与系统性淀粉样变性相关的μ重链病

Mu-heavy chain disease associated with systemic amyloidosis.

作者信息

Kinoshita Koji, Yamagata Toshiaki, Nozaki Yuji, Sugiyama Masafumi, Ikoma Shinya, Funauchi Masanori, Kanamaru Akihisa

机构信息

Department of Hematology, Nephrology and Rheumatology, Kinki Univeristy School of Medicine, Osaka-Sayama, Osaka, Japan.

出版信息

Hematology. 2004 Apr;9(2):135-7. doi: 10.1080/10245330410001671561.

DOI:10.1080/10245330410001671561

PMID:15203869

Abstract

mu-heavy chain disease (HCD) is very rare, with only 30 cases reported in the literature. We report a patient with mu-HCD associated with systemic amyloidosis. The diagnosis of mu-HCD was based on findings of mu-heavy chain fragments in the serum, Bence Jones proteinuria and vacuolated plasma cells in the bone marrow. To our knowledge, this is the third case in which systemic amyloidosis led to the patient's death.

摘要

μ重链病（HCD）非常罕见，文献中仅报道过30例。我们报告了1例与系统性淀粉样变性相关的μ-HCD患者。μ-HCD的诊断基于血清中μ重链片段的检测结果、本周氏蛋白尿以及骨髓中出现空泡的浆细胞。据我们所知，这是系统性淀粉样变性导致患者死亡的第三例病例。