카파 및 람다 면역글로불린 경쇄 유전자의 클론성 재배열을 동시에 보이는 람다 경쇄 표현 폐 MALT 림프종 1예
A Case of Lambda-expressing Pulmonary MALT Lymphoma with Dual Clonal Rearrangements of Kappa and Lambda Immunoglobulin Light Chain Gene
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초록
70세 여자 환자가 마른 기침을 주소로 내원하였다. 기관지 내 시경 검사와 경기관지 폐생검을 실시하여 조직 검사와 면역조직 화학염색을 시행하였으나 검체의 crush artifact로 인해 그 병변이 반응성 또는 악성인지 판단하기 어려웠다. 면역글로불린 유전자의 클론성 재배열의 검출을 위해 BIOMED-2 다중 중합 효소연쇄반응을 이용해 B세포 클론성 검사를 시행하였다. 그 결 과 카파와 람다 경쇄 모두에서 클론성 재배열을 보였다. 그러나 면역조직화학염색에서 카파 경쇄는 발현되지 않고 람다 경쇄만 발현되었다. 본 증례는 임상 소견 및 검사 결과들에 준하여 폐 MALT 림프종으로 진단되었다. 저자들은 다중 중합효소연쇄반 응 검사법을 사용하여 카파 및 람다 면역글로불린 경쇄 유전자 의 클론성 재배열을 동시에 보이지만 람다 경쇄 만을 분비하는 MALT 림프종의 국내 첫 증례를 보고하는 바이다.
Abstract
A 70-yr-old woman was hospitalized with a history of dry cough. Bronchial endoscopy and transbronchial lung biopsy were performed. However, the findings of histopathology and immunohistochemistry were not sufficient to decide whether the lesion was benign or malignant, because of the presence of crush artifacts in the biopsy specimens. We performed B-cell clonality studies using BIOMED-2 multiplex PCR (InVivoScribe Technologies, USA) to detect clonal rearrangements in the immunoglobulin gene. The results of multiplex PCR showed clonal rearrangements of both kappa and lambda immunoglobulin light chain genes. The findings of immunochemistry revealed that the lesion expressed lambda light chain, but not kappa light chain. Based on the clinical, pathologic, and molecular findings, this case was diagnosed as pulmonary MALT lymphoma. We report the first case in Korea of lambda-expressing MALT lymphoma that is shown to have dual clonal rearrangements of kappa and lambda immunoglobulin light chain gene by multiplex PCR.
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