Werlhof's disease is a severe pathology of the blood, which is characterized by a decrease in the number of platelets, an increase in their tendency to stick together (aggregation) and the occurrence of pinpoint hemorrhages and hematomas under the skin. This disease has been known since ancient times. In 1735, the German physician Paul Werlhof described the symptoms of this disease. Currently, this is one of the most common blood pathologies. Another name for Werlhof's disease is thrombocytopenic purpura.
Causes of disease
Currently, the exact causes of the pathology have not been established. It is possible to single out only provoking factors for the development of the disease. These include:
- past infections of bacterial or viral etiology;
- operations on vessels with mechanical damage to platelets;
- malignant neoplasms of the bone marrow;
- impactradiation on the hematopoietic system;
- pathological response of the body to the introduction of vaccines and therapeutic sera;
- long-term use of hormonal contraceptives;
- severe hematopoiesis suppression (aplastic anemia).
One of the names of Werlhof's disease is idiopathic thrombocytopenic purpura. It indicates the ambiguity of the etiology of this disease. Idiopathies in medicine are called diseases that occur independently, regardless of lesions of other organs. The causes of such pathologies are usually unknown.
In some cases, Werlhof's disease develops as a result of autoimmune disorders. The body mistakenly recognizes platelets as foreign substances and begins to produce antibodies to them. However, it is impossible to establish what exactly causes such a failure. The autoimmune form of the disease is also an idiopathic pathology.
There are also hereditary forms of this disease. But they are rarely noted. Usually the pathology is acquired.
How pathology develops
The mechanism of the development of Werlhof's disease and the blood picture is characterized by a sharp and rapid decrease in the level of platelets. For this reason, blood clotting decreases. The nutrition of blood vessels is deteriorating. Their walls undergo dystrophic changes. As a result, the permeability of blood vessels decreases, they begin to pass red blood cells. This is how subcutaneous hemorrhages occur, as well as external and internal bleeding.
immune disorders. The body begins to produce pathological protective proteins that destroy platelets. There is a massive death of these blood cells.
Children often have hereditary forms of thrombocytopenic purpura. In this case, the pathogenesis of Werlhof's disease is associated with excessive activity of enzymes that destroy blood cells. And also with genetic forms of pathology, the structure of platelets is often disturbed.
Disease forms
In medicine, it is customary to subdivide Werlhof's disease according to the severity of hemorrhagic manifestations and the severity of symptoms.
According to the classification of the World He alth Organization, there are several gradations of pathology:
- Zero. The patient is not bleeding or bleeding.
- First. There are single pinpoint hemorrhages (petechiae) and spots (ecchymosis).
- Second. There are multiple petechiae and ecchymosis on the face, trunk and extremities.
- Third. There are not only skin hemorrhagic rashes, but also bleeding of mucous membranes.
- Fourth. The patient is bleeding heavily.
Also, the disease is classified according to the severity of the course:
- Easy shape. Hemorrhages and bleeding are absent or are observed no more than 1 time per year.
- Pathology of moderate severity. Hemorrhagic manifestations occur no more than 2 times a year and quickly disappear after treatment.
- Heavy form. Exacerbations of pathology occur more than 3 times inyears and are difficult to treat. Such complex cases of the disease often lead to the patient's disability.
Refractory thrombocytopenia is considered a separate form of the disease. It is difficult to treat and recurs even after surgery.
Disease code
According to ICD-10, Werlhof's disease is included in the group of pathologies characterized by blood clotting disorders, purpura and hemorrhages. These diseases are designated by codes D65 - D69.
Code D69 refers to diseases with purpura and hemorrhages. In the ICD, Werlhof's disease is designated by code D69.3.
Clinical picture
Usually, the symptoms of the disease appear suddenly. Sometimes their occurrence is preceded by a respiratory or intestinal infection. The initial symptoms of Werlhof's disease are manifested in weakness, fatigue, headache, nausea, and general malaise. However, the prodromal stage does not last long, and is soon replaced by a hemorrhagic syndrome:
- Point hemorrhages and hematomas form under the skin. A small rash can coalesce and form patches. Bruises appear even without bruises or with little impact on the tissues (for example, with pressure).
