N. Li et al. / Case Reports in Clinical Medicine 2 (2013) 35 1-35 7
356
kine/chemokine levels in ECD patients compared with
controls, with principal component analysis identifying
an ECD-specific signature based on increased expression
of IFN-α, IL-1/IL-1RA, IL-6, IL-12, monocyte chemo-
tactic protein-1, and decreased expression of IL-4 and
IL-7. The authors conclude that the systemic immune
Th-1-oriented perturbation associated with this condition,
and provides clues for the choice of more focused
therapeutic agents.
4. CONCLUSION
ECD is a rare form of non-Langerhans’ cell histiocy-
tosis. ECD appears to be the pathognomonic clinical,
radiographic and histological features. Diagnosis of ECD
is based on the identification in tissue biopsy of histio-
cytes, which are typically foamy and immunostain for
CD68+ CD1a−. Central nervous system involvement is a
major prognostic factor in ECD. Because the incidence
of ECD is low, the data from literature remains limited.
More case data for this histiocytosis need therefore be
obtained for a better understanding of its characteristics
and an exploration of appropriate therapy.
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