Lyapichev Kirill A, Chapman Jennifer R, Iakymenko Oleksii, Ikpatt Offiong F, Teomete Uygar, Sanchez Sandra Patricia, Vega Francisco
Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Miami and Sylvester Comprehensive Cancer Center, Miami, FL, USA.
Department of Radiology, University of Miami and Sylvester Comprehensive Cancer Center, Miami, FL, USA.
Case Rep Hematol. 2017;2017:8496978. doi: 10.1155/2017/8496978. Epub 2017 Aug 1.
Recently, an unusual subtype of large B-cell lymphoma (LBCL) with distinctive clinicopathologic features has been recognized; it is characterized by involvement of bone marrow with or without liver and/or spleen, but no lymph node or other extranodal sites, usually associated with fever, anemia, and hemophagocytic lymphohistiocytosis (HLH). Because of this distinctive clinical presentation, it has been designated "bone marrow-liver-spleen" (BLS) type of LBCL. To date there is only one series of 11 cases of BLS type of LBCL with detailed clinical, pathologic, and cytogenetic data. Herein, we describe a case of BLS type LBCL presenting with associated HLH in a 73-year-old female. The bone marrow core biopsy showed cytologically atypical large lymphoma cells present in a scattered interstitial distribution and hemophagocytosis and infrequent large lymphoma cells were seen in the bone marrow aspirate smears. Circulating lymphoma cells were not seen in the peripheral blood smears. The patient underwent treatment with chemotherapy (R-CHOP) but unfortunately passed away 2 months after initial presentation. BLS type of LBCL is a very rare and clinically aggressive lymphoma whose identification may be delayed by clinicians and hematopathologists due to its unusual clinical presentation and pathologic features.
最近，一种具有独特临床病理特征的大B细胞淋巴瘤（LBCL）异常亚型已被识别；其特征是骨髓受累，可伴有或不伴有肝脏和/或脾脏受累，但无淋巴结或其他结外部位受累，通常伴有发热、贫血和噬血细胞性淋巴组织细胞增生症（HLH）。由于这种独特的临床表现，它被命名为LBCL的“骨髓-肝脏-脾脏”（BLS）型。迄今为止，仅有一组11例BLS型LBCL病例，具有详细的临床、病理和细胞遗传学数据。在此，我们描述一例73岁女性的BLS型LBCL病例，伴有相关的HLH。骨髓活检显示细胞学上非典型的大淋巴瘤细胞呈散在的间质分布，骨髓穿刺涂片可见噬血细胞现象及罕见的大淋巴瘤细胞。外周血涂片未见循环淋巴瘤细胞。该患者接受了化疗（R-CHOP）治疗，但不幸的是，在初次就诊后2个月去世。BLS型LBCL是一种非常罕见且临床侵袭性强的淋巴瘤，由于其不寻常的临床表现和病理特征，临床医生和血液病理学家可能会延迟对其的识别。
