DOI QR코드

DOI QR Code

폐 종괴로 나타난 원발성 골수섬유증 환자의 골수 외 조혈 1예

A Case of Extramedullary Hematopoiesis Presenting as a Lung Mass in a Patient with Primary Myelofibrosis

  • 김여명 (한국원자력의학원 원자력병원 내과) ;
  • 김현태 (한국원자력의학원 원자력병원 내과) ;
  • 노금엽 (한국원자력의학원 원자력병원 내과) ;
  • 강민수 (한국원자력의학원 원자력병원 내과) ;
  • 장윤환 (한국원자력의학원 원자력병원 진단검사의학과) ;
  • 김혜련 (한국원자력의학원 원자력병원 내과) ;
  • 이재철 (한국원자력의학원 원자력병원 내과) ;
  • 김철현 (한국원자력의학원 원자력병원 내과)
  • Kim, Yeo Myeong (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Kim, Hyeon Tae (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Noh, Geum Youb (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Kang, Min Soo (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Chang, Yoon Hwan (Department of Laboratory Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Kim, Hye-Ryoun (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Lee, Jae Cheol (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences) ;
  • Kim, Cheol Hyeon (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences)
  • 투고 : 2009.07.29
  • 심사 : 2009.09.02
  • 발행 : 2009.09.30

초록

Primary myelofibrosis is characterized by replacement of bone marrow with fibrotic tissue and the development of extramedullary hematopoiesis. Extramedullary hematopoiesis primarily involves the spleen and liver, but can also occur in the lungs. We report the case of an 80-year-old male who was admitted for evaluation of a lung mass and persistent thrombocytopenia. A percutaneous needle aspiration from the mass in the right lower lung showed myelopoietic cells with fatty tissue. A bone marrow biopsy revealed a hypercellular marrow with an increased number of atypical megakaryocytes. The final diagnosis was a prefibrotic stage of primary myelofibrosis leading to extramedullary hematopoiesis in the lung.

키워드

참고문헌

  1. Bowling MR, Cauthen CG, Perry CD, Patel NP, Bergman S, Link KM, et al. Pulmonary extramedullary hematopoiesis. J Thorac Imaging 2008;23:138-41 https://doi.org/10.1097/RTI.0b013e31815b89aa
  2. Koch CA, Li CY, Mesa RA, Tefferi A. Nonhepatosplenic extramedullary hematopoiesis: associated diseases, pathology, clinical course, and treatment. Mayo Clin Proc 2003;78:1223-33 https://doi.org/10.4065/78.10.1223
  3. Lund RE, Aldridge NH. Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr 1984;8:788-90 https://doi.org/10.1097/00004728-198408000-00042
  4. MacKinnon S, McNicol AM, Lee FD, McDonald GA. Myelofibrosis complicated by intestinal extramedullary haemopoiesis and acute small bowel obstruction. J Clin Pathol 1986;39:677-9 https://doi.org/10.1136/jcp.39.6.677
  5. Oh HA, Kang HJ, Lee G, Choi JH, Lee JL, Lee KH, et al. A case of chronic myelogenous leukemia with extramedullary hematopoiesis presenting as liver mass. Korean J Hematol 2002;37:80-3
  6. Lee HJ, Hong JH, Kang YH, Seo KS, Seong EY, Yu YI, et al. A case of cutaneous extramedullary hematopoiesis in idiopathic myelofibrosis. Korean J Hematol 1997;32:476-80
  7. Goh DH, Lee SH, Cho DC, Park SH, Hwang JH, Sung JK. Chronic idiopathic myelofibrosis presenting as cauda equina compression due to extramedullary hematopoiesis:a case report. J Korean Med Sci 2007;22:1090-3 https://doi.org/10.3346/jkms.2007.22.6.1090
  8. Kwak HS, Lee JM. CT findings of extramedullary hematopoiesis in the thorax, liver and kidneys, in a patient with idiopathic myelofibrosis. J Korean Med Sci 2000;15:460-2 https://doi.org/10.3346/jkms.2000.15.4.460
  9. Spivak JL. Chapter 103. Polycythemia vera and other myeloproliferative diseases. In: Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, editors. Harrison’s principles of internal medicine. 17th ed. New York: McGraw-Hill Co, Inc.; 2008. p. 674-5
  10. Ward HP, Block MH. The natural history of agnogenic myeloid metaplasia (AMM) and a critical evaluation of its relationship with the myeloproliferative syndrome. Medicine (Baltimore) 1971;50:357-420 https://doi.org/10.1097/00005792-197109000-00001
  11. Bradford CR, Smith SR, Wallis JP. Pericardial extramedullary hematopoiesis in chronic myelomonocytic leukemia. J Clin Pathol 1993;46:674-5 https://doi.org/10.1136/jcp.46.7.674
  12. Fielding JR, Owens M, Naimark A. Intrathoracic extramedullary hematopoiesis secondary to B12 and folate deficiency: CT appearance. J Comput Assist Tomogr 1991;15:308-10 https://doi.org/10.1097/00004728-199103000-00023
  13. Asakura S, Colby TV. Agnogenic myeloid metaplasia with extramedullary hematopoiesis and fibrosis in the lung: report of two cases. Chest 1994;105:1866-8 https://doi.org/10.1378/chest.105.6.1866
  14. Kopecky KK, Moriarty AT, Antony AC, Baker MK. Extramedullary hematopoiesis in acute lymphocytic leukemia masquerading as hepatic, renal, and splenic microabscesses. AJR Am J Roentgenol 1986;147:846-7 https://doi.org/10.2214/ajr.147.4.846
  15. Kupferschmid JP, Shahian DM, Villanueva AG. Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest 1993; 103:974-5 https://doi.org/10.1378/chest.103.3.974