PDF(Pubmed)
Abstract：
Gelatinous transformation of bone marrow (GTBM) is a rare hematologic condition in which hematopoietic cells in the bone marrow are replaced by extracellular gelatinous substances, often resulting in cytopenias. The true incidence of this condition is presently unknown, as the current body of literature primarily consists of case reports. However, an analysis of a large bone marrow registry suggests that this is a highly rare entity even among a population requiring bone marrow biopsy. We present a case of a 24-year-old man with a history of diffuse large B cell lymphoma and an associated 45-kilogram weight loss, who was later found to have GTBM. The extent of his cytopenias resulted in a prolonged hospitalization with numerous complications, eventually leading to experimental treatment with allogeneic stem cell transplantation (ASCT). To our knowledge, this is the first reported case of GTBM in which ASCT was employed as a potential treatment modality. While our patient did have clinical improvement following ASCT, the permanence of these results is presently unclear. Furthermore, it is uncertain if the ASCT was truly causative of the stabilization of the patient. Given this, we are currently unable to advocate for ASCT as a treatment for GTBM. We report this case to raise awareness of this rare entity in the context of refractory cytopenias.
摘要：
骨髓凝胶状转化（GTBM）是一种罕见的血液学疾病，其中骨髓中的造血细胞被细胞外凝胶状物质取代，经常导致血细胞减少。这种情况的真实发生率目前尚不清楚，因为目前的文献主要由病例报告组成。然而,对大型骨髓登记的分析表明，即使在需要骨髓活检的人群中，这也是一种非常罕见的实体。我们介绍了一个24岁的男性，有弥漫性大B细胞淋巴瘤和45公斤体重减轻的病史，后来被发现患有GTBM。他的血细胞减少程度导致住院时间延长，并伴有许多并发症，最终导致异基因干细胞移植（ASCT）的实验治疗。据我们所知,这是首例GTBM病例,其中ASCT被用作潜在治疗方式.虽然我们的患者在ASCT后确实有临床改善，这些结果的持久性目前尚不清楚。此外，不确定ASCT是否真的是患者稳定的原因.鉴于此,我们目前无法主张ASCT作为GTBM的治疗方法.我们报告此病例是为了在难治性血细胞减少症的背景下提高对这种罕见实体的认识。
