Abstract | BACKGROUND: CASE PRESENTATION: A 73-year-old man presented with thrombocytopenia, hemolytic anemia, disturbance of consciousness, and acute kidney injury after 4-year course of biopsy-proven idiopathic MN. ADAMTS13 activity was undetectable and the ADAMTS13 inhibitor was identified. Additionally, he was positive for anti-PLA2R antibody. The patient did not have any diseases that could cause secondary thrombotic microangiopathy, and he was diagnosed with acquired TTP. Steroid therapy and plasma exchange were initiated and the acquired TTP resolved. MN achieved remission 3 months after the anti-PLA2R antibody disappeared. CONCLUSIONS: This is the first reported case of acquired TTP developed during conservative treatment of idiopathic MN, with both ADAMTS13 inhibitor and anti-PLA2R antibody positive at the onset of the TTP. The present case suggests that idiopathic MN might be associated with the development of some cases of acquired TTP.
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Authors | Rei Iio, Shin'ichi Akiyama, Kensuke Mitsumoto, Yukimasa Iwata, Hiroki Okushima, Karin Shimada, Naomi Ota, Kodo Tomida, Hiroaki Fushimi, Tatsuya Shoji, Masanori Matsumoto, Terumasa Hayashi |
Journal | BMC nephrology
(BMC Nephrol)
Vol. 21
Issue 1
Pg. 431
(10 12 2020)
ISSN: 1471-2369 [Electronic] England |
PMID | 33046023
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Autoantibodies
- PLA2R1 protein, human
- Receptors, Phospholipase A2
- Creatinine
- ADAMTS13 Protein
- ADAMTS13 protein, human
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Topics |
- ADAMTS13 Protein
(antagonists & inhibitors, immunology, metabolism)
- Aged
- Autoantibodies
(blood)
- Conservative Treatment
- Creatinine
(blood)
- Glomerulonephritis, Membranous
(complications, immunology, pathology, therapy)
- Humans
- Kidney Glomerulus
(pathology)
- Male
- Microscopy, Electron
- Purpura, Thrombotic Thrombocytopenic
(etiology, therapy)
- Receptors, Phospholipase A2
(immunology)
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