소아 histiocytosis의 임상적 고찰 : 특히 항암요법 결과를 중심으로

Abstract

[한글]

Histiocytosis는 골수의 단핵구에서 유래한 조직구가 증식하는 질환으로 eosinophilic granuloma, Hand-Schuller-Christan disease, Letterer-Siwe disease, histiocytic medullary reticulosis, histiocytic lymphoms, monocytic leukemia 총괄하여야 하나 일반적으

로 histiocytic Iymphoma와 monocytic leukemia를 제외한 4가지의 질환을 지칭한다.

본 연구는 각종 histiocytosis의 임상적 특징, 치료 및 예후를 비교하고 분석 검토함으로써 진단과 치료에 도움이 되고자하는 목적으로 시행되었다.

관찰대상은 1975년 1월부터 1981년 12월까지 연세 의대 부속 세브란스 병원 및 연세 암센타에서 histiocytosis로 진단 및 치료를 받은 환자중 15세 이하의 소아연령 환자 34예를 대상으로 하였으며 각 질환별 임상적 특징을 조사하는 한편. 추적관찰이 가능하였던 2

6예에 대하여 치료결과를 분석하여 다음과 같은 결과를 얻었다.

각 질환별 빈도는 Hand-Schuller-Christian disease와 Letterer-Siwe disease가 각각 11예로 가장 많았고, eosinophilic granuloma가 7예, histiocytic medullary reticulosis가 5예 였으며, 발병연령은 histiocytic medullary reticulosis와 eosinophilic granuloma는 주로 유년기에 발병하는 반면에 Lettermer-Swice disease는 주로 1세 미만에서, Hand-Schuller-Christian disease는 그 중간 연령층에서 발병하였다.

임상증상은 eosinophilic granuloma와 Hand-Schu11er-Christitan disease는 주로 골병변이었는데 비하면 Letterer-Siwe disease와 histiocyic medullary reticulosis에서는 반복되는 고열, 복부장기의 종대, 출혈성 경향 및 체중감소등이 중요 증상이었으며 특히 Le

tterer-Siwe disease에서는 피부발진과 화농성이루가 특징적이었고, histiocytic medullary reticulosis에서는 거대한 임파선 종대와 출혈성 경향이 특징적이었다. 폐침윤은 Letterer-Siwe disease와 histiocytic medullary reticulosis의 각 3예에서 관찰되었다.

골병변의 호발부위는 eosinophilic granuloma의 경우에는 척추골. Hand-Schaller- Christian disease의 경우에는 두개골이었다.

검사소견상 Letterer-Siwe disease와 histiocytic medullary reticulosis의 대부분에소 조혈기능 이상과 간기능 이상 소견을 보였다.

치료결과를 보면 eosinophilic granuloma와 Hand-Schu11er -Christian diseaae에서는 수술요법 단독 혹은 수술요법과 방사선 치료후 추적되었던 13예중 6예는 완전히 회복되었고 1예만이 사망하였으며 나머지 6예는 계속적인 추적관찰이 불가능하였다. 이에 반하여 Letterer-Siwe disease와 histiocytic medullary

reticulosis의 경우는 모두 항암제 치료를 하였으나 치료결과가 매우 불량하여 추적된 15예중 Letterer-Siwe diseaae와 histiocytic medullary reticulosis의 각 2예에서 일시적호전을 보였으나 결국 모두 사망하였고 현재까지 불과하였다.

환자의 치료 결과는 발령연령이 낮을수록 불량하였으며(Lettere-Siwe disease :median 생존기간 1.5개월 + ) 피부, 간. 비장, 혈소판감소나 출혈성경향 및 귀침범이 있는 경우

에 불량하였고 (P<0.05), 특히 피부, 비장, 혈소판감소나 출혈성경향 밑 귀의 침법이 있을때 더욱 불량하였다 (p< 0.01).

Clinical Observation of Histiocytosie in Childhood-With Special Reference to

Chemetherapy-

Hee Jung Chung

Department of Medical Science The Graduate School, Yonsei University

(Directed by professor Byung Soo Klm, M.D.)

A study on Histiocytosis in childhood was conducted based on 34 cases diagnosed

at the Department of Pediatrics of the Severance Hospital and Yonsei Cancer Center

from January 1975 to December 1981. The results may be summarized as follows :

1. Among histiocytosis HSC and LSD were each present in eleven cases, E-G in

seven and HMR in five cases. HMR and E-G usually occured in older children and

adolescents, while LSD occured in children under one year and HSC between the ages

of the former and the latter.

2. The major clinical symptom of I-G and HSC was bone involvement, while those of

LSD and HHR were recurring high fever, visceral organ involvement, weigh loss and

bleeding tendency. In addition, skin rash and purulent otorrhea were seen

especially in LSD, and Large Iymphadenopathy and bleeding tendency were observed in

HHR.

3. The most common states of bone Involvement were vertebrae in E-G and the skull

in HSC.

4. In the laboratory findings, llver dysfunction and hematopoietic disorder were

seen in LSD and HHR. Lung infiltration was seen In three cases each of LSD and HMR.

5. I-G and HSC were treated either by surgery or by surgery In conjunotion with

radiation therapy. Among the 18 cases, 13 ceases were fellowed up. In six cases the

patients recovered, the observers lst track of six others during the courae of

follow up and the remaining one died.

Based upon their poor prognosis, the LSD and HMR cases were treated with

anticancer drugs, mainly with prednisone and vinblastin. Among the 16 cases, eleven

were fellowed up, but only one survives at present.

6. The Prognosis was Poorer the younger the patient was at the onset of the

disease, and the greater the incidence and the severity of visceral organ

involvement.

7. Skin, liver, spleen, thrombocytopenia &/or hemorhagic skin lesion and ear

Involvement were also correlated with poor prognosis (P < 0.05).

[영문]

A study on Histiocytosis in childhood was conducted based on 34 cases diagnosed at the Department of Pediatrics of the Severance Hospital and Yonsei Cancer Center from January 1975 to December 1981. The results may be summarized as follows :

1. Among histiocytosis HSC and LSD were each present in eleven cases, E-G in seven and HMR in five cases. HMR and E-G usually occured in older children and adolescents, while LSD occured in children under one year and HSC between the ages of the former and the latter.

2. The major clinical symptom of I-G and HSC was bone involvement, while those of LSD and HHR were recurring high fever, visceral organ involvement, weigh loss and bleeding tendency. In addition, skin rash and purulent otorrhea were seen

especially in LSD, and Large Iymphadenopathy and bleeding tendency were observed in HHR.

3. The most common states of bone Involvement were vertebrae in E-G and the skull in HSC.

4. In the laboratory findings, llver dysfunction and hematopoietic disorder were seen in LSD and HHR. Lung infiltration was seen In three cases each of LSD and HMR.

5. I-G and HSC were treated either by surgery or by surgery In conjunotion with radiation therapy. Among the 18 cases, 13 ceases were fellowed up. In six cases the patients recovered, the observers lst track of six others during the courae of

follow up and the remaining one died.

Based upon their poor prognosis, the LSD and HMR cases were treated with anticancer drugs, mainly with prednisone and vinblastin. Among the 16 cases, eleven were fellowed up, but only one survives at present.

6. The Prognosis was Poorer the younger the patient was at the onset of the disease, and the greater the incidence and the severity of visceral organ involvement.

7. Skin, liver, spleen, thrombocytopenia &/or hemorhagic skin lesion and ear Involvement were also correlated with poor prognosis (P < 0.05).