陣發性夜間血紅素尿症併發動脈性腦梗塞：病例報告

陣發性夜間血紅素尿症(paroxysmal nocturnal hemoglobinuria)是一個後天罕見疾病，因為造血幹細胞的基因突變使得其生成的血球缺乏了某些特殊的蛋白，以致於血球容易受到補體的破壞而溶血或栓塞；過去的報告顯示，栓塞的血管常常是發生在靜脈，發生在動脈較爲少見。本次個案報告是一位男性，自高中時期之後，每當有發燒的情形發生，就會伴隨有黑深色尿，並導致貧血，當時即診斷有陣發性夜間血紅素尿症，之後長期藥物控制，但部份藥物的服用遵從性並不高；此外，病患並無高血壓、糖尿病、高血脂等病史，平日也不抽菸，也沒有腦中風的家族史，但卻在52歲的時候發生腦梗塞，並被發現右側中大腦動脈有長達約十公分的動脈內栓塞，以致於第一時間無法進行動脈內溶栓治療(intra-arterial thrombolysis)，改以緊急手術，切除部分腦葉並減壓。狀況穩定後開始進行復健治療，情況稍有進步，但仍因腦葉切除而有後遺症。復健上大致的方針與腦中風病患相同，但因爲感染症可以誘發其溶血發作，所以預防因爲腦中風而增加的泌尿道感染和吸入性肺炎等危險也就變得更加重要；此病患也的確在復健的期間發生過泌尿道感染並導致黑深色尿而貧血。藉此病例，我們將回顧並討論其致病的可能機轉以及治療與復健上應考慮的事項。

關鍵字

陣發性夜間血紅素尿症；栓塞；動脈性腦梗塞

並列摘要

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder caused by a somatic mutation of the hematopoietic stem cells, which results in a loss of the complement regulatory proteins usually present on the membrane of blood cells. The outcome is an increased tendency for complement to damage the blood cells and, after a series of reactions, hemolytic and thrombotic episodes may occur. On reviewing past reports, PNH-related thrombosis usually occurs in the venous system and only rarely happens in the arterial system. In our case, a male patient was found to have suffered from dark urine and anemia after each of a series of febrile episode and these had occurred since senior high school. The patient had no past history of hypertension, diabetes mellitus, hyperlipidemia and/or smoking; in addition, a family history of stroke was also denied. PNH was diagnosed and long term medication control was applied; however, compliance with the medication was not good due to side effects. Unfortunately, at the age of 52, an arterial cerebral infarction occurred and there was thrombosis of the right middle cerebral artery over a length of about 10 cm. Based on this, primary intra-arterial thrombolysis was not indicated. An emergency decompression operation was carried out to decrease the intracranial pressure. After he was in a stable condition, a rehabilitation program was initiated and there has been partial improvement in the patient's situation. The rehabilitation program for this patient was similar to those of other stroke patients, but because an infectious event in a PNH patient may induce a hemolytic episode, it was very important to prevent the occurrence of any infection, including urinary tract infection and aspiration pneumonia. Notwithstanding this, the patient actually suffered from dark urine and anemia after an episode of urinary tract infection during the rehabilitation period. Using this case, we review and discuss the pathophysiology of PNH and what we consider to be the appropriate treatment regimen and rehabilitation for stroke patients suffering from PNH.

並列關鍵字

paroxysmal nocturnal hemoglobinuria ； thrombosis ； arterial cerebral infarction

國際替代計量

陣發性夜間血紅素尿症併發動脈性腦梗塞：病例報告

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