A Case of Atypical Hemolytic Uremic Syndrome Triggered by Acute Pancreatitis in a Patient with a Membrane Cofactor Protein (CD46) Genetic Variant

Kosuke Mochizuki; Naohiro Toda; Masaaki Fujita; Satoshi Kurahashi; Hisako Hirashima; Kazuki Yoshioka; Tomoya Kitagawa; Akira Ishii; Toshiyuki Komiya

doi:10.2169/internalmedicine.3169-23

A Case of Atypical Hemolytic Uremic Syndrome Triggered by Acute Pancreatitis in a Patient with a Membrane Cofactor Protein (CD46) Genetic Variant

Intern Med. 2024 Feb 26. doi: 10.2169/internalmedicine.3169-23. Online ahead of print.

Authors

Kosuke Mochizuki¹, Naohiro Toda¹, Masaaki Fujita², Satoshi Kurahashi¹, Hisako Hirashima¹, Kazuki Yoshioka³, Tomoya Kitagawa³, Akira Ishii¹, Toshiyuki Komiya¹

Affiliations

¹ Department of Nephrology, Kansai Electric Power Hospital, Japan.
² Department of Rheumatology, Kansai Electric Power Hospital, Japan.
³ Department of Hematology, Kansai Electric Power Hospital, Japan.

PMID: 38403763
DOI: 10.2169/internalmedicine.3169-23

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a type of HUS. We herein report a case of aHUS triggered by pancreatitis in a patient with a heterozygous variant of membrane cofactor protein (MCP; P165S), a complement-related gene. Plasma exchange therapy and hemodialysis improved thrombocytopenia and anemia without leading to end-stage kidney disease. This MCP heterozygous variant was insufficient to cause aHUS on its own. Pancreatitis, in addition to a genetic background with a MCP heterozygous variant, led to the manifestation of aHUS. This case supports the "multiple hit theory" that several factors are required for the manifestation of aHUS.

Keywords: MCP variant; atypical HUS; multiple hit theory; pancreatitis.