만성 백혈병 (Chronic Leukemia) / CML, CLL

만성 백혈병 (Chronic Leukemia): Comprehensive Overview for Medical Professionals

출처 - 하이닥

개요 (Overview)

만성 백혈병은 조혈모세포가 비정상적으로 분화하여 성숙한 혈액 세포로 증식하는 질환으로, 만성 골수성 백혈병(CML)과 만성 림프구성 백혈병(CLL)로 구분됩니다.

Chronic leukemia is a disorder in which hematopoietic stem cells abnormally differentiate and proliferate into mature blood cells. It is classified into chronic myeloid leukemia (CML) and chronic lymphocytic leukemia (CLL).

원인 (Etiology)

1. 유전적 요인 (Genetic Factors): 필라델피아 염색체(CML의 주요 원인).

2. 화학 물질 (Chemical Exposure): 벤젠 등 특정 화학물질.

3. 방사선 노출 (Radiation Exposure): 고용량의 방사선 노출.

4. 기타 (Others): 가족력, 바이러스 감염.

5. Genetic Factors: Philadelphia chromosome (major cause of CML).

6. Chemical Exposure: Benzene and certain chemicals.

7. Radiation Exposure: High doses of radiation.

8. Others: Family history, viral infections.

발생빈도 (Incidence)

만성 백혈병은 주로 성인에서 발생하며, CML은 연간 10만 명당 약 1-2명의 발생률을 보이고, CLL은 서구 국가에서 더 흔합니다.

Chronic leukemia primarily affects adults, with an incidence of approximately 1-2 per 100,000 annually for CML. CLL is more common in Western countries.

임상증상 (Clinical Presentation)

1. 피로 (Fatigue): 만성적인 피로.

2. 체중 감소 (Weight Loss): 원인 불명의 체중 감소.

3. 감염 (Infections): 빈번한 감염.

4. 출혈 경향 (Bleeding Tendency): 멍, 잇몸 출혈, 비출혈.

5. 비장 및 간비대 (Splenomegaly and Hepatomegaly): 비장 및 간의 비대.

6. Fatigue: Chronic fatigue.

7. Weight Loss: Unexplained weight loss.

8. Infections: Frequent infections.

9. Bleeding Tendency: Bruising, gum bleeding, epistaxis.

10. Splenomegaly and Hepatomegaly: Enlargement of the spleen and liver.

진단 (Diagnosis)

1. 혈액 검사 (Blood Tests): 말초 혈액 도말 검사에서 비정상 백혈구 증가 확인.

2. 골수 검사 (Bone Marrow Examination): 골수 흡인 및 생검.

3. 세포유전학 검사 (Cytogenetic Studies): 염색체 이상 확인 (필라델피아 염색체 등).

4. 분자 검사 (Molecular Testing): 유전자 돌연변이 확인 (BCR-ABL1 등).

5. Blood Tests: Detection of abnormal increase in white blood cells in peripheral blood smear.

6. Bone Marrow Examination: Bone marrow aspiration and biopsy.

7. Cytogenetic Studies: Detection of chromosomal abnormalities (Philadelphia chromosome, etc.).

8. Molecular Testing: Identification of genetic mutations (BCR-ABL1, etc.).

병리조직 (Pathology)

• 만성 골수성 백혈병 (CML): 골수성 계열의 성숙 백혈구 증가.

• 만성 림프구성 백혈병 (CLL): 림프구 계열의 성숙 백혈구 증가.

• Chronic Myeloid Leukemia (CML): Increase of mature myeloid cells.

• Chronic Lymphocytic Leukemia (CLL): Increase of mature lymphoid cells.

영상검사 소견 (Imaging Findings)

• 흉부 X선 (Chest X-ray): 폐 감염 또는 종격동 림프절 비대 확인.

• CT/MRI: 비장 및 간 비대 평가.

• Chest X-ray: Detection of lung infection or mediastinal lymphadenopathy.

• CT/MRI: Evaluation of splenomegaly and hepatomegaly.

만성 백혈병의 병기 (Staging)

• CML 병기 (CML Staging): 만성기, 가속기, 급성기.

