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Case report of nasal pseudotumor – a rare presentation in severe haemophilia A with high titre inhibitors

Case report of nasal pseudotumor – a rare presentation in severe haemophilia A with high titre... AbstractHaemophilia patients with inhibitors suffer from increased morbidity and mortality due to the ineffectiveness of factor VIII replacement. Pseudotumors are rare but dangerous complications in these patients, and nasal pseudotumors are even rarer. Here, we present the case of a young child with severe haemophilia A with high titre inhibitors who developed a nasal pseudotumor. When immune tolerance therapy was not possible due to financial constraints, he was treated with FEIBA prophylaxis and rituximab. The pseudotumor was managed with surgical excision. We conclude that epistaxis in haemophiliacs can be due to an underlying nasal pseudotumor, and highlight the use of rituximab for the eradication of inhibitors. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png The Journal of Haemophilia Practice de Gruyter

Case report of nasal pseudotumor – a rare presentation in severe haemophilia A with high titre inhibitors

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Publisher
de Gruyter
Copyright
© 2021 Prakas Kumar Mandal et al., published by Sciendo
eISSN
2055-3390
DOI
10.17225/jhp00172
Publisher site
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Abstract

AbstractHaemophilia patients with inhibitors suffer from increased morbidity and mortality due to the ineffectiveness of factor VIII replacement. Pseudotumors are rare but dangerous complications in these patients, and nasal pseudotumors are even rarer. Here, we present the case of a young child with severe haemophilia A with high titre inhibitors who developed a nasal pseudotumor. When immune tolerance therapy was not possible due to financial constraints, he was treated with FEIBA prophylaxis and rituximab. The pseudotumor was managed with surgical excision. We conclude that epistaxis in haemophiliacs can be due to an underlying nasal pseudotumor, and highlight the use of rituximab for the eradication of inhibitors.

Journal

The Journal of Haemophilia Practice – de Gruyter

Published: Jan 1, 2021

Keywords: haemophilia with inhibitors; rituximab; nasal pseudotumor; surgical removal; case report

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