UNLABELLED
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.