Werlhof's disease: causes, symptoms, features and treatment

Werlhof’s disease is a severe blood pathology characterized by a decrease in the number of platelets, an increase in their tendency to stick together (aggregation) and the occurrence of point hemorrhages and hematomas under the skin. This ailment has been known since ancient times. In 1735, German doctor Paul Werlhof described the symptoms of this disease. Currently, this is one of the most common blood pathologies. Another name for Verlhof's disease is thrombocytopenic purpura.

Causes of the disease

At present, the exact causes of the pathology have not been established. Only provoking factors for the development of the disease can be distinguished. These include:

• previous infections of bacterial or viral etiology;
• operations on vessels with mechanical damage to platelets;
• bone marrow malignancies;
• the effect of radiation on the hematopoietic system ;
• the pathological response of the body to the administration of vaccines and therapeutic sera;
• long-term use of hormonal contraceptives;
• severe inhibition of hematopoiesis (aplastic anemia).

One of the names for Verlhof's disease is idiopathic thrombocytopenic purpura. It indicates the uncertainty of the etiology of this ailment. Idiopathies in medicine are called diseases that arise on their own, regardless of damage to other organs. The causes of such pathologies are usually unknown.

In some cases, Werlhof 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 this failure. The autoimmune form of the disease is also an idiopathic pathology.

There are hereditary forms of this disease. But they are rarely noted. Usually pathology is acquired.

How does pathology develop?

The mechanism of development of Werlhof’s disease and the blood picture is characterized by a sharp and rapid decrease in platelet count. For this reason, blood coagulation decreases. Vascular nutrition is deteriorating. Their walls undergo dystrophic changes. As a result, vascular permeability decreases, they begin to pass red blood cells. So there are subcutaneous hemorrhages, as well as external and internal bleeding.

Immune disorders also play a role in the pathophysiology of Werlhof disease. Pathological protective proteins that destroy platelets begin to be produced in the body. Mass death of these blood cells occurs.

In children, hereditary forms of thrombocytopenic purpura are often observed. In this case, the pathogenesis of Verlhof'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 violated.

Forms of the disease

In medicine, it is customary to subdivide Verlhof disease by the severity of hemorrhagic manifestations and by the severity of symptoms.

According to the classification of the World Health Organization, there are several gradations of pathology:

1. Zero The patient has no bleeding and hemorrhage.
2. First one. There are single point hemorrhages (petechiae) and spots (ecchymosis).
3. The second one. There are multiple petechiae and ecchymoses on the face, trunk and extremities.
4. The third. Not only skin hemorrhagic rashes are noted, but also bleeding of the mucous membranes.
5. Fourth. The patient has severe bleeding.

The disease is also classified by severity:

1. Light form. Hemorrhages and bleeding are absent or observed no more than 1 time per year.
2. Pathology of moderate severity. Hemorrhagic manifestations are noted no more than 2 times a year and quickly disappear after treatment.
3. Severe form. Exacerbations of the pathology occur more than 3 times during the year and are difficult to treat. Such complex cases often lead to patient disability.

Refractory thrombocytopenia is considered a separate form of the disease. She is difficult to treat and relapses even after surgery.

Disease code

According to ICD-10, Verlhof disease is included in the group of pathologies characterized by bleeding disorders, purpura, and hemorrhages. These diseases are indicated by codes D65 - D69.

Code D69 refers to diseases with purpura and hemorrhages. In the ICD, Werlhof's disease is indicated by code D69.3.

Clinical picture

Usually signs 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 hemorrhagic syndrome:

1. Under the skin, point hemorrhages and hematomas form. A small rash can merge and form spots. Bruises appear even without bruises or with a slight effect on the tissue (for example, with pressure).
2. There are bleeding from the nose and gums.
3. Blood is released from the intestines during bowel movements.
4. Severe hemorrhages from the digestive tract are sometimes observed. This causes nausea and diarrhea. Vomit and feces are black.
5. In rare cases, there is a discharge of blood from the lungs when coughing.
6. Monthly discharge in women becomes excessively plentiful. Uterine bleeding occurs during the menstrual period.
7. In the urine, a bloody admixture can be noticed.
8. Hemorrhages in the brain tissue are especially dangerous. They lead to the development of severe neurological symptoms (paralysis, seizures, lethargy, visual impairment) and often end in death. Such phenomena are very rare, in about 1% of cases, with very low platelet counts.

If the patient has only hemorrhagic rashes on the skin, then this form of the disease is called "dry" thrombocytopenia by the doctors. 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. Excessive nosebleeds appear. Hematomas on the skin of a sick child are formed very easily, even without external influences. Girls have uterine bleeding. Most often, the disease develops acutely after infections.

Complications

Complications of the disease include anemia. Hemoglobin level drops due to severe blood loss. This condition is characterized by weakness, dizziness, fainting.

Another dangerous consequence of the pathology is cerebral hemorrhage. It manifests itself as a severe headache with vomiting, convulsions and other disorders of the central nervous system.

