Severe chronic neutropenia includes a heterogeneous group of rare diseases in childhood. Differential diagnosis of neutropenia in peripheral blood can be sometimes difficult and parents and physicians are often afraid of severe infectious complications. The most frequent form of severe chronic neutropenia is idiopathic and autoimmune neutropenia. Both disorders typically develope in early childhood and achieve spontaneous remission before school age in 90% of children. The cause of both disorders is probably an autoimmune reaction against granulocytes. The disorders manifest with recurrent respiratory and/or skin infections which are not life threatening. There is no need for special therapy in most affected children. Congenital severe neutropenia (Kostmann’s syndrome) and cyclic neutropenia are rare causes of severe chronic neutropenia. Affected children suffer from severe recurrent infections and need long-term therapy with granulocyte colony stimulating factors. The authors demonstrate the case-reports of single disorders and discuss current diagnostic and therapeutic attitudes.

        Key words: severe chronic neutropenia, severe congenital neutropenia, idiopathic neutropenia, autoimmune neutropenia, cyclic neutropenia, differential diagnosis, therapy, vaccination, children