Idiopathic hypereosinophilic syndrome terminating as disseminated T-cell lymphoma.
The authors describe a case of idiopathic hypereosinophilic syndrome (HES) terminated as a T-cell lymphoma in a 3-year-old girl. The clinical course was chronic and characterized by chronic eczema, persistent peripheral blood eosinophilia, organomegaly, interstitial lung change, and pericarditis. Postmortem examination demonstrated a disseminated T-cell lymphoma involving the inguinal lymph node, liver, lung, and kidney. The findings of the current case suggest a possibility that certain abnormalities in this case of idiopathic HES per se may have triggered the development of malignant lymphoma, and it may represent a transition of idiopathic HES into a T-cell lymphoma. Other possible sequences are discussed. The development of T-cell malignancy in idiopathic HES in a girl is quite an unusual presentation.
Kim
C J
CJ
Department of Pathology, College of Medicine, Seoul National University, Korea.
Park
S H
SH
Chi
J G
JG
D004485:Eczema / Q000150:complications; D004802:Eosinophilia / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000473:pathology; D010493:Pericarditis / Q000150:complications; D013577:Syndrome