Das R, Kaur U, Garewal G
Department of Haematology, Postgraduate Institute of Medical Education and Research, Chandigarh-160 012, India.
Clin Lab Haematol. 2002 Apr;24(2):131-3. doi: 10.1046/j.1365-2257.2002.00234.x.
We report a patient with splenic vein thrombosis (SVT) in whom splenectomy resulted in the unmasking of essential thrombocythemia (ET). He had portal hypertension with haematemesis, resulting in anaemia requiring repeated blood transfusions. Investigations revealed SVT. Following splenectomy, he suffered a transient ischaemic attack episode, associated with persistent thrombocytosis (> 2000 x 10(9)/l). Other myeloproliferative disorders were excluded and a diagnosis of ET was established. He responded to hydroxyurea but, due to financial constraints, he discontinued treatment and subsequently relapsed. The association of ET with SVT is rare and the diagnosis of ET was missed initially as the platelet count was normal prior to splenectomy.
我们报告了一名患有脾静脉血栓形成(SVT)的患者,其脾切除术后原发性血小板增多症(ET)得以显现。他患有门静脉高压并伴有呕血,导致贫血,需要反复输血。检查发现了SVT。脾切除术后,他发生了一次短暂性脑缺血发作,伴有持续性血小板增多(>2000×10⁹/L)。排除了其他骨髓增殖性疾病,确诊为ET。他对羟基脲有反应,但由于经济限制,他停止了治疗,随后病情复发。ET与SVT的关联很少见,最初由于脾切除术前血小板计数正常而漏诊了ET。