Hahn Logan A, Torlakovic Emina, Bosch Mark
Internal Medicine, University of Saskatchewan, Saskatoon, CAN.
Hematopathology, University of Saskatchewan, Saskatoon, CAN.
Cureus. 2024 Apr 30;16(4):e59354. doi: 10.7759/cureus.59354. eCollection 2024 Apr.
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.
骨髓胶样变性(GTBM)是一种罕见的血液系统疾病,其中骨髓中的造血细胞被细胞外胶样物质取代,常导致血细胞减少。由于目前的文献主要由病例报告组成,这种疾病的真实发病率目前尚不清楚。然而,一项对大型骨髓登记处的分析表明,即使在需要进行骨髓活检的人群中,这也是一种极为罕见的病症。我们报告一例24岁男性病例,该患者有弥漫性大B细胞淋巴瘤病史且体重减轻了45公斤,后来被发现患有GTBM。他血细胞减少的程度导致住院时间延长并出现了许多并发症,最终促使其接受异基因干细胞移植(ASCT)的试验性治疗。据我们所知,这是首例报道使用ASCT作为潜在治疗方式的GTBM病例。虽然我们的患者在ASCT后确实有临床改善,但这些结果的持久性目前尚不清楚。此外,不确定ASCT是否真的是患者病情稳定的原因。鉴于此,我们目前无法主张将ASCT作为GTBM的治疗方法。我们报告此病例是为了提高在难治性血细胞减少背景下对这种罕见病症的认识。