| A clinical study on histiocytosis X. |
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Sang Oh Na, Joong Gon Kim, Hyo Seop Ahn |
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Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea |
| 조직구 증식 증후군의 임상적 고찰 |
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나상오, 김중곤, 안효섭 |
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서울대학교 의과대학 소아과학교실 |
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Received: 30 August 1989 • Accepted: 30 August 1989 |
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| Abstract |
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We made a clinical study on 31 cases of histiocytosis X who had been admitted to the Dept, of Ped.
of S.N.U.C.H. from Jan. 1981 to Dec. 1987.
Among 31 cases, 28 patients received chemotherapy to include prednisolone, vinblastine, methotrex-
ate and cyclophosphamide as induction therapy, and 6-mercaptopurine, methotrexate and cyclophos-
phamide as maintenance therapy.
The results obtained were as follows.
1) The sex incidence revealed male predominance with the ratio 1.6:1.
2) Among 31 cases, 13 cases were classified as eosinophilic granuloma, 15 cases as Hand-Schtiller-
Christian disease and 3 cases as Letterer-Siwe disease.
3) The mean age of symptom onset was 4 10/12 years in all disease group, 6 3/12 years in
eosinophilic granuloma, 3 7/12 years in Hand-Schiiller-Christian disease, and 1 10/12 years in
Letterer-Siwe disease.
4) The common clinical symptoms and signs at diagnosis were mass, skin lesion, fever, and
palpable lymph node.
5) The most frequent clinical impression without invasive study was osteomyelitis.
6) The most common organ involved among 9 organ systems was bone, and the number of organ
systems involved were 1 in 13 cases (42%), 2 in 6 cases, 4 in 5 cases, 5 in 2 cases, and 6 and 9 organs
in each 1 case.
7) The abnormal hematologic findings (low hemoglobin level and/or low total leukocyte count
and/or low platelet count) were found in 11 cases.
8) The abnormal liver function (elevated liver enzyme and/or low serum protein and/or low serum
albumin and/or elevated serum bilirubin) was found in 3 cases.
9) The common findings on tissue biopsy were histiocytic proliferation and infiltration, which were
found in all 21 cases of bone biopsy.
10) Among 28 patients who recieved chemotherapy, 2 patients expired during induction chemother-
apy, 2 patients were lost during induction chemotherapy, 3 patients were lost during maintenance
chemotherapy, 3 patients have been undergone maintenance chemotherapy, 15 pateints are alive
without evidence of disease, and the recurrence was found in remaining 3 patients.
11. The event free survival rate after initiating chemotherapy at 1 year, 2 years, and 3 years was
88%, 84%, 70% respectively. |
| Key Words:
Histiocytosis X, Chemotherapy, Survival rate |
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