DIAGNOSIS AND MANAGEMENT OF CHRONIC NEUTROPENIA DURING CHILDHOOD

摘要: Chronic neutropenia is defined as an abnormally low absolute neutrophil count (ANC) lasting more than 6 months. Many attempts have been made to classify the different forms of chronic during childhood, but unfortunately a comprehensive classification system lacking. Pathophysiologically, can occur result decreased bone marrow production (e.g., Kostmann syndrome), failure release mature neutrophils from (myelokathexis), increased margination neutrophils, or destruction in peripheral circulation autoimmune hypersplenism) (Table 1). The risk for infection inversely proportional ANC. When ANC falls below 1000 per mm3, stomatitis, gingivitis, and cellulitis dominate clinical picture. More severe infections when 500 with perirectal abscesses, pneumonia, sepsis particularly common. S taphylococcus nu yeus most common isolated pathogen; however, severely affected patients, develop gram-negative enteric bacilli, including Klebsiella species, Escherichia coli, Pseudornonas neyuginosa. Uncommonly, fungal organisms also cause patients neutropenia. Considerable variability exists regarding susceptibility similar circulating counts. Monocytosis often occurs diverse types neutropenia, which