Rocha-Rodrigues Beatriz, Miranda Roberto N, Medeiros L Jeffrey
Universidade Anhembi Morumbi, São Paulo, Brazil.
The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
J Hematop. 2025 Apr 12;18(1):16. doi: 10.1007/s12308-025-00630-0.
Various types of lymphoma/leukemia can present with leukemic involvement by neoplastic cells with prominent nucleoli. Patients with chronic lymphocytic leukemia (CLL) most commonly can present in this fashion but other, less common types include mantle cell lymphoma, splenic marginal zone lymphoma, splenic diffuse red pulp small B-cell lymphoma, and splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN). The latter is a newly proposed category in the fifth edition of the World Health Organization classification of hematolymphoid neoplasms. This category includes most cases that were previously designated hairy cell leukemia-variant and rare cases of CD5-negative B-prolymphocytic leukemia. A case of extranodal marginal zone lymphoma (EMZL) is reported to highlight the presence of nucleolated lymphocytes in the peripheral blood and bone marrow and their impact on the differential diagnosis, particularly with SBLPN. An 80-year-old woman with a reported history of "CLL", diagnosed and treated with clinical remission, developed gastric EMZL which relapsed twice after chemotherapy. At time of last relapse, a peripheral blood smear and bone marrow aspiration and biopsy specimens were evaluated to assess the presence and extent of blood and bone marrow involvement. The pathologic findings and differential diagnosis are discussed. A complete blood count showed a leukocyte count of 7.9 × 10/L and review of the peripheral blood smear showed 32% nucleolated lymphocytes (absolute lymphocyte count, 2.5 × 10/L). Bone marrow aspiration and biopsy showed a nodular, interstitial, and focal sinusoidal lymphoid infiltrate with a subset of lymphocytes displaying nucleoli. Flow cytometry immunophenotypic analysis of the bone marrow showed a CD5-negative, monotypic small B-cell population consistent with extranodal MZL. This case illustrates that nucleolated lymphocytes can be observed in the peripheral blood smear of patients with EMZL. These findings expand the differential diagnosis of circulating nucleolated lymphocytes in the blood.
各种类型的淋巴瘤/白血病可表现为肿瘤细胞累及白血病，肿瘤细胞有明显核仁。慢性淋巴细胞白血病（CLL）患者最常以此种方式表现，但其他较罕见类型包括套细胞淋巴瘤、脾边缘区淋巴瘤、脾弥漫性红髓小B细胞淋巴瘤以及伴有明显核仁的脾B细胞淋巴瘤/白血病（SBLPN）。后者是世界卫生组织血液淋巴系统肿瘤分类第五版中新提出的类别。该类别包括大多数先前被指定为毛细胞白血病变异型的病例以及罕见的CD5阴性B原淋巴细胞白血病病例。报告1例结外边缘区淋巴瘤（EMZL）病例，以突出外周血和骨髓中核仁化淋巴细胞的存在及其对鉴别诊断的影响，特别是与SBLPN的鉴别。1名80岁女性，有“CLL”病史，经诊断和治疗后临床缓解，发生胃EMZL，化疗后复发2次。在最后一次复发时，评估外周血涂片、骨髓穿刺和活检标本，以评估血液和骨髓受累的存在和程度。讨论了病理结果和鉴别诊断。全血细胞计数显示白细胞计数为7.9×10/L，外周血涂片复查显示32%核仁化淋巴细胞（绝对淋巴细胞计数为2.5×10/L）。骨髓穿刺和活检显示结节状、间质和局灶性窦状淋巴浸润，一部分淋巴细胞有核仁。骨髓的流式细胞术免疫表型分析显示CD5阴性、单型小B细胞群，与结外MZL一致。该病例说明EMZL患者外周血涂片中可观察到核仁化淋巴细胞。这些发现扩展了血液中循环核仁化淋巴细胞的鉴别诊断。
