Fiegl M, Greil R, Pechlaner C, Krugmann J, Dirnhofer S
Department of Internal Medicine, Division of Hematology and Oncology University of Innsbruk, Innsbruck, Austria.
Ann Oncol. 2002 Sep;13(9):1503-6. doi: 10.1093/annonc/mdf214.
A patient is described who presented with pancytopenia, splenomegaly and excessively elevated lactate dehydrogenase levels in concurrence with signs of extramedullary hematopoiesis. Although initially considered in the differential diagnostic spectrum, a highly aggressive lymphoma could not be identified before the patient died, 6 weeks after admission. Even an intensive diagnostic work-up including splenectomy and repeated bone marrow biopsies was inconclusive. Finally, the diagnosis of an intravascular large B-cell lymphoma, a highly aggressive clinical subtype of a diffuse large B-cell lymphoma, spreading within vascular structures of multiple organs was established by autopsy. Intravascular large B-cell lymphoma is often not diagnosed before death due to the exclusive intravascular growth pattern of the tumor cells and a fulminant clinical course. The heterogeneous clinical features of this lymphoma subtype are discussed.
本文描述了一名患者，其表现为全血细胞减少、脾肿大、乳酸脱氢酶水平极度升高，并伴有髓外造血迹象。尽管最初在鉴别诊断范围内考虑过，但在患者入院6周后死亡前，未能确诊为高度侵袭性淋巴瘤。即使进行了包括脾切除术和多次骨髓活检在内的强化诊断检查，结果仍不明确。最后，通过尸检确诊为血管内大B细胞淋巴瘤，这是弥漫性大B细胞淋巴瘤的一种高度侵袭性临床亚型，在多个器官的血管结构内扩散。由于肿瘤细胞独特的血管内生长模式和暴发性临床病程，血管内大B细胞淋巴瘤在死亡前常难以诊断。本文还讨论了该淋巴瘤亚型的异质性临床特征。
