A subtype of myeloid leukemia, this disease involves autonomous proliferation of abnormally young cells (immature cells) with maturation disorders in not only white blood cells but also red blood cells. The abnormal immature red blood cells are characterized by various morphological abnormalities, such as megaloblast-like cells that resemble the megaloblasts seen in pernicious anemia, and their appearance in peripheral blood. Mature red blood cells also show marked deformation. In the early stages of the disease, the abnormality in the white blood cells is not obvious (erythrocyte disease), and the abnormality in the red blood cells disappears over time and the disease progresses to normal leukemia, so it is sometimes called Di Guglielmo syndrome. Source: Heibonsha World Encyclopedia, 2nd Edition Information |
骨髄性白血病の一亜型で,白血球系細胞のみならず赤血球系細胞にも成熟障害を伴って異常に若い細胞(幼若細胞)が自律的に増殖する疾患。赤血球系異常幼若細胞は,悪性貧血にみられる巨赤芽球に類似する(巨赤芽球様細胞)など種々の形態異常を伴うこと,末梢血液中に出現することなどを特徴とする。また,成熟した赤血球も著しい変形を伴う。病初期に白血球系細胞の異常のほうが明らかでなかったり(赤血病),赤血球系の異常がやがて消失して通常の白血病に移行することが少なくないためディ・グーグリエルモ症候群Di Guglielmo syndromeと呼ぶこともある。
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