Blood A M, Lowenthal E A, Nowakowski R W
School of Optometry, University of Alabama at Birmingham, USA.
J Am Optom Assoc. 1997 Nov;68(11):734-8.
Polycythemia vera is a chronic clonal disorder associated with excessive proliferation of erythrocytes, leukocytes, and thrombocytes, as well as an accompanying splenomegaly. Ocular manifestations of polycythemia vera include occipital cortex transient ischemic attacks, transient monocular blindness, vaso-occlusive disease, and retinal hemorrhages.
A 56-year old man with longstanding polycythemia vera sought treatment for a chief symptom of blurred vision in the left eye and a red tinge to things first noticed on awakening that morning. He had preretinal and intraretinal hemorrhages and was subsequently found to be severely anemic as a result of postpolycythemic myeloid metaplasia after years of phlebotomy. Splenectomy controlled his anemia and thrombocytopenia, allowing transient improvement of the retinal hemorrhages. Acute leukemia subsequently developed and the patient died 7 weeks after initial examination.
In this case, preretinal and intraretinal hemorrhages were found in a patient with longstanding polycythemia vera. The exact origin of these hemorrhages is uncertain. They are probably secondary to anemia, but the possibility that they are sites of extramedullary hematopoiesis must be considered. The appearance of retinal hemorrhages warrants careful investigation to rule out diabetes, hypertension, and anemia, as well as the various other blood dyscrasias.
真性红细胞增多症是一种慢性克隆性疾病，与红细胞、白细胞和血小板过度增殖以及脾脏肿大有关。真性红细胞增多症的眼部表现包括枕叶皮质短暂性脑缺血发作、一过性单眼盲、血管闭塞性疾病和视网膜出血。
一名患有长期真性红细胞增多症的56岁男性因左眼视力模糊和当天早晨醒来时首次注意到的视物发红为主诉症状前来就诊。他有视网膜前和视网膜内出血，随后因多年放血治疗后发生真性红细胞增多症后骨髓化生而被发现严重贫血。脾切除术控制了他的贫血和血小板减少症，使视网膜出血得到短暂改善。随后患者发展为急性白血病，并在初次检查后7周死亡。
在本病例中，一名患有长期真性红细胞增多症的患者出现了视网膜前和视网膜内出血。这些出血的确切起源尚不确定。它们可能继发于贫血，但必须考虑它们是髓外造血部位的可能性。视网膜出血的出现需要仔细检查以排除糖尿病、高血压和贫血以及其他各种血液系统疾病。
