We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan. 1982 to Dec. 1991.
@ES The results were obtained as follows
@EN 1) The sex incidence revealed male predominance with the ratio 4:1.
2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe disease, 1 case as linfection associated hemophagocytic syndrome and 4 cases as malignant histiocytosis.
3) The mean age of symptom onset was 4 and 7/12 years in all disease group.
4) The common clinical symptoms and signs at dignosis were dyspnea, mass, pain on lower extremities and fever.
5) The most common organ involved among 9 organ systems was liver-spleen and the number of organ systems involved were 1 in 3 2 ases(30%), 3 in 2 cases(20%), 8 in 2 cases (20%) and 6, 7 and 10 organs in each 1 case.
6) The abnormal hematologic findings (Hb; 10g/dl and/or WBC; 4,000/mm3 and/or PLT; 100,000/mm³) were found in 6 cases.
7) Low serum albumin was found in 2 cases. Results of blood culture were Pseudomonas aeruginosae and Salmonella typhi in each 1 case.
8) The common findings on tissue biopsy were histiocytic proliferation and infiltration.
9) 4 patients of MH who recieved chemotherapy, a combination of adriamycin, vincristine, cyclophosphamide and prednisone were given in a total of four courses every 2 weeks ad induction therapy. When complete response was attained, a combination
of
adriamycin, vincristine, prednisone (AOP) and cyclophosphamide, vincristine, prednisone(COP) was administered alternately every 4 weeks as maintenance therapy for 6-64 months.
10) Among 4 patiens of MH who recieved chemotherapy, 1 patient was lost during induction chemotherapy, for 1 day.2 patients expired during induction chemotherapy, for 1 month, 1 patient expired during maintenance chemotherapty, for 8months,
Eosinophilic
granuloma cases (3) were recieved currettage and no recurrence. IAHS case due to typhoid fever was improved spontaneously.
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