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IgM-κ 및 IgA-λ 이중클론감마병증을 가진 림프형질세포림프종/발덴스트롬마크로글로불린혈증 1례

A Case of Lymphoplasmacytic Lymphoma/Waldenström’s Macroglobulinemia with IgM-κ and IgA-λ Biclonal Gammopathy

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초록

림프형질세포림프종은 소림프구, 형질세포양림프구, 형질세포로 이루어진 저등급 B-세포림프종으로서 골수, 림프절, 비장을 침범하는 질환이다. 림프형질세포림프종은 대개 골수를 침범하는데 IgM 단클론감마병증이 동반되는 경우 발덴스트롬마크로글로불린혈증이라고 일컫는다. IgM이 아닌 단클론감마병증 또는 이중클론감마병증을 동반하는 림프형질세포림프종은 매우 드물다. 통상적으로 골수 및 유세포검사를 통해 그 형태학적 소견과 세포의 면역표현형으로 진단을 하였는데, 최근에는 myeloid differentiation factor 88 (MYD88) 유전자의 L265P 돌연변이 검사가 림프형질세포림프종을 진단하는 데 많은 도움이 되고 있다. 저자들은 아직 국내에서 보고되지 않은 IgM-κ/IgA-λ 이중클론감마병증을 동반한 림프형질세포림프종 1예를 경험하였기에 문헌고찰과 함께 보고하고자 한다.

Abstract

Lymphoplasmacytic lymphoma (LPL) is a low-grade B-cell neoplasm, composed of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and sometimes lymph nodes or spleen. LPL with bone marrow involvement and an IgM monoclonal gammopathy of any concentration is designated as Waldenström macroglobulinemia (WM). LPL associated with non-IgM monoclonal gammopathy or biclonal gammopathy is rarely observed. LPL diagnosis was based on clinical, morphological, and immunophenotypic findings. Recently, the test for L265P mutation of the myeloid differentiation factor 88 (MYD88) gene has been helpful in the diagnosis of LPL. Here, we reported the first case of LPL/WM with IgM-κ/IgA-λ biclonal gammopathy in Korea.

참고문헌 (24)

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