- Bleeding from the nose and gums.
- Blood is excreted from the intestines during bowel movements.
- Sometimes there are severe hemorrhages from the digestive tract. This results in nausea and diarrhea. Vomit and feces are black.
- In rare cases, there is bleeding from the lungs when coughing.
- Women's monthly discharge becomesexcessively abundant. Uterine bleeding occurs in the intermenstrual period.
- You can see blood in your urine.
- Hemorrhages in the brain tissue are especially dangerous. They lead to the development of severe neurological symptoms (paralysis, convulsions, lethargy, visual impairment) and often end in death. Such phenomena are observed very rarely, in about 1% of cases, with very low platelet counts.
If the patient has only hemorrhagic rashes on the skin, then doctors call this form of the disease "dry" thrombocytopenia. If the patient suffers from bleeding, then such symptoms are called "wet".
Werlhof's disease in children in 30% of cases is accompanied by a slight increase in the spleen. There is profuse bleeding from the nose. Hematomas on the skin of a sick child are formed very easily, even without external influence. Girls have uterine bleeding. Most often, the disease develops acutely after past infections.
Complications
Anemia is a complication of the disease. The hemoglobin level drops due to severe blood loss. This condition is characterized by weakness, dizziness, fainting.
Another dangerous consequence of pathology is cerebral hemorrhage. It is manifested by a severe headache with vomiting, convulsions and other disorders of the central nervous system.
Diagnosis
If a patient with hemorrhagic rashes seeks medical help, thenthis allows the doctor to suspect Werlhof's disease. The clinical guidelines state that this pathology is a diagnosis of exclusion. It is necessary to conduct a comprehensive examination of the body. It is necessary to exclude other diseases in which the platelet count is less than 100x109/L.
The following diagnostic measures are recommended:
- Examination and history taking. The doctor examines the patient's skin. It is necessary to identify the presence of rashes, bleeding, genetic factors, past infections. It is also necessary to determine the tendency to hemorrhages by the "pinch method". If you grab with your fingers and slightly squeeze the patient's skin, then the patient will form a hematoma. A cuff test is also carried out. On the forearm of the patient put on a cuff from the apparatus for measuring blood pressure and pump air into it. In a sick person, after strong compression, hemorrhages form on the skin.
- General hematology test. A decrease in platelets to a level of less than 100x109 / l can be observed in Werlhof's disease. In the blood picture, the number of erythrocytes and leukocytes did not change. Hemoglobin levels usually remain normal, anemia is observed only with heavy bleeding.
- Coagulogram. This study helps to determine blood clotting, with this pathology, this indicator is usually sharply reduced.
- Bone marrow puncture. This hematopoietic organ contains large cells called megakaryocytes. Platelets are parts of their cytoplasm. They separate from megakaryocytes. In purpura, this process is disturbed, which is revealed during puncture.
- Test onbleeding rate. In patients, the indicators of this analysis exceed the norm.
- Blood test for biochemistry. Helps identify comorbidities.
- Research for HIV, hepatitis and herpes infection. Allows you to separate the disease from secondary forms of purpura.
- Myelogram. Helps to exclude oncological diseases of the hematopoietic system.
- Research for antibodies to platelets. With this method, you can identify the autoimmune form of the disease.
In addition, women need to be examined by a gynecologist and mammologist, and men - by a urologist or andrologist. This will help to exclude oncological pathology.
Drug therapy
In the treatment of Werlhof's disease, the patient is prescribed hormonal agents and immunosuppressants. These drugs reduce the body's pathological reaction to its own platelets. The immune system stops destroying blood cells.
Prescribe the following drugs from the group of glucocorticoid hormones:
- "Prednisolone";
- "Danazol";
- "Hydrocortisone";
- "Methylprednisolone".