• CLL 병기 (CLL Staging): Rai 시스템 및 Binet 시스템.

• CML Staging: Chronic phase, accelerated phase, blast phase.

• CLL Staging: Rai system and Binet system.

병기에 따른 치료 (Treatment by Stage)

• CML 치료 (CML Treatment):

  • 만성기 (Chronic Phase): 티로신 키나제 억제제(TKI) (이매티닙 등).
  • 가속기 (Accelerated Phase): TKI 용량 증가, 추가 화학요법.
  • 급성기 (Blast Phase): TKI + 화학요법, 조혈모세포 이식.

• CLL 치료 (CLL Treatment):

  • 초기 단계 (Early Stage): 관찰.
  • 진행 단계 (Advanced Stage): 화학요법, 면역요법 (리툭시맙 등), 표적 치료 (이브루티닙 등).

• CML Treatment:

  • Chronic Phase: Tyrosine kinase inhibitors (TKIs) (e.g., Imatinib).
  • Accelerated Phase: Increased dose of TKIs, additional chemotherapy.
  • Blast Phase: TKIs + chemotherapy, stem cell transplantation.

• CLL Treatment:

  • Early Stage: Observation.
  • Advanced Stage: Chemotherapy, immunotherapy (e.g., Rituximab), targeted therapy (e.g., Ibrutinib).

방사선치료 (Radiation Therapy)

1. 적응증 (Indications): 비장 비대, 통증 조절, 림프절 비대.

2. 기술 (Techniques):

   • 국소 방사선 치료 (Localized Radiation Therapy): 비장 또는 림프절 부위.

3. 선량 (Dose):

   • 국소 방사선 치료: 20-30 Gy.

4. Indications: Splenomegaly, pain control, lymphadenopathy.

5. Techniques:

   • Localized Radiation Therapy: Spleen or lymph node areas.

6. Dose:

   • Localized Radiation Therapy: 20-30 Gy.

항암제 (Chemotherapy)

1. CML 치료 약제 (Agents for CML): 이매티닙(Imatinib), 닐로티닙(Nilotinib), 다사티닙(Dasatinib).

2. CLL 치료 약제 (Agents for CLL): 플루다라빈(Fludarabine), 사이클로포스파마이드(Cyclophosphamide), 리툭시맙(Rituximab).

3. Agents for CML: Imatinib, Nilotinib, Dasatinib.

4. Agents for CLL: Fludarabine, Cyclophosphamide, Rituximab.

병기별 예후 (Prognosis by Stage)

1. 5년 생존율 (5-year Survival Rate):
   • CML: 만성기 약 90%, 가속기 약 50-60%, 급성기 약 20-30%.
   • CLL: 초기 단계 약 80-90%, 진행 단계 약 50-60%.
2. 국소 재발율 (Local Recurrence Rate): 약 20-30%.

The prognosis for chronic leukemia varies by stage, with a 5-year survival rate of approximately 90% for chronic phase CML, 50-60% for accelerated phase, and 20-30% for blast phase. For CLL, the 5-year survival rate is approximately 80-90% for early stage and 50-60% for advanced stage. The local recurrence rate is about 20-30%.

참고문헌 (References)

1. Goldman, J. M., & Melo, J. V. (2003). Chronic myeloid leukemia—advances in biology and new approaches to treatment. New England Journal of Medicine, 349(15), 1451-1464.
2. Hallek, M., Cheson, B. D., Catovsky, D., Caligaris-Cappio, F., Dighiero, G., Döhner, H., ... & Montserrat, E. (2008). Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute–Working Group 1996 guidelines. Blood, 111(12), 5446-5456.

만성 백혈병 (Chronic Leukemia) 요약표 (Summary Table)

항목 (Item)한국어 (Korean)영어 (English)

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만성 골수성 백혈병(CML)과 만성 림프구성 백혈병(CLL) 비교 요약표 (Comparison Summary Table of CML and CLL)

항목 (Item)만성 골수성 백혈병 (CML)만성 림프구성 백혈병 (CLL)

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