The rare coexistence of high titer inhibitor development and gastrointestinal stromal tumor in a patient with severe hemophilia: A case report

Yıldız, Abdülkerim; Şahin, Osman; Yayar, Okan; Öztürk, Çiğdem; Öztürk, Hacer Berna Afacan; Maral, Senem; Okutan, Harika; Albayrak, Murat

doi:10.1016/j.transci.2018.04.023

Cited by 1 publication

(2 citation statements)

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“…One case of femoral vein thrombosis was reported in a surgical patient with gastrointestinal malignancy after 27 days of rFVIIa without chemical thromboprophylaxis. This was managed by compression stockings and without anticoagulation 22 . There was no other risk factor for thrombosis in the patient, apart from a newly diagnosed gastrointestinal stromal tumour, which may be a predisposing factor for thrombosis, and the overtreatment with rFVIIa 22 .…”

Section: Results and Interpretationmentioning

confidence: 96%

“…This was managed by compression stockings and without anticoagulation 22 . There was no other risk factor for thrombosis in the patient, apart from a newly diagnosed gastrointestinal stromal tumour, which may be a predisposing factor for thrombosis, and the overtreatment with rFVIIa 22 . One patient who underwent liver transplantation had catastrophic bleeding associated with refractory micro‐angiopathy, which was suspected by the authors to be due to disordered thromboregulation with endothelial inflammation (the authors did not attribute rFVIIa as the primary cause of this development though in theory it may have contributed to disturbed coagulation imbalance).…”

Section: Results and Interpretationmentioning

confidence: 99%

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Recombinant FVIIa in elective non‐orthopaedic surgery of adults with haemophilia and inhibitors: A systematic literature review

Dolan¹,

Astermark

Hermans

2021

Haemophilia

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Aim To assess available evidence on the use of rFVIIa in non‐orthopaedic surgery including dental surgery in adult patients with congenital haemophilia with inhibitors (PWHI). Methods A systematic literature search was performed according to a prespecified search string; prespecified criteria were used to select applicable studies including PWHI ≥18 years of age who underwent any non‐orthopaedic surgery using rFVIIa. Results Thirty‐three publications met the eligibility criteria, of which 26 publications – including 46 procedures in 44 patients – were selected for the qualitative analysis. Most publications were case reports or case series (21/26). Primary authors assessed rFVIIa as effective in maintaining haemostasis during and after most major surgeries (22/32). rFVIIa dose was mainly on label, with higher doses used in 4 cases, and a lower dose in 1 case. Duration of treatment was mostly 5–10 days (range: 3 days to 1 month post‐operatively). Adverse events related to rFVIIa were rare. Conclusions Assessing non‐orthopaedic surgery in this patient population is hampered by a paucity of published data; nevertheless, the current evidence indicates that rFVIIa is effective in achieving haemostasis in haemophilia patients with inhibitors undergoing elective non‐orthopaedic or dental surgery. rFVIIa was generally well tolerated in these patients, with thrombotic events occurring rarely. These data, generated to help clinicians manage congenital haemophilia with inhibitors, highlight the need for more systematic reporting of rFVIIa and all other therapeutic agents in non‐orthopaedic surgery and dental surgery in patients with congenital haemophilia and inhibitors.

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Section: Results and Interpretationmentioning

confidence: 96%

Section: Results and Interpretationmentioning

confidence: 99%