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表现为白血病前期综合征的胶样变性

Gelatinous degeneration presenting as a preleukaemic syndrome.

作者信息

Arranz R, Gil-Fernandez J J, Acevedo A, Tomas J F, Alegre A, Fernandez-Rañada J M

机构信息

Department of Hematology, Hospital Universitario de la Princesa, Madrid, Spain.

出版信息

J Clin Pathol. 1996 Jun;49(6):512-4. doi: 10.1136/jcp.49.6.512.

Abstract

Gelatinous degeneration of marrow is a rare histological disorder associated with chronic debilitating diseases, such as anorexia nervosa, AIDS and postchemotherapy 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 hyaluronic 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.

摘要

骨髓的胶样变性是一种罕见的组织学病症,与慢性消耗性疾病相关,如神经性厌食症、艾滋病和化疗后再生障碍性贫血。实体瘤也与这种情况有关,但仅在两名白血病患者中报告过。在这些病例中,白血病和胶样变性是同时诊断出来的。在本文报道的病例中,一名48岁男性,在诊断出伴有7号染色体单体的急性髓性白血病的两年多之前,胶样变性是唯一观察到的组织学发现。透明质酸沉积的意义仍不确定。提出了两种假说:(1)胶样变性发生在组织修复过程中;(2)胶样变性通过改变骨髓微环境来抑制造血。在本文报道的病例中,7号染色体单体的存在表明骨髓发育异常是潜在疾病,最终发展为急性白血病。

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