한국인 위장관계 원발성 악성 임파종의 조직학적 및 면역세포학적 연구

Abstract

[한글]

위장관의 원발성 악성 임파종은 임파절이외의 조직에서 발생하는 임파종 중 가장 흔하며, 우리나라의 경우 전체 악성 임파종의 13.7내지 26.7%를 차지하는 것으로 보고되어 있다. 그러나 이들에 대한 체계적인 분류와 면역세포화학적 분류는 아직 문헌 보고된 것이 없다. 따라서 본 연구는 1976년 1월부터 1985년 12월까지 만10년간 연세대학교 의과대학 병리학교실, 영동세브란스병원 및 원주의과대학 부속 원주기독병원 병리과에서 위장관계 원발성 악성 임파종으로 진단된 71예를 Working Formulation, Rappaport와 Lukes-Collins

분류법에 의해 조직학적 재분류를 시행하고, 그 분포와 육안적 및 임상적 특징을 조사 분석하였으며, 면역글로불린 kappa와 lambda light chain, IgG.A.M heavy chain, lysozyme과 α^^1 -antichymotrypsin에 대한 면역과산화효소염색을 시행하여 다음과 같은 결론을 얻었다.

1. 총 71예의 악성임파종 중 68예가 비호지킨씨 임파종이며, 이중 57예는 외과적으로 절제된 조직이었고, 호지킨씨 임파종은 3예였다.

2. 악성 임파종의 발생부위는 위장이 28예(2예는 호지킨씨 임파종), 소장이 22예, 회맹부가 15예(1예는 호지킨씨 임파종), 대장이 6예였다.

3. 악성 임파종의 진단 당시 평균 연령은 45세로 위치별로는 소장이 35세, 대장이 57세이었다. 성별 분포는 1.9:1로 남자에 약간 호발하였다.

4. 입원 당시의 주소는 복부 동통이 가장 많았고, 그 외에 종괴 촉지, 토혈, 혈변, 소화불량, 설사, 체중 감소, 변비 등의 순이었으며, 병력기간은 수시간부터 12개월까지 광범위하지만 비교적 고르게 분포하였다.

5. 악성 임파종의 육안적 형태는 궤양형(53.5%)이 가장 많았으며, 용종형, 다결절형, 미만성 비후형의 순이었고, 종양의 크기는 대부분 10cm미만이었다(79.3%).

임파절 침범의 빈도는 종양의 침습깊이가 장막에 국한되었던 경우 44.4%에 비하여, 장막을 뚫은 경우 82.6%로서 의의있게 임파절 침범의 빈도가 높았다.

6. 비호지킨씨 임파종 68예를 Working Formulation에 의해 분류한 결과 intermediate grade가 50예로 가장 많았고, low grade가 5예, high grade가 13예였다. Rappaport분류법에 따라서는 전 예가 미만성이었고, 이 중 조직구형이 28예로 가장 많았다.

Lukes-Collins분류법에 의해서는 60예가 형태학적으로 B세포형이었고, 3예는 T세포형이었으며, 4예가 순수 조직구형(genuine histiocytic type)이었고, 1예는 분류 불가능한 임파종이었다.

7. 총 55예에서 면역과산화효소염색을 시행하여 B세포 임파종으로 생각된 47예중 3 1예(65.9%)가 B세포 표면표적에 양성반응을 보였고, 이 중 23예(48.9%)에서 monoclonality를 나타냈으며 ,IgG를 분비하는 형이 많았다. 4예의 순수 조직구형의 종양세포는 lysozyme보다 α^^1 -antichymotrypsin에 민감하게 양성반응을 나타냈다. 순수 조직구형을 제외한 비호지킨씨 임파종 종괴내의 양성 조직구의 침윤 정도는 다양하게 나타났으며 환자의 추후관찰도 불가능하여 예후와의 특정한 결과는 얻을 수 없었다.

[영문]

Gatrointestinal(GI) tract is the most frequent site of extranodal malignant lymphoma, and primary GI lymphoma has been reported up to 13.7-26.7% of all lymphomas in Korean patients. However comprehensive studies on the histopathological and immunocytochemical features of primary gastrointestinal(GI) lymphomas are not reported yet in Korea. The present study is aimed to investigate the clinical and pathological features of GI lymphomas and the inmunocytochemical characteristics.

The material consisted of 71 cases of primary GI lymphomas examined at the Department of Pathology, Yonsei University College of Medicine, Youngdong Severance Hospital and Yonsei University Wonju College of Medicine from January 1976 to December 1985. Clinical and pathological parameters assessed were age, sex, presenting symptoms, duration of the disease, size and shape of the mass, and correlation between the depth of the tumor invasion and lymph node involvement. Eaeh case was classified according to Working Formulation, Rappaport and Lukes-Collins classifications. Immunocytochemical study was done using kappa,

lambda light chains and IgG, IgA, IgM heavy chains for B cell markers and lysozyme and alpha-1-anti-chymotrypsin for histiocytic markers.

Results obtained were as follows :

1) Among 71 cases of primary gastrointestinal lymphomas, 68cases were non-Hodgkin's lymphoma and 3 were Hodskin's lymphoma. Surgical resection was done in 58 patients and surgical biopsy in 13. Pseudolymphoma was found in 8 cases.

2) The primary sites of the tumors were stomach, small intestine, ileocecal area and large intestine in a descending order of frequency.

3) The mean age at the time of diagnosis was 45 years. The tumor of the small intestine was generally detected in the younger age and that of the large intestine in the older age. The overall male to female ratio was 1.9:1, showing a slight male predominance.

4) Abdominal pain was the most common presenting symptom irrespective of the sites of the tumor. The others were palpable mass, hematemesis, melena, indigestion, weight loss, diarrhea, constipation and nausea. Duration of the symptoms were evenly distributed from hours to 12 months.

5) The size of the tumors was less than 10 on in the largest dimension in most of the cases (79.3%). The gross types of the GI lymphomas were ulcerative (27.6%) or ulcerofungating diffuse thickening in a descending order of frequency. The rate of Lymph node involvement was 44.4% in tumors confined to the serosa, while 82.6% in tumors with serosal penetrations.

6) Classification of 68 cases of non-Hodgkin's lymphomas according to Working Formulation revealed 50 cases of intermediate grade, 5 cases of low grade(all small lymphocytic plasmacytoid types) and 13 oases of high grade(5 large cell immunoblastic, 3 lymphoblastic, 4 true histiocytic and 1 unclassified types). According to Rappaport classification all were diffuse types, among which diffuse histiocytic type was most common. According to Lukes-Collins classification, 60 cases were B cell types, 3 cases T cell types and 4 cases genuine histiooytic types. Most of the gastric and intestinal lymphomas belonged to the follicular center cell(small and large cleaved, large cleaved) types.

7) Immunoperoxidase stains were done in 55 cases of malignant lymphomas. Thirty one(65.9%) of 47 B cell lymphomas revealed a positivity for B cell markers. Twenty three(48.9%) of them showed a monoclonality, in which kappa/IgG pattern was most frequently demonstrated. Four cases of true histiocytic lymphomas were positive for alpha-1-antichymotrypsin and for lysozyme, the former was seemed to be a more sensitive marker for histiocytic differentiation . Pseudolymphomas revealed a polyclonal pattern. An attempt was made to evaluate the significante of the decree

of reactive histiocytic infiltrates within the lymphoma in relation to the prognosis, but correlation could not be made because of the limitted cases.