Abstract:Acquired angioedema due to C1 esterase inhibitor deficiency (C1INH-AAE) is most associated with lymphoproliferative disorders (LPD) particularly low-grade B-LPD However, this diagnosis may be challenging. A recent study reported a median diagnostic delay of 1.1 (0.5-2.6) years from onset of angioedema. Possible reasons are lack of awareness of the condition, misdiagnosis of the angioedema symptoms as part of the hematologic disorder.
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