and dosage.
• Avoid both severe overheating and significant hypothermia of the body.
• Eliminate bad habits and adjust your diet.

Secondary prevention of Werlhof's disease is determined by taking adequate measures to prevent relapses of thrombocytopenic purpura. In light of this pathology, the following recommendations can be made:

• The patient will have to eliminate spicy foods, spices, seasonings and vinegar from his diet.
• Physical activity should be gentle. Children with the diagnosis in question are exempted from physical education and sports.
• Such patients are not recommended to use non-steroidal anti-inflammatory drugs for the treatment of other pathologies.
• Physiotherapeutic procedures are also contraindicated for such patients. For example, UHF (exposure to the affected area of the body or organ by high frequencies of an electromagnetic field) or various warmings (for example, UV radiation).

Prognosis of Werlhof's disease

The prognosis of Werlhof's disease is quite 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 into the chronic stage, it has a non-immune or, conversely, immune character.

If a pathology of the megakaryocytic 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.

A patient with the diagnosis in question is under constant observation by a specialist and is registered with him. Periodically, once every two to three months, such a patient undergoes a control examination, using hemorrhagic tests. Even the appearance of minor bleeding for this patient is an indication for the prescription of hemostatic drugs.

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 their health and, accordingly, their life. It may not always be possible to avoid certain diseases, but it is up to the person how soon this pathology will be recognized and adequate measures will be taken to prevent significant damage to the body. This theory is best suited to such a disease as Werlhof's disease. In this case, the sooner you sound the alarm by contacting a specialist, the more effectively he will be able to help you. In this situation, not only future health, but also life directly depends on how soon measures are taken to stop the pathology in question.