초록 close

저자들은 비종대와 피부 발진 및 점상출혈을 주소로 내원한 50세 남자 환자에서 말초혈액 및 골수검사에서 모발상세포를 보이고 TRAP 음성, 면역표현형 검사에서 전형적인 HCL-variant의 소견을 보인 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.


Hairy cell leukemia-variant (HCL-variant) is a rare B-cell disorder of hairy cell leukemia cases. The main features are splenomegaly, lymphocytosis and cytopenias without monocytopenia. Diagnosis is based on the distinctive hairy cell morphology and immunophenotype. Cells from hairy cell leukemia (HCL) and HCL-variant have a distinct immunophenotype which is of a mature but not terminally differentiated activated B-cell. We experienced a case of HCL- variant in a 50-year-old man with marked splenomegaly and skin eruptions. Peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow was easily aspirated and showed hypercellularity and diffuse interstitial infiltration of hairy cells. Tartrate-resistant acid phosphatase (TRAP) reactivity was negative in the hairy cells. Immunophenotyping results of lymphoid cells were CD2 (-), CD3 (-), CD5 (-), CD7 (-), CD10 (-), CD19 (++), CD20 (+++), sKappa (-), sLambda (+), CD23 (-), CD25 (-), FMC7 (+) and CD103 (++).