Acquired factor X deficiency associated with atypical AL-amyloidosis.

Abstract

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 β2-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.

• Authors: Masanori Furuhata, Noriko Doki, Tsunekazu Hishima, Tomomi Okamoto, Takatoshi Koyama, Satoshi Kaito, Gaku Oshikawa, Takeshi Kobayashi, Kazuhiko Kakihana, Hisashi Sakamaki, Kazuteru Ohashi
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 53 Issue 16 Pg. 1841-5 ( 2014) ISSN: 1349-7235 [Electronic] Japan
• PMID: 25130122 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin Light Chains
• Serum Amyloid A Protein
• Factor X

Topics

• Aged
• Amyloidosis (complications, metabolism)
• Factor X (metabolism)
• Factor X Deficiency (diagnosis, etiology)
• Female
• Humans
• Immunoglobulin Light Chains (metabolism)
• Serum Amyloid A Protein (metabolism)