Chronic neutropenia (CN) is defined by an absolute neutrophil count (ANC) below 1500/ul, lasting at least 6 months.
Aim: clinical course and treatment of children afflicted with CN was analysed.
Material and methods: we present 60 children treated in our department due to CN. The diagnosis was based on: bone marrow smears, ANC, immunologic investigation.
Results: we established the diagnosis of: Kostmann disease (KD), cyclic neutropenia (CyN), hyperIgM syndrome (HIGM), Shwachman-Diamond syndrome (SDS), severe chronic neutropenia (SCN) and chronic benign neutropenia (CBN) in: 4, 2, 2, 1, 21 and 20 children respectively. Due to positive results of tests: MAIGA, GIFT or GAT autoimmune neutropenia of infancy (AIN) was confirmed in 7 children. In 3 infants neutropenia was connected with HCMV- infection and Gancyclovir therapy. RHuG-CSF treatment was implemented in 14 and effective in 13 patients. A girl, suffering from KD, during rHuG-CSF therapy, developed chronic myeloblasts leucaemia and died. A boy, with the same diagnosis, underwent bone marrow transplantation from related donor but died from invasive pulmonary aspergillosis. Antibacterial prophylaxis was necessary in 29 children. We used Amoxicillin or Trimethoprim/Sulfametoxazole, obtaining decrease of frequency and severity of infections. During observation period all children suffered from upper respiratory tract infections, 19 had chronic gingivitis. Severe infections- bacterial pneumonia, sepsis, severe varicella and measles were observed in 30, 5, 2 and 1 patient respectively. A teenager, affected with SCN, died due to fulminant Clostridium perfringens infection.
Conclusions: 1. RHuG-CSF therapy is essential in children with KD and SCN (when accompanied by severe infections). 2. AIN proved to be a mild condition, although ANC decreased below 500. In this entity rHuG-CSF is recommended during severe infections and before surgery. 3. Antibiotic prophylaxis is recommended for children with: KD, CyN, GSD1b, CN in 1st year of life, HIGM; in other cases it is considered individually.