S-Space College of Medicine/School of Medicine (의과대학/대학원) Dept. of Medicine (의학과) The Seoul Journal of Medicine The Seoul Journal of Medicine Vol. 23 No.1 (1982)
Histiocytosis증후군의 임상적 고찰
A Clinical Study on Histiocytosis Syndrome
- 고영열; 강순웅; 이종국; 최정연; 안효섭; 홍창의
- Issue Date
- 서울대학교 의과대학
- Seoul J Med, Vol.23 No.1, pp. 76-89
- 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
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
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
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.