베체트병 환자에서 발생한 특발성 혈소판증가증 1예
A case of essential thrombocythemia in a patient with Behçet’s disease
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초록
베체트병에서 혈액학적 질환이 연관된 증례들이 소수 보고되어 왔으나, 치료와 관련 없이 본태성 혈소판증가증이 동반된 예는 없었다. 저자들은 베체트병에 동반된 본태성 혈소판증가증을 경험하여 이에 보고하는 바이다. 베체트병은 정확한 병인은 완전히 알려지지는 않았으나, 유전적인 소인이 있는 환자에서 감염과 같은 환경적인 요인이 면역기능에 이상을 초래하는 것으로 알려져 있으며 이 가운데 일부는 본태성 혈소판증가증의 발생 원인과도 관련성이 있는 것으로 밝혀지고 있으므로 향후 지속적인 연구가 필요할 것으로 보인다.
Abstract
Behçet’s disease (BD) is a chronic inflammatory disorder characterized by vasculitis of unknown cause and involving multiple organs. Its pathogenesis includes neutrophil hyperfunction and the overproduction of inflammatory cytokines, including INF‐α. BD is often accompanied by leukocytosis, but is rarely associated with myeloproliferative disease. Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by marked thrombocytosis and marrow megakaryocytic hyperplasia. Only one case of ET associated with incomplete‐type intestinal BD during hydroxyurea treatment has been reported. Here, we report a case of essential thrombocythemia in 53‐year‐old female with BD who had taken no medication. Based on the history, physical examination, and routine laboratory and bone marrow examination, we diagnosed her with ET.
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