The Korean Journal of Medicine

  • 제83권6호
  • /
  • Pages.752-756
  • /
  • 2012
  • /
  • 1738-9364(pISSN)
  • /
  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지
DOI QR코드

DOI QR Code

위 원발성 ALK 양성 역형성대세포림프종 1예

A Case of Primary Gastric ALK-Positive Anaplastic Large-Cell Lymphoma

  • 장우성 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 정우진 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 강경훈 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 권장훈 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 홍종삼 (울산대학교 의과대학 강릉아산병원 내과학교실) ;
  • 엄대운 (울산대학교 의과대학 강릉아산병원 병리학교실) ;
  • 천갑진 (울산대학교 의과대학 강릉아산병원 내과학교실)
  • Chang, Woo Sung (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Jeong, Woo Jin (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Kang, Kyung Hoon (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Kwon, Jang Hoon (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Hong, Jong Sam (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Eom, Dae Woon (Department of Pathology, Gangneung Asan Hospital, University of Ulsan College of Medicine) ;
  • Cheon, Gab Jin (Department of Internal Medicine, Gangneung Asan Hospital, University of Ulsan College of Medicine)
  • 발행 : 2012.12.01
⟨ 이전 논문 다음 논문 ⟩

초록

위 원발성 역형성대세포림프종은 위 원발성 림프종 중 매우 드물게 발생하는 것으로 알려져 있다. 이에 대한 표준화된 치료나 예후에 대해 잘 알려져 있지 않다. 저자들은 37세 여자가 상복부 통증으로 내원하여 시행한 상부위장관검사에서 다발성, 중심 함몰이 있는 융기성 미란이 있어 조직검사를 시행하였고, 조직 검사상 CD30 양성, ALK 양성 역형성 대세포림프종이 나왔으며, 타장기 침범 소견이 없어 위 원발성 ALK 양성 역형성대세포림프종으로 진단하였다. CHOP 항암화학치료를 하였으나 뇌 전이, 림프절 전이 및 연수막 파종으로 사망한 예를 경험하여 문헌고찰과 함께 보고한다.

Anaplastic large-cell lymphoma (ALCL) is a rare subgroup of non-Hodgkin's lymphoma. Primary gastric ALCL is extremely rare. Patients with anaplastic lymphoma kinase (ALK)-positive primary systemic ALCL are known to have better overall survival than those with ALK-negative ALCL. We herein report a case of primary gastric ALK-positive anaplastic large-cell lymphoma. A 37-year-old woman presented with postprandial epigastric pain for 2 months. Endoscopic examination of the upper gastrointestinal tract showed multiple variably sized, round, elevated lesions with friable crater-erosion on the body and fundus. Pathologic examination revealed atypical large lymphoid cell infiltration in the lamina propria; the cells were positive for CD3, CD30, and ALK. We diagnosed the patient with ALCL. She underwent a cycle of chemotherapy with cyclophosphamide, adriamycin, vincristine, and prednisolone. However, she developed brain metastases. Despite two cycles of palliative chemotherapy with high-dose methotrexate for brain metastases, she died due to ALCL progression.

키워드

참고문헌

  1. Al-Sheneber I I, Shibata H. Primary gastric lymphoma. Cancer Control 1997;4:245-252. https://doi.org/10.1177/107327489700400306
  2. Koch P, del Valle F, Berdel WE, et al. Primary gastrointestinal non-Hodgkin's lymphoma: I. anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92. J Clin Oncol 2001;19:3861-3873. https://doi.org/10.1200/JCO.2001.19.18.3861
  3. Stein H, Mason DY, Gerdes J, et al. The expression of the Hodgkin's disease associated antigen Ki-1 in reactive and neoplastic lymphoid tissue: evidence that Reed-Sternberg cells and histiocytic malignancies are derived from activated lymphoid cells. Blood 1985;66:848-858.
  4. Savage KJ, Harris NL, Vose JM, et al. ALK- anaplastic large-cell lymphoma is clinically and immunophenotypicallydifferent from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood 2008;111:5496-5504. https://doi.org/10.1182/blood-2008-01-134270
  5. Song HH, Baik GH, Kwon JH, et al. A case of primary gastric CD30-positive anaplastic large-cell lymphoma. J Korean Med Sci 2005;20:1062-1065. https://doi.org/10.3346/jkms.2005.20.6.1062
  6. Kang YK, Kim BS, Kim TW, et al. Clinicopathologic charcteristics of Korean non-Hodgkin's lymphomas based on REAL classification. J Korean Cancer Assoc 1999;31:641-652.
  7. Kelleher FC, McDermott R. The emerging pathogenic and therapeutic importance of the anaplastic lymphoma kinase gene. Eur J Cancer 2010;46:2357-2368. https://doi.org/10.1016/j.ejca.2010.04.006
  8. Shiota M, Nakamura S, Ichinohasama R, et al. Anaplastic large cell lymphomas expressing the novel chimeric protein p80NPM/ALK: a distinct clinicopathologic entity. Blood 1995;86:1954-1960.
  9. Suekane H, Iida M, Kuwano Y. Diagnosis of primary early gastric lymphoma: usefulness of endoscopic mucosal resection for histologic evaluation. Cancer 1993;71:1207-1213. https://doi.org/10.1002/1097-0142(19930215)71:4<1207::AID-CNCR2820710406>3.0.CO;2-O
  10. Al-Akwaa AM, Siddiqui N, Al-Mofleh IA. Primary gastric lymphoma. World J Gastroenterol 2004;10:5-11. https://doi.org/10.3748/wjg.v10.i1.5