Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

Abstract

Background: Hereditary angioedema (HAE) is a rare disease caused by the deficiency of C1 esterase inhibitor. HAE has a risk of life-threatening complications such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC).Case: A 42-year-old man with HAE presented for deceased-donor kidney transplantation. Prophylactic fresh frozen plasma (FFP) was given before surgery because of the risk of edema development. With careful management during anesthesia, there were no problems during surgery. However, generalized edema, hypotension, hypoalbuminemia, massive drainage of serosanguineous fluids from the intraabdominal space, and DIC occurred on the day after surgery. CLS was suspected and sustained hypotension with generalized edema became worse despite treatment with albumin, danazol, FFP, and vasoactive drugs. The patient’s condition worsened despite intensive care and he died due to shock.Conclusions: The anesthesiologist should prepare for the critical complications of HAE and prepare the appropriate treatment options.

TEMPI syndrome: A clinical, light‐microscopic and phenotypic evaluation with review of the literature

“…Another potential mechanism involves the direct targeting by the paraprotein of a critical endothelial‐based protein that is critical for maintaining vascular integrity, a hypothesis that is supported by the extent of C5b‐9 deposition within the microvessels during an active leak phase of idiopathic systemic capillary leak syndrome. Perhaps, similar postulated mechanisms of enhanced vasopermeability seen in patients with idiopathic capillary leak syndrome could apply to TEMPI syndrome 30–38 …”

“…Perhaps, similar postulated mechanisms of enhanced vasopermeability seen in patients with idiopathic capillary leak syndrome could apply to TEMPI syndrome. [30][31][32][33][34][35][36][37][38] The majority of patients with TEMPI present with erythrocytosis resembling PV. These patients lack the JAK2 mutation, which allows the exclusion of PV.…”