[Chronic neutropenia - experience from the Department of Immunology, Children's Memorial Health Institute].

Abstract

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.

• Authors: Maja Klaudel-Dreszler, Barbara Pietrucha, Hanna Skopczynska, Malgorzata Pac, Magdalena Kurenko-Deptuch, Edyta Heropolitanska-Pliszka, Beata Wolska-Kusnierz, Krystyna Maslanka, Ewa Bernatowska
• Journal: Medycyna wieku rozwojowego (Med Wieku Rozwoj) 2007 Apr-Jun Vol. 11 Issue 2 Pt 1 Pg. 145-52 Poland
• Vernacular Title: Przewlekła neutropenia - Doświadczena Własne Oddzialu Immunologii Instytutu ,, Pomnik -Centrum Zdrowia Dziecka" w Warszawie z Lat 1985-2006.
• PMID: 17625284 (Publication Type: Journal Article)

Chemical References

• Anti-Bacterial Agents
• Autoantibodies
• Recombinant Proteins
• Granulocyte Colony-Stimulating Factor

Topics

• Adolescent
• Anti-Bacterial Agents (therapeutic use)
• Autoantibodies (immunology)
• Autoimmune Diseases (diagnosis, immunology, therapy)
• Bone Marrow Transplantation
• Child
• Child, Preschool
• Chronic Disease
• Female
• Granulocyte Colony-Stimulating Factor (therapeutic use)
• Hospitals
• Humans
• Infant
• Infections (diagnosis, immunology, therapy)
• Leukemia (diagnosis, immunology, therapy)
• Leukocyte Count
• Male
• Neutropenia (diagnosis, immunology, therapy)
• Neutrophils (immunology)
• Poland
• Recombinant Proteins
• Retrospective Studies
• Treatment Failure
• Treatment Outcome