Böhm J
Institute of Pathology, Albert Ludwigs University, Freiburg, Germany.
Am J Surg Pathol. 2000 Jan;24(1):56-65. doi: 10.1097/00000478-200001000-00007.
Gelatinous bone marrow transformation (GMT) is a rare disorder of unknown pathogenesis, characterized by fat cell atrophy, focal loss of hematopoietic cells, and deposition of extracellular gelatinous substances, which histochemically are mucopolysaccharides, rich in hyaluronic acid. To elucidate the conditions in which GMT develops, 155 cases of GMT that had been found in our archives among 80,000 marrow biopsies were revised. GMT occurred exclusively in adults, more often in males (n = 101) than in females (n = 54). Incidence and severity of GMT lesions were maximal in young adults. The spectrum of underlying diseases was heterogeneous and age-dependent. Anorexia nervosa, acute febrile states, and AIDS in younger ages (<40 years), alcoholism and lymphomas in middle ages, and carcinomas, lymphomas, and chronic heart failure in older ages (>60 years) were most commonly associated with GMT. Seventy-eight percent of patients with GMT showed weight loss; 81% were anemic. GMT in some cases may be a reversible lesion if the underlying disorder can be eliminated. In conclusion, GMT represents an indicator of severe illness in a patient but is not specific for a particular disease. Because GMT develops in different pathologic conditions, we suggest that basic bioregulatory processes play a role in its pathogenesis.
胶冻样骨髓转化(GMT)是一种发病机制不明的罕见疾病,其特征为脂肪细胞萎缩、造血细胞局灶性缺失以及细胞外胶冻样物质沉积,这些物质在组织化学上为富含透明质酸的黏多糖。为阐明GMT发生的条件,我们对在80000例骨髓活检档案中发现的155例GMT病例进行了复查。GMT仅发生于成年人,男性(n = 101)比女性(n = 54)更常见。GMT病变的发生率和严重程度在年轻人中最高。潜在疾病谱具有异质性且与年龄相关。年轻年龄段(<40岁)的神经性厌食症、急性发热状态和艾滋病,中年的酒精中毒和淋巴瘤,以及老年(>60岁)的癌症、淋巴瘤和慢性心力衰竭最常与GMT相关。78%的GMT患者体重减轻;81%贫血。如果潜在疾病能够消除,某些情况下GMT可能是可逆性病变。总之,GMT是患者严重疾病的一个指标,但并非特定疾病所特有。由于GMT在不同病理状态下发生,我们认为基本的生物调节过程在其发病机制中起作用。