Acquired Angioedema Associated with Lymphoproliferative Disorders.

ABSTRACT:

Introduction

Acquired angioedema due to C1 esterase inhibitor deficiency (C1INH-AAE) is most associated with lymphoproliferative disorders (LPDs), particularly low-grade B-cell subtypes. The condition remains under-recognized with long diagnostic delays due to various challenges including a lack of awareness of the condition.

Case presentation

We discuss 4 cases of C1INH-AAE associated with low-grade B-cell LPDs, including various diagnostic and management challenges. As our cases illustrate, constitutional symptoms or overt manifestations of LPD at diagnosis are often absent. Hence, a comprehensive multimodal approach to screening for an underlying B-LPD is important when a diagnosis of acquired angioedema is made. Levels of complement C4, C1q, and C1INH are useful for diag

SUBMITTER: Ng JY 

PROVIDER: S-EPMC10890797 | biostudies-literature | 2024 Jan-Dec

REPOSITORIES: biostudies-literature