급성 A형간염에 의해 발생한 혈구포식증후군 1예
A case of hemophagocytic syndrome complicated by acute viral hepatitis A infection
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초록
혈구포식증후군은 매우 드물지만 발생할 경우 매우 심각한 임상 경과를 보인다. 조직병리학적으로는 활성화된 대식세포나 조직구가 골수나 세망내피계에서 혈구를 포식하는 모습을 관찰할 수 있으며 임상적으로는 고열, 간비종대, 혈구감소, 간기능이상 및 고페리틴혈증을 보인다. A형간염바이러스는 이차성 혈구포식증후군을 일으키는 매우 드문 원인이다. 저자들은 급성 A형간염 후 혈구포식증후군이 합병된 22세 여자 환자를 보고한다. 환자는 급성 A형간염의 전형적인 경과를 보이며 검사실 검사 및 임상적으로 호전되다가 혈구포식증후군이 발생하면서 간부전 및 파종혈관내응고가 진행되었고 결국 복강 내 출혈로 사망하였다.
Abstract
Hemophagocytic syndrome (HPS) is a rare but serious condition that is histopathologically characterized by activation of macrophage or histiocytes with hemophagocytosis in bone marrow and reticuloendothelial systems. Clinically it presents with high fever, hepatosplenomegaly, pancytopenia, liver dysfunction, and hyperferritinemia. Hepatitis A virus is a very rare cause of secondary HPS. We report a case of a 22-year-old woman infected by hepatitis A virus who was consequently complicated with HPS. She presented typical clinical features of acute hepatitis A, and showed clinical and biochemical improvements. However, HPS developed as a complication of acute hepatitis A and the patient died of intraperitoneal bleeding caused by hepatic decompensation and disseminated intravascular coagulation.
참고문헌 (14)
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