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Gelatinous degeneration presenting as a preleukaemic syndrome

机译:胶质变性表现为白血病前综合征

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Gelatinous degeneration of marrow is a rare histological disorder associated with chronic debilitating diseases, such as anorexia nervosa, AIDS and postchemo-therapy aplasia. Solid tumours have been associated with this condition but it has been reported in only two patients with leukaemia. In these cases leukaemia and gelatinous degeneration were diagnosed simultaneously. In the case reported here, a 48 year old man, gelatinous degeneration was the only histological finding observed more than two years before the diagnosis of acute myelogenous leukaemia with monosomy 7. The significance of hyalu-ronic acid deposition remains uncertain. Two hypotheses have been put forward: (1) that gelatinous degeneration occurs during tissue repair; and (2) that gelatinous degeneration inhibits haemopoiesis by altering the microenvironment of the bone marrow. In the case reported here, the presence of monosomy 7 suggests that myelodysplasia was the underlying disorder which finally evolved into acute leukaemia.
机译:骨髓胶质变性是与慢性衰弱性疾病(例如神经性厌食症,艾滋病和化疗后发育不良)相关的罕见组织学疾病。实体瘤与这种情况有关,但仅在两名白血病患者中有报道。在这些情况下,可同时诊断出白血病和胶质变性。在此报道的一名48岁男性中,胶质变性是在诊断为单核7的急性骨髓性白血病之前两年多才观察到的唯一组织学发现。透明质酸沉积的意义仍然不确定。提出了两个假设:(1)组织修复过程中发生了凝胶状变性。 (2)凝胶状变性通过改变骨髓的微环境而抑制造血作用。在这里报道的病例中,7号单体病的存在表明骨髓增生异常是潜在的疾病,最终演变为急性白血病。

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