Severe Chronic Neutropenia

Overview

Severe chronic neutropenia includes a heterogeneous group of rare diseases in childhood. Differential diagnosisof neutropenia in peripheral blood can be sometimes difficult and parents and physicians are often afraid of severeinfectious complications. The most frequent form of severe chronic neutropenia is idiopathic and autoimmuneneutropenia. Both disorders typically develope in early childhood and achieve spontaneous remission before schoolage in 90% of children. The cause of both disorders is probably an autoimmune reaction against granulocytes. Thedisorders manifest with recurrent respiratory and/or skin infections which are not life threatening. There is noneed for special therapy in most affected children. Congenital severe neutropenia (Kostmann’s syndrome) andcyclic neutropenia are rare causes of severe chronic neutropenia. Affected children suffer from severe recurrentinfections and need long-term therapy with granulocyte colony stimulating factors. The authors demonstrate thecase-reports of single disorders and discuss current diagnostic and therapeutic attitudes.

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