[Chronic neutropenia - experience from the Department of Immunology, Children's Memorial Health Institute].
Chronic neutropenia (CN) is defined by an absolute neutrophil count (ANC) below 1500/ul, lasting at least 6 months.


OBJECTIVE
clinical course and treatment of children afflicted with CN was analysed.


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.
Klaudel-Dreszler
Maja
M
Oddział Immunologii, Klinika Gastroenterologii, Hepatologii i Immunologii, Instytut Pomnik Centrum Zdrowia Dziecka, Al. Dzieci Polskich 20, 04-730 Warszawa, Poland.
Pietrucha
Barbara
B
Skopczynska
Hanna
H
Pac
Malgorzata
M
Kurenko-Deptuch
Magdalena
M
Heropolitanska-Pliszka
Edyta
E
Wolska-Kusnierz
Beata
B
Maslanka
Krystyna
K
Bernatowska
Ewa
E
D000293:Adolescent; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006761:Hospitals; D006801:Humans; D007223:Infant; D007239:Infections / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D007938:Leukemia / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D007958:Leukocyte Count; D008297:Male; D009503:Neutropenia / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D009504:Neutrophils / Q000276:immunology*; D011044:Poland; D011994:Recombinant Proteins; D012189:Retrospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents; D001323:Autoantibodies; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor