Acquired Factor X Deficiency Associated with Atypical AL-amyloidosis

作者：Furuhata Masanori; Doki Noriko^*; Hishima Tsunekazu; Okamoto Tomomi; Koyama Takatoshi; Kaito Satoshi; Oshikawa Gaku; Kobayashi Takeshi; Kakihana Kazuhiko; Sakamaki Hisashi; Ohashi Kazuteru

来源：Internal Medicine, 2014, 53(16): 1841-1845.

DOI：10.2169/internalmedicine.53.2191

• acquired factor X deficiency
• atypical amyloidosis
• nephrotic syndrome
• AL-amyloidosis

We herein describe the case of a 77-year-old woman with acquired factor X deficiency that was likely caused by atypical amyloidosis. The patient developed severe gastrointestinal bleeding as a result of a significant decrease of factor X activity. Neither proteinuria nor diarrhea was observed as an initial manifestation. Although a bone marrow examination revealed direct fast scarlet-positive extracellular deposits, they did not exhibit red-to-green dichroism under polarized light. Immunofluorescence microscopy showed that the fibrillar proteins were positive for CD138 but negative for beta(2)-microglobulin or amyloid A antibodies. These atypical pathological features of immunoglobulin light chain-amyloidosis in this patient might be related to its unique clinical presentation.

• 出版日期2014