골수섬유화를 동반한 급성범골수증식증 1예
A Case of Acute Panmyelosis with Myelofibrosis
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초록
4세 남자환자는 호흡곤란, 어지러움증 및 전신허약 증세가 있으면서, 간비장종대는 관찰되지 않았으며, 말초혈액에서 범혈구감소증과 백적아구증을 보이고, 골수흡인검사에서는 건성천자로 인하여 약 8%의 아세포가 관찰되면서 과립구, 적아구, 거핵아구에서 형태 이상이 관찰되었다. 골수생검에서는 증가된 세포충실도,특히 거핵아구가 심하게 증식되면서 세 계열 세포 모두 증가가 관찰되고, 아세포의 증가와 함께 골수섬유화가 관찰되어 WHO분류법에 의해 분류된 골수섬유화를 동반한 급성범혈구증식증으로 진단하였다. 화학요법 치료 후 추적 골수검사에서 범혈구감소증과골수섬유화는 의미있는 변화는 없었으나, 세포충실도 및 아세포수는 감소되었던 골수섬유화를 동반한 급성범혈구증식증을 문헌고찰과 함께 보고하는 바이다.
Abstract
We experienced a case of acute panmyelosis with myelofibrosis, one of the acute myeloblastic leukemia (AML) subtypes according to WHO classification. A 44 year old man presented with a laborious dyspnea, dizziness, and generalized weakness. On admission, the peripheral blood revealed pancytopenia (hemoglobin 4.0 g/dL, WBC 2,300/ L, platelet 92,000/ L) with leukoerythroblastosis. The bone marrow (BM) aspirate yielded inadequate material but histological sections showed hypercellularity, proliferation of trilineage cell lines (panmyelosis) with extensive myelofibrosis, and clusters of immature cells at paratrabecular area. On ultrasonographic examination, no evidence of hepatosplenomegaly or any other abnormalities were noted. The follow-up BM study after the chemotherapy with Ara C for 7 days and idarubicin for 3 days showed no significant changes in pancytopenia and myelofibrosis but revealed a significant decrease in BM cellularity and blasts. We review and report the literature on acute panmyelosis with myelofibrosis. (Korean J Lab Med 2004; 24: 207-11)
참고문헌 (11)
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