Immune thrombocytopenic purpura is also known as idiopathic thrombocytopenic purpura or Werlholf’s disease. It is a condition caused by the production of anti-platelet autoantibodies for no apparent reason, which shorten the survival time of platelets. Mostly people aged 20-25 suffer from this disease.
Werlholf’s disease – causes
The cause of Werlholf’s disease is unknown. Specific antiplatelet autoantibodies lead to the destruction of platelets or inhibition of their production in the bone marrow. So far, the reasons for the production of antibodies are not known, but viral infections and the role of Helicobacter pylori are taken into account. ITP occurs in the adult population at a frequency of 3,2-6,6 / 100 per year. Most often, however, people aged 000-20 are ill, and women 25 times more often. In the case of children, boys under 4 years of age are more likely to suffer from the disease. and girls over 2 years of age
Symptoms of Werlholf’s disease
The most common symptom of Werlholf’s disease is bleeding of varying intensity:
- nose bleeds
- bleeding gums
- heavy menstrual bleeding
- skin ecchymoses.
Usually, petechiae can be found on the mucous membranes and on the distal parts of the limbs. In aspiration biopsy or trepanobiopsy, there is an increased number of megakaryocytes without dysplasia.
Werlholf’s disease – treatment
Werholf’s disease is most often treated with glucocorticosteroids (prednisone -1 mg / kg / d) and splenectomy. If the above-mentioned actions are not effective, immunosuppressants are also included in the therapy (cyclophosphamide – 2-3 mg / kg / dpo daily, azathioprine – 1-3 mg / kg / d, cyclosporine).
Lit.: [1] Shad A.T., Gonzalez C.E., Handler S.G.: Treatment of immune thrombocytopenic purpura in children. Pediatr Drugs 2005, 7; 325 -36. [2] Stasi R., Provan D.: Management of immune thrombocytopenic purpura in adults. Mayo Clin Proc 2004, 79; 504 -22.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House