McGinnis Eric, Medvedev Nadia, Richards Mikhyla J, Chen Luke Y C, Wong Michelle P
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, Canada.
Vancouver General Hospital, Vancouver, British Columbia, Canada.
Mayo Clin Proc Innov Qual Outcomes. 2019 Oct 16;3(4):517-522. doi: 10.1016/j.mayocpiqo.2019.07.001. eCollection 2019 Dec.
Hemophagocytosis refers to ingestion of hematopoietic elements or mature blood cells by another cell, typically by cells conventionally associated with phagocytic capacity. Although the finding of hemophagocytosis as a prominent feature in a patient's bone marrow might prompt consideration of a hemophagocytic syndrome (HPS) such as hemophagocytic lymphohistiocytosis (HLH) in a clinician's or pathologist's differential diagnosis, this morphologic feature can be nonspecific in the absence of other clinical and laboratory features of pathologic immune activation, which is the sine qua non of HPS/HLH. We describe three patients whose clinical presentations included transfusion-dependent anemia and whose bone marrow aspirates showed unexpectedly brisk hemophagocytosis of mature red blood cells. Despite striking morphologic hemophagocytosis, no patient met criteria for diagnosis of an HPS. Transfusion-associated hemophagocytosis and hyperferritinemia must be carefully distinguished from HLH through clinical and laboratory assessment. Biomarkers of pathologic immune activation are important diagnostic aids.
噬血细胞现象是指一个细胞摄取造血成分或成熟血细胞,通常是由传统上具有吞噬能力的细胞来摄取。尽管在患者骨髓中发现噬血细胞现象作为一个突出特征可能会促使临床医生或病理学家在鉴别诊断时考虑噬血细胞综合征(HPS),如噬血细胞性淋巴组织细胞增生症(HLH),但在缺乏病理免疫激活的其他临床和实验室特征时,这种形态学特征可能是非特异性的,而病理免疫激活是HPS/HLH的必要条件。我们描述了三名患者,他们的临床表现包括依赖输血的贫血,其骨髓穿刺显示成熟红细胞出现意外活跃的噬血细胞现象。尽管有明显的形态学噬血细胞现象,但没有患者符合HPS的诊断标准。必须通过临床和实验室评估仔细区分输血相关的噬血细胞现象和高铁蛋白血症与HLH。病理免疫激活的生物标志物是重要的诊断辅助手段。