Epsilon Amino Caproic Acid Therapy as a Cause of Intrarenal Obstruction in Haematuria of Haemophiliacs

Abstract

1. Three patients with classic haemophilia presented with a similar intrarenal obstructive pattern. Two of the patients had been treated for idiopathic haematuria. The third patient developed the renal obstructive problem in his post‐operative period. 2. The pyelography studies implicate an intrarenal unilateral obstructive mechanism in each patient as cause for abnormality. Intratubular fibrin deposition is suggested as a possibility. 3. E‐ACA was being used as a therapeutic endeavor in all these patients and was discontinued just prior to the development of symptoms. The evidence presented suggests that the intrarenal obstruction may be secondary to E‐ACA therapy rather than a complication of the bleeding disease.

Systematic review of hematuria and acute renal failure with tranexamic acid

“…27,28 Case reports of other non-TXA antifibrinolytic agents have also described an association with renal clot formation leading to renal obstruction. 29,30 A small RCT involving pediatric patients receiving TXA to reduce blood loss while undergoing ureteric re-implantation found that six out of ten patients receiving TXA developed clots in the urinary bladder compared with no patients in the control group. Two of the patients were noted to have "transient bladder retention," but the authors did not specifically describe any cases of renal failure.…”

“…The mechanism of renal injury was hypothesized to be from fibrin deposition and clot formation within the urinary tract leading to renal obstruction and failure 15‐17 or due to acute renal cortical necrosis 27,28 . Case reports of other non‐TXA antifibrinolytic agents have also described an association with renal clot formation leading to renal obstruction 29,30 . A small RCT involving pediatric patients receiving TXA to reduce blood loss while undergoing ureteric re‐implantation found that six out of ten patients receiving TXA developed clots in the urinary bladder compared with no patients in the control group.…”

Epsilon-Aminocaproic Acid (EACA)

“…Hematuria in patients with hemophilia is of particular interest. EACA has been reported to be frequently effective in the control of protracted spontaneous hematuria in these patients [32,48], but reports of renal and ureteral obstruction with clots, occasionally fatal, have become numerous [41,[49][50][51]. However, Prentice [21] and others [52] have observed a high rate of spontaneous urinary tract clot formation in hemophiliacs in the absence of EACA therapy.…”

“…Treatment of hematuria with EACA deserves special mention because of numerous case reports of intrarenal or ureteral obstruction with clots, particularly in hemophiliacs [41,48,49,[96][97][98]. This complication is often heralded by cessation of hematuria with development of flank pain.…”

A review of hyperfibrinolysis in cats and dogs

“…Empirical use of these drugs is therefore not recommended in patients with prothrombotic conditions. Caution is also advised in the use of antifibrinolytic agents in cats and dogs with renal haemorrhage due to the risk of clot formation causing intra-renal and ureteric obstruction (Stark et al 1965, Vujkovac & Sabovic 2006. Both tranexamic acid and EACA are primarily excreted by the kidneys, and, in people with renal impairment, tranexamic acid administration is associated with seizures (Montes et al 2012).…”