Hemophilic Pseudotumor

Anderson, C. L. V.; Álvarez, Ofelia; Saigal, Gaurav; Davis, Joanna

doi:10.1097/mph.0000000000000187

Cited by 5 publications

(1 citation statement)

References 21 publications

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“…Doyle et al evaluated the available HPT treatment options associated with haemophilia [ 67 ]. The authors collected 48 articles: 40 single case reports, seven single centre case series, and one multicentre case series [ 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 ]. They concluded that “timely surgical intervention with adequate haemodynamic support and the consideration of adjuvant therapies in selected cases can achieve acceptable outcomes in this cohort of patients”.…”

Section: Resultsmentioning

confidence: 99%

Haemophilia and Cancer: A Literature Review

Zanon,

Porreca,

Simioni

2024

JCM

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Background: Opinions in the literature on the impact of cancer on patients with haemophilia are contradictory. There is a lack of data on the clinical presentation and management of cancer in patients with haemophilia (PWH). Methods: Papers were found following a comprehensive search in PubMed, Google Scholar, and Scopus using the terms “cancer” and “haemophilia” without time limits and using the English language as a filter. The references from all the retrieved original articles and reviews were assessed for additional relevant articles. Results: The emergence of malignancies is one of the important causes of morbidity and mortality in PWH. In the past decade, the literature mainly focused on the epidemiology and outcome of blood-borne cancers in the haemophilia patient group, as the incidence of hepatitis B virus (HBV), hepatitis C (HCV), and HIV infection were high among them. However, with the introduction of recombinant clotting factor concentrates (CFCs), physicians now pay attention to non-virus-related malignancies. Bleeding and thrombotic complications are important causes of morbidity and mortality in critically ill patients with cancer; replacement therapy with factor VIII or IX or others should be maintained during antitumour treatment. Conclusion: Overall, managing cancer in patients with haemophilia requires careful evaluation and individualised planning involving a multidisciplinary team of physicians experienced in haematology, oncology, and surgery.

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Section: Resultsmentioning

confidence: 99%

Haemophilia and Cancer: A Literature Review

Zanon,

Porreca,

Simioni

2024

JCM

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Successful resection of giant abdominal hemophilic pseudotumor

et al. 2019

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Giant abdominal hemophilic pseudotumor is exceedingly rare, thus may bring great challenges to the timely and proper diagnosis and treatment of clinicians. The only definitive management is complete removal of the abdominal hemophilic pseudotumor. The objective of this article is to report surgical treatment and follow-up outcomes of three unusual cases with giant abdominal hemophilic pseudotumor. We describe 3 patients with giant hemophilic pseudotumor involving the abdomen who were successfully treated with tumor resection. On presentation to our institution, the patients all had signs of giant cystic lesions in abdomen, and the patients’ most outstanding complaints were aggravated abdominal pain. All of three patients underwent complete excision of abdominal hemophilic pseudotumor. The patients showed adequate pain relief compared with the previous status. Surgical resection is the most effective treatment option for patients with giant abdominal hemophilic pseudotumor who can undergo appropriate surgical treatment. This represents a safe and reasonable approach to sustainably relieve pain and other symptoms with giant hemophilic pseudotumor in the abdomen. Perioperative coagulation factor replacement therapy is also of great significance in reducing the risks and complications.

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Characteristics and management of the haemophilia‐associated pseudotumours

2019

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| INTRODUC TI ONHaemophilia-associated pseudotumours (HPT) were first described by Starker in 1918 and are a continuing cause of severe morbidity and mortality for patients with congenital haemophilia. 1 The prevalence of HPT has been described between 1% and 2% of patients with haemophilia with similar levels persisting despite the use of recombinant factor replacement over the last decades. 2,3 The clinical course of HPT can be heterogenous due to a variety of factors including age of onset, disease severity, presence of an inhibitor and inadequate factor replacement. 4 They can result in a variety of problems including bleeding, organ compression and loss of function, bone destruction and fistula formation.HPT typically develops at a site of injury such as a bone fracture, or from spontaneous bleeding in subperiosteal and soft tissues. A preceding traumatic event has been described in 57%-75% of patients with HPT. 3,5 Subsequent bleeding, inadequate blood reabsorption and capsular formation around the haematoma lead to progressive enlargement of the HPT. This can ultimately lead to the eventual mass effect on local structures or bone and skin AbstractThe management of haemophilia-associated pseudotumours presents an ongoing challenge to the haematologist, surgeon and interventional radiologist alike. There is a range of therapeutic approaches including factor replacement, embolization, radiotherapy and a variety of surgical interventions. However, there remains little evidence regarding the most appropriate treatment. We aimed to evaluate the available options of management for the haemophilia-associated pseudotumour. A literature review was performed using relevant terminology and reviewed for treatment approaches and outcomes. The results demonstrated that most of the data is from single case reports with a small number of single-and multicentre case series. In total, 133 patients with 134 described pseudotumours were identified. Adequate haemostatic control with factor replacement was a key component to successful treatment. Surgical excision was the most commonly reported surgical intervention with various composites used for filling of the surgical cavity. The use of radiotherapy has been described particularly in the paediatric population and sites of difficult surgical access. Embolization can be considered as a method of presurgical optimization.Patients with both factor inhibitors and pseudotumours have poorer postoperative outcomes. This review demonstrates that although a lack of large-centre, randomized studies, timely surgical intervention with adequate haemostatic support and the consideration adjuvant therapies in selected cases can achieve acceptable outcomes in this cohort of patients. K E Y W O R D Scomplication, haemophilia A, haemophilia B, pseudotumour, surgery S U PP O RTI N G I N FO R M ATI O NAdditional supporting information may be found online in the Supporting Information section.

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Hemophilic Pseudotumor

Cited by 5 publications

References 21 publications

Haemophilia and Cancer: A Literature Review

Haemophilia and Cancer: A Literature Review

Successful resection of giant abdominal hemophilic pseudotumor

Characteristics and management of the haemophilia‐associated pseudotumours

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