골수 검사에서 진단된 간비장T세포림프종 1예
A Case of Hepatosplenic T-cell Lymphoma Diagnosed by Bone Marrow Examination
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간비장T세포림프종은 간, 비장 그리고 골수의 동모양혈관에 림프종세포가 침범하며 림프절 비대는 없다. 본 증례는 좌상복부 통증과 오심을 주소로 내원한 베트남인 여자 환자에서 골수 검사로간비장T세포림프종이 진단된 예이다. 전산화단층촬영에서 비장비대와 간에 다발성 저음영 결절이 관찰되었고, 혈소판 감소가 보였다. 림프절비대는 관찰되지 않았다. 극심한 혈소판감소증으로 인하여 비장 조직생검을 시행하지 못하였다. 골수흡인도말에서 림프종세포가 보였고, 골수 조직에서 동모양혈관주위에 있는 림프종세포는 CD3(+), CD20(-) 면역조직화학염색 결과를 보였다. 골수 검체를 이용한 T세포 수용체(T-cell receptor, TCR) 델타쇄(δ) 유전자(TRD@) 재배열 검사에서 TCR의 단클론성 재배열을 보여 간비장T세포림프종으로 진단할 수 있었다. 간, 비장 조직 생검을 할 수 없는 경우에는 골수 조직 검사와 이를 이용한 TCR 유전자 재배열 검사가 진단에 유용할 수 있다.
Abstract
Hepatosplenic T-cell lymphoma (HSTL) is a condition in which lymphoma cells infiltrate the sinusoids of the liver, spleen, and bone marrow, without lymph node involvement. We encountered a case of hepatosplenic T-cell lymphoma in a Vietnamese woman. The patient was hospitalized with epigastric pain and nausea. Splenomegaly and multiple poorly defined, low-attenuating nodular lesions in the liver were visualized on computed tomography (CT), and thrombocytopenia was noted. The lymph nodes were not significantly enlarged. Splenic biopsy could not be performed because of severe thrombocytopenia. Neoplastic lymphoid cells were present in bone marrow aspirates. Bone marrow sections revealed infiltration of CD3(+) and CD20(-) neoplastic lymphoid cells in the sinusoids. A clonality assay (IdentiClone T-Cell Receptor Delta Gene Clonality Assay; Invivoscribe Technologies, USA) showed gene rearrangements in the T-cell receptor delta gene. Thus, we made a confirmatory diagnosis of HSTL. When splenic biopsy is not available, bone marrow aspirates and clonality assessment may become useful diagnostic tools.
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