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Moschcowitz syndrome, or thrombotic thrombocytopenic purpura, is an immediate life-threatening condition, and 90 percent of it if left untreated. cases lead to death. Moschcowitz syndrome is at risk of, among others, people suffering from autoimmune diseases (e.g. lupus) and pregnant women. What are the causes and symptoms of thrombocytopenic purpura? What is the treatment?

Moschcowitz syndrome , orthrombotic thrombocytopenic purpura(thrombotic thrombocytopenic purpura - TTP) belongs to the group of thrombocytopenic bleeding disorders. In the course of TTP, platelets stick together, and as a consequence, scattered blood clots form in small vessels (in small arteries and capillaries). Blood clots that block blood vessels impair blood flow to various organs, leading to hypoxia and failure.

On average, 40 out of 100,000 people suffer from Moschcowitz syndrome annually.

In addition, the presence of blood clots damages the erythrocytes flowing through them, which results in microangiopathic hemolytic anemia. In turn, thrombocytopenia (thrombocytopenia) develops as the platelets are used up to form clots. Thus, Moschcowitz syndrome is one of the few conditions in which thrombocytopenia is associated with the formation of blood clots.

Moschcowitz syndrome (thrombotic thrombocytopenic purpura) - causes

The cause of thrombotic thrombocytopenic purpura may be damage to the vascular endothelium, but most often it is a deficiency (and hence - decreased activity) of the ADAMTS13 enzyme, which is responsible for the breakdown of von Willebrand factor multimers - one of the coagulation factors. As a consequence, extremely large von Willebrand factor multimers appear in the blood, which show an increased ability to bind to platelets and to form platelet aggregates and further clots.

IgG autoantibodies against this enzyme may be responsible for the ADAMTS13 enzyme deficiency. Their presence is observed:

  • in the course of autoimmune diseases (e.g. systemic lupus erythematosus - develops in 1-4% of cases);
  • following bacterial infections andviral;
  • after treatment with thienopyridine derivatives: ticlopidine and clopidogrel;
  • in disseminated neoplastic disease;
  • after allogeneic bone marrow transplant;

In addition, pregnant women are at risk of developing TTP in the flu.

Thrombotic thrombocytopenic purpura can also be caused by an inherited deficiency of the ADAMTS-13 enzyme (akaUpshaw-Schulman syndrome ).

Moschcowitz syndrome (thrombotic thrombocytopenic purpura) - symptoms

In the case of Moschcowitz syndrome suddenly the following appear:

  • symptoms of hypoxia and damage to the central nervous system (occurring in 60-88% of cases) - headache, behavioral changes, visual and hearing impairment, in extreme cases - coma;
  • renal dysfunction (18-76% of cases);

However, the symptoms of hypoxia and damage (resulting from impaired circulation in the capillaries) can affect any organ, e.g. the heart (myocardial ischemia), pancreas or adrenal glands.

  • fever (22-86% of cases);
  • jaundice, enlargement of the spleen and liver (46% of cases);

Moschcowitz syndrome (thrombotic thrombocytopenic purpura) - diagnosis

Diagnosis is made by the results of a blood test. During the diagnosis, other diseases should be excluded, such as: acute intravascular coagulation syndrome, antiphospholipid syndrome, autoimmune destruction of platelets and erythrocytes (Evans syndrome) and hemolytic uremic syndrome (HUS).

Moschcowitz syndrome (thrombotic thrombocytopenic purpura) - treatment

In patients with thrombotic thrombocytopenic purpura,plasmapheresis , i.e. plasma exchange, is used. The procedure involves the separation of plasma into blood cells and its removal along with undesirable components (in this case together with antibodies blocking the activity of the ADAMTS13 enzyme and an excess of von Willebrand factor multimers).

Plasma exchange significantly increases the chance of survival - 70-90 percent. TTP symptoms regress.

The removed plasma is then replaced with fresh frozen plasma, albumin solution and / or crystalloids or colloids. In addition, in Moschcowitz syndrome, the action of plasmapheresis consists not only in the exchange of plasma, but also in replenishing the missing plasma components - the patient receives the active ADAMTS13 enzyme, which prevents the formation of platelet clots. Usually 3-4 liters of plasma are replaced every day, until the symptoms of the disease disappear ( although some sources say that

Usuallycorticosteroids are administered in parallel (their effectiveness has not been assessed in clinical trials, but there are many reports supporting this treatment). If these methods are ineffective, immunosuppressive treatment is administered. In addition, all patients should receive folic acid.

Additionally, a platelet concentrate is used to neutralize thrombocytopenia.

In recent years, very promising reports of attempts to treat resistant forms of the disease with rituximab have been published.

Important

U 30-60 percent relapses of the disease were observed - most of them occurred in the first month after treatment of the acute episode. A symptom of a relapse is a gradual decline in the number of platelets in the blood.

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Category:

Cardiology