Background: Pseudotumors are a rare complication (1-2%) of Hemophilia. They present as chronic, slowly expanding, encapsulated cystic masses, and most are located in the long bones, pelvis and maxillofacial region. Pseudotumors pose significant morbidity and management challenges.

Aims: Systematic review of literature to identify incidence, presentation, management and outcome of Hemophilia patients with pseudotumors.

Methods: Literature search (1961‐2019) on pseudotumor formation in Hemophilia A and B patients was done using PubMed, Embase and Cochrane. The search strategies consisted of MeSH headings, keywords and text words related to hemophilia pseudotumor management. The results are expressed as P value with 95% Confidence Interval.

Results: 92 articles met our inclusion criteria (15 case series, 68 case reports and 9 review articles). 125 Hemophilia patients with pseudotumor formation were identified. Mean age was 28.5 years. Presenting features included large tumors of long bones (57%), abdominal or pelvic mass(19%), mass in the maxillofacial region(24%). Diagnostic modalities included X-ray, ultrasonography, Computed Tomography (CT) scan and Magnetic Resonance Imaging (MRI).16% of pseudotumors responded to conservative treatment with FVIII concentrates (P=0.02), 64% required surgical intervention i.e arterial embolization and surgical excision (P= 0.016), and 12% were treated with radiotherapy (P=0.03). In 15% of patients with masses in long bones, amputation was done due to intractable bleeding. Recurrence over 1 year period was 14%.

Conclusions: Although rare, pseudotumors pose significant morbidity in Hemophilia. It is always best to prevent these complications rather than trying to salvage later. Most of the developing countries lack the economic resources to meet the factor VIII needs of their Hemophilia patients. The need of the hour is to manage these patients early in specialized inter-disciplinary clinics designed specifically to meet the needs of hemophilic population.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/incidence-presentation-and-management-of-pseudotumors-in-hemophilics-a-systemic-review/