고등급 림프종으로 형질전환된 원발성 림프절 변연부 B-세포 림프종 -1예 보고-

Abstract

Primary nodal marginal zone B-cell lymphoma (MZBCL) is recognized as a rare and distinct entity. The rate of histologic transformation into diffuse large B-cell lymphoma (DLBCL) seems lower than the rate of transformation in follicular lymphoma. We herein report a rare case of nodal MZBCL showing transformation into DLBCL. The patient was a 73-year-old female with cervical lymphadenopathy. On the initial biopsy, the lymph node architecture was diffusely effaced with an extensive interfollicular and parafollicular infiltrate of monocytoid B-cells. There were scattered large blastic B-cells without formations of compact sheets. The diagnosis of nodal MZBCL was made. The patient did not receive chemotherapy and was treated with a conservatively supportive regimen. Forty two months later, the patient developed a new cervical lymphadenopathy and a biopsy was performed. Histologically, the lymph node revealed diffuse sheets of transformed large B-cells showing prominent nucleoli. The diagnosis of DLBCL transformed from nodal MZBCL was made. The patient was treated with 3 cycles of combined CHOP chemotherapy and she showed clinical improvement. These observations suggest that an untreated primary nodal MZBCL may undergo high-grade transformation.