Kim Young, Weiss Lawrence M, Chen Yuan-Yuan, Pullarkat Vinod
Division of Pathology, City of Hope National Medical Center, 1500 East Duarte Road, Duarte, CA 91010, USA.
Leuk Res. 2007 Dec;31(12):1749-54. doi: 10.1016/j.leukres.2007.04.008. Epub 2007 Jun 4.
Systemic mastocytosis (SM) may rarely be associated with lymphoproliferative disorders. In such cases, the relationship between the neoplastic mast cells and the malignant lymphocytes remains unclear. We describe a patient with indolent SM whose bone marrow showed evidence of low-grade B-cell lymphoma. By detecting the activating KIT mutation D816V in the microdissected bone marrow mast cells, but not in the neoplastic B-lymphocytes, we demonstrate the distinct clonal origins of the mastocytosis and lymphoma when these two entities coexist. We also highlight the clinical and pathologic differences between SM associated with lymphoid as opposed to myeloid neoplasms and discuss their pathogenesis.
系统性肥大细胞增多症（SM）很少与淋巴增殖性疾病相关。在这种情况下，肿瘤性肥大细胞与恶性淋巴细胞之间的关系仍不清楚。我们描述了一名惰性SM患者，其骨髓显示有低度B细胞淋巴瘤的证据。通过在显微切割的骨髓肥大细胞中检测到激活的KIT突变D816V，而在肿瘤性B淋巴细胞中未检测到，我们证明了这两种实体共存时肥大细胞增多症和淋巴瘤的不同克隆起源。我们还强调了与淋巴样肿瘤而非髓样肿瘤相关的SM之间的临床和病理差异，并讨论了它们的发病机制。
