A disease of unknown cause characterized by bone marrow fibrosis, hematopoiesis in the liver and spleen, and the appearance of immature granulocytes and erythroblasts in the peripheral blood vessels. The average survival time is not much different from that of chronic myeloid leukemia, 1 to 5 years after initial diagnosis, but some patients may survive for more than 10 years. Symptoms at the time of initial diagnosis are anemia (normocytic, normochromic) and splenomegaly, with a mild increase in white blood cells. The white blood cell picture shows so-called leukoerythroblastosis, and there are also significant morphological changes in red blood cells and platelets. The bone marrow becomes fibrotic, and in many cases, no bone marrow aspirate can be obtained even when a bone marrow aspirate is performed. Source: Heibonsha World Encyclopedia, 2nd Edition Information |
骨髄の繊維化,肝臓や脾臓での造血作用,幼若顆粒球や赤芽球の末梢血管への出現を特徴とする原因不明の疾患。平均生存期間は慢性骨髄性白血病と大差なく,初診後1~5年であるが,10年以上の長期生存をみることもある。初診時の症状は,貧血(正球性,正色素性)と脾腫で,白血球は軽度に増加する。白血球像はいわゆる白赤芽球症leukoerythroblastosisを呈し,赤血球や血小板の形態変化も著しい。骨髄は繊維化して,骨髄穿刺(せんし)を行っても穿刺液は得られないことが多い。
出典 株式会社平凡社世界大百科事典 第2版について 情報 |
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