Bahmad Hisham F, Gogola Samantha, Burton Lorena, Alloush Ferial, Cusnir Mike, Schwartz Michael, Howard Lydia, Sriganeshan Vathany
The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USA.
Herbert Wertheim College of Medicine, Florida International University, Miami, FL 33199, USA.
Hematol Rep. 2023 Jun 6;15(2):370-383. doi: 10.3390/hematolrep15020038.
Amyloidosis is a term describing the extracellular deposit of fibrils composed of subunits of several different normal serum proteins in various tissues. Amyloid light chain (AL) amyloidosis contains fibrils that are composed of fragments of monoclonal light chains. Many different disorders and conditions can lead to spontaneous splenic rupture, including AL amyloidosis. We present a case of a 64-year-old woman with spontaneous splenic rupture and hemorrhage. A final diagnosis of systemic amyloidosis secondary to plasma cell myeloma was made with infiltrative cardiomyopathy and possible diastolic congestive heart failure exacerbation. We also provide a narrative review of all documented cases of splenic rupture associated with amyloidosis from the year 2000 until January 2023, along with the main clinical findings and management strategies.
淀粉样变性是一个术语,用于描述由几种不同正常血清蛋白的亚基在各种组织中组成的纤维原纤维的细胞外沉积。淀粉样轻链(AL)淀粉样变性包含由单克隆轻链片段组成的纤维。许多不同的疾病和状况可导致自发性脾破裂,包括AL淀粉样变性。我们报告一例64岁女性自发性脾破裂并出血的病例。最终诊断为继发于浆细胞骨髓瘤的系统性淀粉样变性,伴有浸润性心肌病和可能的舒张性充血性心力衰竭加重。我们还对2000年至2023年1月期间所有记录在案的与淀粉样变性相关的脾破裂病例进行了叙述性综述,以及主要临床发现和管理策略。