J Korean Pediatr Soc.
1998 Nov;41(11):1575-1582.
A Clinical Study of Histiocytosis Syndrome
- Affiliations
-
- 1Department of Pediatrics, College of Medicine, Hallym University, Seoul, Korea.
Abstract
-
PURPOSE: We conducted this study to find factors related to clinical aspects of patients with histiocytosis syndrome.
METHODS
We performed a retrospective clinical study with chart review in twenty patients with histiocytosis syndrome from January 1987 to June 1997.
RESULTS
The sex incidence revealed female predominance with a ratio of 1.2 : 1. Of twenty cases, fifteen were classified as class l, three cases as class ll, and two cases as class lll. The mean age of symptom onset was 7 and 9/12 years. Common clinical symptoms and signs at diagnosis were fever, mass, headache, pain on lower extremities, skin rashes and weight loss. The most common involved organ was the skeleton. Abnormal hematologic findings (hemoglobin;<10g/dL and/or white blood cell<4,000/mm3 and/or platelet<150,000/mm3) were found in four cases. Common findings on tissue biopsies were histiocytic proliferation and infiltration. Two patients with malignant histiocytosis (MH) and one patient of eosinophilic granuloma (EG) received chemotherapy. Among three patients who started chemotherapy, one patient was discharged freely, one patient died because of recurrence, and one patient had been treated with a maintenance regimen until now.
CONCLUSION
The survival rate depends mainly on onset age, severity, Lahey's organ dysfunction score, histologic findings and sites of involved organs. But most patients had nonspecific symptoms before diagnosis. Therefore, early recognition and aggressive medical treatment before development of serious symptoms improved the prognosis.
