Histiocytosis증후군의 임상적 고찰

Abstract

We made a clinical study of 17 cases of Histiocytosis ·syndrome who had been admitted to the Dept. of Ped. of S.N.D.H. from Jan. 1970 to Dec. 1980. Among 17 cases, 14 cases were classified as Lettercr-Siwe disease (Group I) and 3 cases as Hand-Schul lcr-Christian disease (Group n). The results obtained were as fo11O\\Ts. 1. The sex incidence revealed male predominance with the ratio 12 : 5. 2. The mean age of symptom onset was 10 months in Group f, whereas 1 G/12 yrs in Group n. The mean age at diagnosis was 1 2/12 years in Group I, whereas 3 3/12 years in Group n. 3. The common clinical signs at diagnosis were hepatomegaly, splenomegaly and lymphadenopathy. 4. The most common organ involved among 8 organ systems was liver, and the number of organ systems involved were G-6 in 9 cases (G3?c;). 3-4 in 7 cases (41%), and 7 in 1 case(G96). 5. The hematologic findings were as follows. 1) Anemia (Hb belowl0gm/dl) was found in 10 eases (59%). 2) The leukoeytes count was variable, but leukocytosis was found in 7 cases (41%). 3) Thrombocytopenia (platelet count below lOa, OOO/mm') were eommon (339;). The hemoglobin level and platelet count were inversely related to the extent of splenomegaly. G. Abnormal liver enzyme (GOT/GPT) level was found in 10 cases among 1G cases but the relationship of the extent of hepatomegaly and enzyme level was not found. 7. Bone changes on X-ray finding were revealed in 8 cases, of which the most common bone involved was skull C7G?o:. 8. Bone marrow findings on aspirate were abnormal in D among 11 cases performed, of which the most common was histiocytic infiltration in G cases (43%). 9. The most common finding on tissue biopsy was foamy histiocytic proliferation. 10. Four cases were followed up with therapy through O.P.D. for more than 2 years. The responses totreatment were variable in terms of symptoms.