javascript:void(0)2nd case progression
Without improvement of the clinical state, the boy still required oxygen
and suffered from spiking fever throughout the day, with extensive
sweating at night. Repeated CBC showed persistent leukocytosis (23.0
× 10⁹/L) and eosinophilia, and a new increase in CRP (106 mg/L).
As part of vasculitis work-up, the tests for autoantibodies (antinuclear
antibodies, antineutrophil cytoplasmic antibodies, anti-saccharomyces
cerevisiae antibodies) and serum calprotectin were normal, and screening
for renal involvement did not detect any urine abnormalities, such as
proteinuria or hematuria. Due to the lymphadenopathy on CT scan, the
Mantoux test and the Interferon Gamma Release Assay (IGRA) test were
performed to exclude tuberculosis. Furthermore, the Angiotensin
Converting Enzyme (ACE) levels and Chitotriosidase levels for the
diagnosis of sarcoidosis were normal for age. Lung function tests
uncovered a severe reduction in lung vital capacity with 30% of the
predicted value (Figure 3a). At this point, bronchoscopy was scheduled
to visualize the airways, obtain samples by bronchoalveolar lavage (BAL)
and to perform endobronchial biopsy for detailed tissue analysis.
Simultaneously, a skin biopsy from one of the lesions on the right arm
was planned.
On bronchoscopy, the macroscopic airway anatomy was described as normal,
with the airway mucosa showing signs of discrete inflammatory changes
and viscous secretions throughout the bronchial tree (Figure 3b). The
differential leukocyte count from BAL shows 10% eosinophils
(physiological value: <1%), 6% lymphocytes (physiological
value: <10%) and 8% neutrophils (physiological value:
revealed high number of cytomegalovirus (CMV) copies (38,000
IU/ml). Also, an elevated Candida species antigen count (157
pg/mL, physiological values: < 62.4 pg/mL), yet normal value
of the Aspergillus galactomannan (0.12, physiological index
Elevated BAL levels of CMV copies with normal values in blood were
highly suggestive of CMV lung infection, possibly CMV pneumonia.
Serologically, there were elevated IgG levels for CMV (CMV IgG 71 U/ml,
reference range 0 – 13 U/ml) but normal IgM levels for CMV (CMV IgM 14
U/ml, reference range 0 – 17 U/ml), which is not suggestive of a recent
CMV primoinfection. Valganciclovir therapy was initiated at dose of 5
mg/kg/day.