Diagnostics

If a patient with hemorrhagic rashes seeks medical help, then this allows the doctor to suspect Verlhof's disease. In clinical recommendations, it is noted 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:

1. Inspection and medical history. The doctor examines the skin of the patient. It is necessary to identify the presence of rashes, bleeding, genetic factors, infections. You also need to determine the tendency to hemorrhages "pinch method." If you grab it with your fingers and squeeze the patient’s skin slightly, a hematoma will form in the patient. A cuff test is also carried out. A cuff from the apparatus for measuring blood pressure is put on the patient’s forearm and air is pumped into it. In a sick person, after severe compression, hemorrhages form on the skin.
2. General hematological test. A decrease in platelet count to a level of less than 100x109 / l can be observed in Verlhof disease. In the blood picture, the number of red blood cells and white blood cells is not changed. Hemoglobin level usually remains normal, anemia is observed only with severe bleeding.
3. Coagulogram. This study helps to determine blood coagulability, with this pathology, this indicator is usually sharply reduced.
4. Bone marrow puncture In this hematopoietic organ there are large megakaryocyte cells. Platelets are parts of their cytoplasm. They are separated from megakaryocytes. With purpura, this process is disturbed, which is detected by puncture.
5. Test for bleeding rate. In patients, the indicators of this analysis exceed the norm.
6. Blood test for biochemistry. Helps to identify concomitant diseases.
7. Research on HIV, hepatitis and herpetic infection. Allows you to separate the disease from secondary forms of purpura.
8. Myelogram. Helps to exclude oncological diseases of the hematopoietic system.
9. A study on antibodies to platelets. Using this method, you can identify an 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 Verlhof disease, the patient is prescribed hormones and immunosuppressants. These drugs reduce the pathological reaction of the body to its own platelets. The immune system stops destroying blood cells.

The following drugs from the group of glucocorticoid hormones are prescribed:

• 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, at the same time, these drugs also act as immunosuppressants.

Immunoglobulin preparations are administered intravenously: Octagam, Sandoglobulin, Human immunoglobulin, Venoglobulin. However, these funds can cause side effects in the form of a headache, chills, fever. To eliminate these undesirable manifestations, "Diphenhydramine" and "Dexamethasone" are taken at the same time.

In the absence of the effect of the use of hormones, interferon preparations are used. But at the same time, care must be taken, in some patients, these drugs cause flu-like symptoms.

With severe hemorrhagic syndrome, hemostatic drugs are prescribed: Dicinon, Thrombin, Ascorutin, Adroxon. The use of a collagen hemostatic sponge is also indicated. It is applied to the site of bleeding, then pressed or tied with a bandage. This helps to stop hemorrhagic manifestations.

In order for the therapy to be effective, the patient must refuse to take the following medicines:

• "Acetylsalicylic acid" and other non-steroidal anti-inflammatory drugs;
• vasodilator "Curantyl";
• hypnotic barbiturate series;
• caffeinated drugs;
• antibiotic "Carbenicillin".

These drugs thin the blood and may make the symptoms worse.

Hematologic procedures

Platelet transfusion is considered ineffective. This can improve the condition only for a short time. After all, platelets are rapidly destroyed in the patient's body. However, if the patient is threatened with hemorrhage in the brain, then such a procedure is carried out.

With severe bleeding, a red blood cell transfusion is performed. This helps to avoid manifestations of anemia.

Apply plasmapheresis treatment. 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 gastric ulcer, malignant tumors and severe iron deficiency anemia.

Surgery

In severe cases, an operation is performed splenectomy (removal of the spleen). This surgical intervention is indicated if the patient does not show improvement after 4 months of hormonal and immunosuppressive therapy . Also, surgery is necessary if the patient has a risk of life-threatening bleeding. Splenectomy is always performed on the background of hormone treatment.

However, in some cases, even the operation does not lead to persistent 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 allows for a remission.

Forecast

The prognosis of Verlhof disease is in most cases favorable. In 85-90% of cases, with proper treatment, stable remission can be achieved. In 10-15% of patients, the disease becomes chronic and recurs periodically.

Mortality in this pathology is 4-5%. Patients die with severe hemorrhage and cerebral hemorrhages. The prognosis is significantly worsened if the disease is not amenable even to surgical treatment.

All patients with idiopathic thrombocytopenic purpura are under medical supervision. Periodically, they need to undergo a clinical examination and take 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 are not 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;
• with the introduction of vaccines and serums, inform the doctor about all available contraindications;
• avoid exposure to excessive heat and extreme cold;
• minimize contact with allergens;
• when taking medications, carefully observe the prescribed dosages and pay attention to side effects.

These measures will help to somewhat reduce the risk of pathology.

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 relapse of the disease. Such patients are contraindicated for prolonged exposure to sunlight and physiotherapy. It is necessary to follow a diet with a restriction of spicy foods and avoid excessive physical exertion.