재발성 공장염으로 나타난 발작성 야간혈색소뇨증 1예
Paroxysmal Nocturnal Hemoglobinuria Presenting as Recurrent Jejunitis
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초록
공장염을 일으킬 수 있는 원인으로는 혈관염, 베체트병, 크론병, 세균 및 기생충 감염, 독소, 약물, 방사선 조사, 장허혈, 호산구 침윤, 림프종 등이 있으며 드문 원인으로는 졸링어-엘리슨 증후군(Zollinger-Ellison syndrome), 복강 스프루(celiac sprue) 등이 있다[1,2]. 공장염은 병력 청취, 신체검사, 혈액 검사, 내시경 및 영상의학적 검사를 통해 대부분 원인 감별이 가능하지만 원인을 찾기 어려운 공장염도 매우 드물게 보고되고 있다. 저자들은재발성공장염을보인발작성 야간혈색소뇨증(paroxysmal nocturnal hemoglobinuria, PNH) 환자를 경험하였기에 보고하고자 한다.
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder characterized by chronic complement-mediated intravascular hemolysis, nocturnal hemoglobinuria, predisposition to thrombosis and secondary bone marrow failure. Small bowel ischemia is a complication of PNH but has not been reported to date in a Korean adult. We report here a case of PNH presenting as recurrent jejunitis. Despite the uncommon etiology, PNH should be considered in the differential diagnosis of patients with unexplained recurrent jejunitis.
참고문헌 (10)
- Wada F, "Chronic nonspecific jejunitis-A case report-", Acta Med Nagasaki, 45, pp.53~56 (2000)
- Gunasekaran TS, "Duodenojejunitis : is it idiopathic or is it Henoch-Schönlein purpura without the purpura?", J Pediatr Gastroenterol Nutr, 30, pp.22~28 (2000)
- Takeda J, "Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria", Cell, 73, pp.703~711 (1993)
- Risitano AM, "Paroxysmal nocturnal hemoglobinuria : pathophysiology, natural history and treatment options in the era of biological agents", Biologics, 2, pp.205~222 (2008)
- Hillmen P, "Natural history of paroxysmal nocturnal hemoglobinuria", N Engl J Med, 333, pp.1253~1258 (1995)
- Rother RP, "The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin : a novel mechanism of human disease", JAMA, 293, pp.1653~1662 (2005)
- Blum SF, "Intrestinal infarction in paroxysmal nocturnal hemoglobinuria", N Engl J Med, 274, pp.1137~1138 (1966)
- Quentin V, "Paroxysmal nocturnal hemoglobinuria associated with intestinal ischemia leading to small bowel perforation", Gastroenterol Clin Biol, 27, pp.927~931 (2003)
- Kinoshita T, "Relationship between aplastic anemia and paroxysmal nocturnal hemoglobinuria", Int J Hematol, 75, pp.117~122 (2002)
- Young NS, "The management of paroxysmal nocturnal hemoglobinuria :recent advances in diagnosis and treatment and new hope for patients", Semin Hematol, 46(1 Suppl 1), pp.1~16 (2009)