In Werlhof disease – colloquially known as Werlhof disease – (thesaurus synonyms: Acute essential thrombocytopenia; Acute idiopathic thrombocytopenia; Chronic essential thrombocytopenia; Chronic idiopathic thrombocytopenia; Chronic idiopathic thrombocytopenic purpura; Essential thrombocytopenia; Evans syndrome; Hemorrhagic purpura; Idiopathic thrombocytopenia; Idiopathic thrombocytopenic purpura; Morbus maculosus haemorrhagicus Werlhof; Werlhof’s disease; Purpura haemorrhagica; Thrombocytopenic purpura; Thrombocytopenic purpura idiopathica; Transfusion refractory essential thrombocytopenia; Transfusion refractory idiopathic thrombocytopenic purpura; Transfusion refractory Evans syndrome; Transfusion refractory Werlhof disease; Transfusion refractory Werlhof purpura; Werlhof purpura; Werlhof-Wichmann syndrome; ICD-10-GM D69. 3: Idiopathic thrombocytopenic purpura) is a form of idiopathic thrombocytopenic purpura (ITP). Idiopathic thrombocytopenic purpura refers to thrombocytopenia (deficiency of platelets < 150,000/μl) without an identifiable cause. Furthermore, platelet survival is shortened and antibodies are found in the blood of affected individuals.
According to its course, idiopathic thrombocytopenic purpura can be divided into:
- Acute idiopathic thrombocytopenic purpura (ITP) – mainly children are affected; usually preceded by a respiratory or gastrointestinal tract infection; self-limiting course (stops by itself).
- Chronic idiopathic thrombocytopenic purpura (ITP) (duration > 6 months) – affects mainly adults; here are observed clustered parallel infections of the stomach with the bacterium Helicobacter pylori
Sex ratio (acute form of progression): boys and girls are equally affected. Sex ratio (chronic form of progression): males to females is 1: 3.
The incidence (frequency of new cases) of idiopathic thrombocytopenic purpura in adults is about 6-8 cases per 100,000 inhabitants per year (in Germany).
Course and prognosis: With optimal therapy, the prognosis of idiopathic thrombocytopenic purpura in adults is favorable. The cure rate is 70-80%. Spontaneous cure is not uncommon, especially in children. The lethality (mortality related to the total number of people suffering from the disease) is 4%. The most common cause of death is intracerebral hemorrhage (ICB; brain bleeding in the brain tissue itself).
The disease is often recurrent (recurring). If this is frequent, splenectomy (surgical removal of the spleen) should be considered. However, recurrence (recurrence) can also occur following splenectomy.
