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Case Reports
. 2024 Oct 1;63(19):2651-2654.
doi: 10.2169/internalmedicine.3169-23. Epub 2024 Feb 26.

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

Kosuke Mochizuki  1 , Naohiro Toda  1 , Masaaki Fujita  2 , Satoshi Kurahashi  1 , Hisako Hirashima  1 , Kazuki Yoshioka  3 , Tomoya Kitagawa  3 , Akira Ishii  1 , Toshiyuki Komiya  1
Affiliations
Case Reports

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

Kosuke Mochizuki et al. Intern Med. .

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.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure.
Figure.
Abdominal computed tomography (CT) findings in this patient. (A) Day X+2 after performing endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). The pancreatic body tail has a mildly reduced contrast effect in the arterial phase, consistent with the findings of acute pancreatitis. (B) Day X+7. CT indicates a low-absorption area suspected of being walled-off necrosis (WON) around the peripancreatic to the gastric body and gastric antrum.
See this image and copyright information in PMC

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