성인 만성 면역성 혈소판감소성 자반증에서 복합화학요법의 효과

Abstract

[한글]

면역성 혈소판감소성 자반증은 혈소판에 대한 자가항체에 의해 혈소판감소증이 유발되는 질환이다. 성인의 경우 대개 만성적이며, 자연적인 회복은 거의 일어나지 않고 재발이 잘 된다. 일반적인 치료로서 부신피질호르몬제 투여 및 비절제술이 시행된다. 75%의 환

자에서 부신피질호르몬 및 비절제술로 장기적인 반응이 유도되며, 나머지 15-25%의 환자는 혈소판감소증이 지속되는 불응성 환자들이다. 불응성 면역성 혈소판감소성 자반증은 치명적인 출혈의 합병증을 일으킬 수 있으므로 안전한 수준의 혈소판수를 유지하기 위해

다른 치료를 필요로 하게 된다. 이를 위해 vinca alkaloids, danazol, cyclophosphamide, azathioprine 흑은 정주용 감마글로불린 등의 다양한 치료가 시도되나 일부 환자들은 여러 치료방법에 효과를 보이지 않는다. 최근 이러한 불응성 환자에서 복합화학요법이 시도

되고, 그 치료 효과가 보고된 바 있다.

본 연구자는 성인 만성 면역성 혈소판감소성 자반증으로 진단 받고 복합화학요법을 받은 환자의 치료 효과를 검토하고자 하였다. 성인 만성 면역성 혈소판감소성 자반증으로 진단받고 복합화학요법을 시행받은 15예의 초진시 연령은 평균 32.7(범위 20-52)세 였다.

대상 환자중 6예는 불응성 예 이었고, 9예는 부신피질호르몬 등의 약제에 대한 부작용으로 계속투약하기 어렵거나, 비절제술에 대한 거부로 수술이 바로 시행하지 못한 예였다.

불응성 예중 1예에서는 비절제술 전에도 복합화학요법이 시행되었다. 대상 환자 15예에서 총 31회, 평균 2.1(1-6)회의 복합화학요법이 시도되었으며, 21회에서는 CVP(cyclophosphamide, vincristine, prednisolone), 10회에서는 CMOPP(cyclophosphamide, vincristine,

prednisolone, procarbazine)이 투여되었다.

복합화학요법에 대한 반응은 다음과 같았다.

1. 총 31회의 치료중 지속적인 완전반응이 2회(6.5%), 일시적인 완전반응이 11회(35.5%), 부분반응이 10회(32.3%)에서 관찰되었고, 8회(25.8%)에서는 무반응을 보였다.

2. 복합화학요법의 종류나 비절제술 여부에 따른 전체 반응률의 유의한 차이는 관찰되지 않았다. 그러나 완전반응률은 비절제술을 받은 군에서 높은 경향을 나타내었다.

3. 복합화학요법에 따른 심각한 합병증은 관찰되지 않았다.

결론적으로 만성 면역성 혈소판감소성 자반증에서 비절제술후 반응이 없거나 제발한 불응성 예, 그리고 부신피질호르몬 및 이차적인 약제에 반응이 없으나 비절제술을 바로 시행하기 어려운 환자에서 복합화학요법이 비교적 안전하게 투여될 수 있는 치료로 사료된다.

Therapeutic effect of combination chemotherapy in adult chronic immune

thrombocytopenic pupura

So Young Chong

Department of Medicine The Graduate School, Yonsei University

(Directed by Professor Yun Woong Ko)

Immune thrombocytopenic purpura(ITP) is a disorder in which autoantibodies

against platelet membrane proteins cause platelet destruction. In adults, ITP

usually takes chronic courses with rare spontaneous remission and frequently

relapse after therapeutic response. Overall 75% of patients attain a prolonged

response to conventional treatment with glucocorticoids and/or splenectomy, while

15-25% of patients do not respond to these treatments. Many patients with

refractoriness can not maintain safe levels of platelet count for adequate

hemostasis, and they require further therapy with drugs such as vinca alkaloids,

danazol, cyclophosphamide, azathioprine or intravenous gammaglobulin. Some patients

do not respond to all therapies. A beneficial effect of combination chemotherapy in

refractory ITP has been reported, but the therapeutic role of combination

chemotherapy is not fully appreciated. We performed combination chemotherapy to

treat 15 patients with ITP.

An average rumber of 4.5(2-11) previous therapies, including splenectomy(6cases),

had been unsuccessful or intolerent. One case underwent combination chemotherapy

before and after splenectomy The mean age of patients at diagnosis of ITP was

32.7(20-52)years. Chemotherapy regimens were CVP(cyclophosphamide, vincristine,

pednisolone) in 21 courses and CMOPP(cyclophosphamide, vincristhe, pednisolone,

procarbazine) in 10 courses. Therapeutic response to combination chemotherapy was

as follows:

1. Among 31 courses of therapy, continuous complete response(CCR),transient

complete response(TCR) and partial response(PR) were observed in 2(6.5%), 11(35.5%)

and 10(32.3%) courses, respectively, There was noresponse(NR) in 8(25.8%) courses.

2. Chemotherapy regimens or status of splenectomy had no significant influence on

response rates. However, there was a tendency towards higher complete remission

rate in splenectomized patients.

3. There was no serious side effects of combination chemotherapy.

In conclusion, combination chemotherapy may have a beneficial role in the

management of refractory cases or selected cases before splenectomy with acceptable

side effects.

[영문]

Immune thrombocytopenic purpura(ITP) is a disorder in which autoantibodies against platelet membrane proteins cause platelet destruction. In adults, ITP usually takes chronic courses with rare spontaneous remission and frequently relapse after therapeutic response. Overall 75% of patients attain a prolonged

response to conventional treatment with glucocorticoids and/or splenectomy, while 15-25% of patients do not respond to these treatments. Many patients with refractoriness can not maintain safe levels of platelet count for adequate hemostasis, and they require further therapy with drugs such as vinca alkaloids,

danazol, cyclophosphamide, azathioprine or intravenous gammaglobulin. Some patients do not respond to all therapies. A beneficial effect of combination chemotherapy in refractory ITP has been reported, but the therapeutic role of combination

chemotherapy is not fully appreciated. We performed combination chemotherapy to treat 15 patients with ITP.

An average rumber of 4.5(2-11) previous therapies, including splenectomy(6cases), had been unsuccessful or intolerent. One case underwent combination chemotherapy before and after splenectomy The mean age of patients at diagnosis of ITP was

32.7(20-52)years. Chemotherapy regimens were CVP(cyclophosphamide, vincristine, pednisolone) in 21 courses and CMOPP(cyclophosphamide, vincristhe, pednisolone, procarbazine) in 10 courses. Therapeutic response to combination chemotherapy was

as follows:

1. Among 31 courses of therapy, continuous complete response(CCR),transient complete response(TCR) and partial response(PR) were observed in 2(6.5%), 11(35.5%) and 10(32.3%) courses, respectively, There was noresponse(NR) in 8(25.8%) courses.

2. Chemotherapy regimens or status of splenectomy had no significant influence on response rates. However, there was a tendency towards higher complete remission rate in splenectomized patients.

3. There was no serious side effects of combination chemotherapy.

In conclusion, combination chemotherapy may have a beneficial role in the management of refractory cases or selected cases before splenectomy with acceptable side effects.