Werlhof's disease
Werlhof Disease (chronic idiopathic thrombocytopenic purpura, ITP)
The ITP is a disorder in which there a paucity of platelets (Thrombocytopenia) comes.
Since the platelets (platelet) is an essential component of the coagulation system, leading to a lack of spontaneous bleeding, which in this (chronic) form of ITP, however, is usually quite low.
As can be at 80% of patients demonstrate autoantibodies against platelets, it is now fairly sure that it is an autoimmune disease
Frequency
It is estimated the incidence of the disease Werlhof to about seven new cases per 100,000 population per year. It affects women about three times more frequently than men.
Symptoms
- Spontaneous bleeding (hematoma, individual petechiae (small blood spots), nose bleeds)
- No swelling of the lymph nodes or the spleen
Diagnosis
- Exclusion of another cause of bleeding
- greatly shortened survival of platelets
Antibody detection
Complications
intracerebral (brain is located) bleeding
Therapy
- unless there are bleeding and the platelet count is not below 30 000, will be awaited
- Corticosteroids and immunoglobulins
- Immunosuppressants (eg, CD-20 antibodies) result in a subset of patients, improvement
- A splenectomy (removal of the spleen) results in 80% of patients experienced an improvement of the clinical picture
Forecast
The lethality of this disease is about 4%