Frey B, Flury R, Senn H J
Schweiz Med Wochenschr. 1986 Oct 18;116(42):1450-2.
Two patients with long known paroxysmal nocturnal haemoglobinuria (PNH) were hospitalized for progressive severe pancytopenia or thrombocytopenia. Clinical and laboratory findings revealed a hematologic complication related to the course of PNH. In view of normocellular bone marrow hematopoiesis and documented increased splenic sequestration of RBC, functional hypersplenism was diagnosed. Selective splenectomy confirmed this hypothesis and was followed by long-standing remission of pancytopenia for 10 or more years in both patients.
两名长期患有阵发性夜间血红蛋白尿（PNH）的患者因进行性严重全血细胞减少或血小板减少而住院。临床和实验室检查结果显示了一种与PNH病程相关的血液学并发症。鉴于骨髓造血细胞正常且有记录表明红细胞在脾脏的潴留增加，诊断为功能性脾功能亢进。选择性脾切除术证实了这一假设，两名患者术后全血细胞减少均长期缓解达10年或更长时间。
