Acquired factor X deficiency in systemic amyloidosis: management of two cases

Chaudhary, Preeti; Wakim, Jad; Khadim, Haider; Karumbaiah, K. K.; Gajra, Ajeet

doi:10.1111/hae.12232

Cited by 2 publications

(3 citation statements)

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Section: Discussionmentioning

confidence: 99%

“…To overcome the unwanted increase in prothrombin levels, the use of bypassing agents such as rFVIIa in patients with acquired factor X deficiency and amyloidosis has been reported . Adequate hemostasis was achieved following the administration of rFVIIa in two patients presenting with life‐threatening bleed . In another case series where rFVIIa was used preoperatively in nine procedures, there were four postprocedural complications of bleeding, thrombosis, or death .…”

Section: Discussionmentioning

confidence: 99%

“…Currently, there are no evidence‐based guidelines on the optimal management for the prevention and treatment of bleeding events in this patient population. Therapeutic options include the use of fresh frozen plasma (FFP), prothrombin complex concentrates (PCCs), activated PCCs (aPCCs), recombinant factor VIIa (rFVIIa), and factor X concentrates . Even so, achieving adequate hemostasis continues to be a challenge.…”

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confidence: 99%

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Importance of pharmacokinetic studies in the management of acquired factor X deficiency

Lim

McCarthy

Chen

et al. 2015

European J of Haematology

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Up to 14% of individuals with systemic AL amyloidosis develop acquired factor X deficiency, which occurs due to adsorption of factor X onto amyloid fibrils. Although baseline factor X levels are not predictive of bleeding risk in these patients, serious hemorrhagic complications can occur, particularly during invasive procedures. Optimal management strategies to attenuate bleeding risk in these patients are unknown. We describe our experience in the management of acquired factor X deficiency, secondary to systemic AL amyloidosis, in a case series of three patients who received prothrombin complex concentrates (PCCs) for treatment and prevention of bleeding events. We performed a retrospective review extracting information on baseline demographics, laboratory data, pharmacokinetic (PK) studies, and clinically documented bleeding events. Our case series demonstrates that individuals with acquired factor X deficiency secondary to amyloidosis have variable laboratory and clinical responses to PCCs. This is likely due to distinct amyloid loads and fibril sequences, leading to different binding avidities for factor X. Our data emphasize the importance of performing PK testing prior to any invasive procedures to determine the dose and frequency interval to achieve adequate factor X levels for hemostasis, given the variable response between individuals.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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