To suppress the immune response, drugs are used: Delagil, Chloroquine, Hingamine, Azathioprine. They are used in medicine to treat protozoal infections. However, these drugs also act as immunosuppressants at the same time.
Intravenously administeredimmunoglobulin preparations: "Oktagam", "Sandoglobulin", "Human immunoglobulin", "Venoglobulin". However, these drugs can cause side effects in the form of headache, chills, fever. To eliminate these unwanted manifestations, they simultaneously take Diphenhydramine and Dexamethasone.
If there is no effect from the use of hormones, interferon preparations are used. However, care must be taken as these drugs cause flu-like symptoms in some patients.
With severe hemorrhagic syndrome, hemostatic drugs are prescribed: Dicinon, Thrombin, Ascorutin, Adroxon. The use of a collagen hemostatic sponge is also shown. It is applied to the site of bleeding, then pressed or tied with a bandage. This helps to stop hemorrhagic manifestations.
For the therapy to be effective, the patient must stop taking the following medications:
- "Acetylsalicylic acid" and other nonsteroidal anti-inflammatory drugs;
- vasodilator "Kurantila";
- barbiturate hypnotics;
- caffeinated drugs;
- antibiotic "Carbenicillin".
These drugs thin the blood and can make symptoms worse.
Hematology procedures
Platelet transfusion is considered ineffective. This may improve the condition only for a short time. After all, platelets quicklydestroyed in the patient's body. However, if the patient is threatened with cerebral hemorrhage, then such a procedure is carried out.
In case of severe bleeding, a transfusion of red blood cells is done. This helps to avoid anemia.
Apply treatment with plasmapheresis. The blood is filtered by passing through a special apparatus. This procedure helps to cleanse the body of antibodies to platelets. However, plasmapheresis is not indicated for all patients. It is not recommended for stomach ulcers, malignant tumors and severe iron deficiency anemia.
Surgical treatment
In severe cases, a splenectomy (removal of the spleen) is performed. This surgery is indicated if the patient does not improve after 4 months of hormonal and immunosuppressive therapy. Also, surgery is necessary if the patient is at risk of life-threatening bleeding. Splenectomy is always done in conjunction with hormonal treatment.
However, in some cases, even an operation does not lead to a stable remission. After splenectomy, most patients stop bleeding. But in some patients, the platelet count is still very low. In this case, together with hormones, strong immunosuppressants are prescribed: Cyclophosphamide, Vincristine. They often cause side effects (hair loss, baldness), but their use can achieve remission.
Forecast
Prognosis of Werlhof's disease infavorable in most cases. In 85-90% of cases, with proper treatment, a stable remission can be achieved. In 10-15% of patients, the disease becomes chronic and recurs periodically.
Lethality in this pathology is 4-5%. Patients die with severe hemorrhage and cerebral hemorrhage. The prognosis worsens significantly if the disease is not even amenable to surgical treatment.
All patients with idiopathic thrombocytopenic purpura are under medical supervision. Periodically, they need to undergo a dispensary examination and pass hematological tests. If the patient has even a slight bleeding, then he is shown an urgent appointment of coagulants.
Prevention
Since the causes of the pathology have not been clarified, special prevention of thrombocytopenic purpura has not been developed. You can only follow the general rules for the prevention of diseases:
- timely treat infectious and viral pathologies;
- when administering vaccines and sera, inform the doctor about all contraindications;
- avoid exposure to excessive heat and extreme cold;
- minimize contact with allergens;
- when taking medication, carefully follow the prescribed dosages and pay attention to side effects.
These measures will help reduce the risk of pathology somewhat.
If a person has already been diagnosed with thrombocytopenic purpura, then it is necessary to follow the recommendations of the attending physician in order to prevent a recurrence of the disease. Such patientsprolonged exposure to sunlight and physiotherapy are contraindicated. It is necessary to follow a diet with limited spicy food and avoid excessive exercise.
