Page 822 - Hematology_ Basic Principles and Practice ( PDFDrive )

708 Part VI Non-Malignant Leukocytes

Management of Infections
The management of infections in patients with primary neutrophil dys- of inflammatory response may take many weeks, usually there is
function syndromes is quite different than in the normal population, and some clear change in the ESR within 1 week. If there is worsening
for the most part different from patients with neutropenias. or no clear response, then an antifungal can be added and the
• Patients tend to present with relatively low fevers and chronic ESR monitored in the same fashion. Return of an elevated ESR can
inflammatory processes associated with marked elevation of ESR be a sign of development of organism resistance.
and CRP. Unless they have untreated abscesses or inflammatory • If a patient with CGD is particularly ill appearing or febrile, it is
masses, they tend not to present with frank sepsis and positive important to make sure that B. cepacia complex bacteria are
blood cultures. covered.
• The frequency of infections decreases somewhat with age in • There is no fixed duration of therapy for any infections in these
children as their normal T-cell and B-cell–mediated immunity patients. If the infections are not completely extinguished, they will
develops. return and will contribute to development of chronic pulmonary
• Although one should always attempt to obtain culture proof of and hepatic fibrosis. Parenteral antibiotics or antibiotics that can
an infection, more often than not, it is not possible to identify an deliver very high tissue levels should be continued significantly past
organism, and it is necessary to treat empirically. normalization of the ESR and disappearance of any radiographic
• Because these patients tend to develop deep-seated tissue evidence of deep tissue infection. This can take many months for
infections, the ESR can be of great value even though it is some pneumonias and liver abscesses.
quite nonspecific. Elevation in the ESR suggests deep tissue • Short pulses of steroids (4–6 days) can be lifesaving, particularly
inflammation; CRP is more acute and suggests monocyte activation. for pulmonary infections in young children with CGD. They reduce
Persistent significant elevation of the ESR (>15–20 mm/hour) even airway inflammation and promote drainage.
in the absence of fever or other symptoms may warrant radiologic • Young children are susceptible to infections with routine childhood
search for deep-seated infection. viruses and infections, and tend to do well with standard
• The authors advocate an “antibiotic sensitivity by ESR response” therapeutic approaches and courses of treatment that are two- to
approach to empiric therapy in stable patients. One can start three-times longer than the usual recommended course. Again,
at parenteral anti-Staphylococcus and gram-negative therapy, monitoring with the ESR can be a guide.
and monitor the ESR daily. A monotonic decrease in the ESR All standard childhood immunizations and influenza vaccinations are
within several days that is clear-cut suggests the process is strongly recommended. Prophylactic antibiotics may be appropriate (see
sensitive to the antibiotic selected. Although complete resolution text).

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