소아과 영역에 있어서의 골수섬유화에 관한 연구

Abstract

[한글]골수검사는 혈액학 영역에서 여러 질환의 진단과 조혈장기의 기능상태 및 질환의 치유판정에 널리 이용되고 있다. 이중 골수세망 섬유의 정량분석은 각종 질환 경과중에 골수경화의 정도를 볼 수 있는 유용한 방법이다.

본 연구는 소아연령츠의 골수섬유화의 정도를 병리조직학적으로 분류하고 임상상에 대한 고찰을 하여 다음과 같은 결과를 얻었다.

1. 연구대상의 질환은 급성임파구성백혈병 30예, 재생불량성빈혈 14예, 기타 빈혈증 11예, 급성골수성백혈병 9예, 신경아세포종 7예, 특발성혈소판감소성자반증 6예, 악성임파종 5예, 만성골수성백혈병 3예, 결핵 3예, 급성골수단구성백혈병 2예, 간염 2예 그리고 기타 15예였다.

2. 이차성 골수섬유증을 유발한다고 알려진 급·만성 백혈병, 전이암종을 제외한 총 54예의 골수섬유화 정도는 "N" 61.1%, "+1" 29.6% 및 "+2" 9.3%로서 전예가 "+2" 이하였으며 이것이 정상적 골수섬유화의 상한선으로 사료된다.

3. 이차성 골수섬유증을 보인 예는 32예 였으며 그중 급성임파구성백혈병이 23예(71.9%), 만성골수성백혈병 3예, 신경아세포종 3예 그리고 기타 3예였다.

4. 건성골수천자 11예는 전 예에서 골수섬유증을 보였으며 그 정도는 모두 "+4"에 속했으며 전체 "+4" 섬유증의 61.1%를 차지하였다.

5. 골수세포충실도와 섬유화 정도를 비교한 바 충실도가 증가된 경우에 섬유화가 53.1%, 정상인 경우에 6.7%, 감소된 경우에 17.9%로 세포충실도가 높을수록 섬유화가 현저하였다.

Quantitative Studies of Myelofibrosis in Infancy and Childhood

Jei Hoon Yoo, MD

Department of Medical Science The Graduate School, Yonsei University

(Directed by Professor Yoo Bock Lee, MD)

Bone marrow aspiration and biopsy are employed in the diagnosis of various

hematological diseases, evaluation of treatments and hematopoietic activity.

Quantitative evaluation of bone marrow reticulin is a useful procedure for

determining the degree of bone marrow sclerosis in various disease processes,

particularly those of myeloproliferative type. These disorders often terminate as

myelofibrosis and it is always serious and fatal.

The purpose of this study is to quantitate bone marrow reticulin and illustrate

clinical features of myelofibrosis in 107 cases of bone marrow biopsies from

various types of diseases. Degree of myelofibrosis was estimated by the criteria of

Bauermeister(1971).

The results are as follows:

1. Bone marrow studies were done in following diseases;

Acute lymphocytic leukemia (30 cases), aplastic anemia(14 cases), other

anemias(11 cases), acute myeloblastic leukemia (9 cases), neuroblastoma (7 cases),

idiopathic thrombocytopenic purpura (6 cases), malignant lymphoma (5 cases),

chronic myelocytic leukemia (3 cases), tuberculosis (3 cases), acute

myelomonoblastic leukemia(2 cases), hepatitis (2 cases) and others (15 cases).

2. Myelofibrosis above "+3" was observed in 33 cases. The agnogenic type was 1

and the secondary type 32. The associated diseases in the secondary type were acute

lymphocytic leukemia (23 cases), chronic myelocytic leukemia (3 cases),

neuroblastoma (3 cases) and others (3 cases).

4. Dry tap was encountered in 11 cases (10.3%) and all of them were scored to

"+4", which comprised 61.1% of total cases of "+4+ score.

5. Myelofibrosis above "+3" was seen in 53.1% of hypocellular, 6.7% of

normocellular and 17.9% of hypocelluar marrow, indicating close relationship

between hypocellularity and myelofibrosis.

[영문]

Bone marrow aspiration and biopsy are employed in the diagnosis of various hematological diseases, evaluation of treatments and hematopoietic activity. Quantitative evaluation of bone marrow reticulin is a useful procedure for determining the degree of bone marrow sclerosis in various disease processes, particularly those of myeloproliferative type. These disorders often terminate as myelofibrosis and it is always serious and fatal.

The purpose of this study is to quantitate bone marrow reticulin and illustrate clinical features of myelofibrosis in 107 cases of bone marrow biopsies from various types of diseases. Degree of myelofibrosis was estimated by the criteria of Bauermeister(1971).

The results are as follows:

1. Bone marrow studies were done in following diseases;

Acute lymphocytic leukemia (30 cases), aplastic anemia(14 cases), other anemias(11 cases), acute myeloblastic leukemia (9 cases), neuroblastoma (7 cases), idiopathic thrombocytopenic purpura (6 cases), malignant lymphoma (5 cases), chronic myelocytic leukemia (3 cases), tuberculosis (3 cases), acute

myelomonoblastic leukemia(2 cases), hepatitis (2 cases) and others (15 cases).

2. Myelofibrosis above "+3" was observed in 33 cases. The agnogenic type was 1 and the secondary type 32. The associated diseases in the secondary type were acute lymphocytic leukemia (23 cases), chronic myelocytic leukemia (3 cases), neuroblastoma (3 cases) and others (3 cases).

4. Dry tap was encountered in 11 cases (10.3%) and all of them were scored to "+4", which comprised 61.1% of total cases of "+4+ score.

5. Myelofibrosis above "+3" was seen in 53.1% of hypocellular, 6.7% of normocellular and 17.9% of hypocelluar marrow, indicating close relationship between hypocellularity and myelofibrosis.