A pseudoleukemic blood differentiation in a 13-year-old child: an extraordinary presentation of Churg-Strauss syndrome

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of the small- and medium-size vessels. It is mostly seen in elderly patients presenting as de novo asthma, eosinophilia, and vasculitic organ involvement. In childhood, CSS is extremely rare. The course of pediatric CSS is usually severe and...

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Bibliographic Details
Published in:Clinical rheumatology 2013-03, Vol.32 (Suppl 1), p.7-9
Main Authors: Mutsaers, E. R., Witteveen, R., van den Bosch-Ruis, W., Kuijpers, T. W., van Houten, M. A., van den Berg, J. M.
Format: Article
Language:English
Subjects:
Adolescent
Case Report
Churg-Strauss Syndrome - blood
Churg-Strauss Syndrome - diagnosis
Churg-Strauss Syndrome - drug therapy
Diagnosis, Differential
Eosinophilia - blood
Eosinophilia - diagnosis
Eosinophils - pathology
Female
Glucocorticoids - therapeutic use
Humans
Hypereosinophilic Syndrome - diagnosis
Medicine
Medicine & Public Health
Prednisolone - therapeutic use
Rheumatology
Treatment Outcome
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Summary:Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of the small- and medium-size vessels. It is mostly seen in elderly patients presenting as de novo asthma, eosinophilia, and vasculitic organ involvement. In childhood, CSS is extremely rare. The course of pediatric CSS is usually severe and often lethal. We present a case of a 13-year-old girl with a short history of asthma, marked eosinophilia, and multiorgan involvement. The extremely high level of blood eosinophilic granulocytes (51.6 × 10 9 /L) prompted a workup for eosinophilic leukemia before the diagnosis CSS could be made. Subsequently, the disease was successfully treated. This case report shows a classical case of childhood CSS, remarkable because of the presence of extreme hypereosinophilia. It underlines the importance of CSS as a life-threatening cause of hypereosinophilia in children.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-009-1265-1