Cancer detection and management in patients with haemophilia: a retrospective European multicentre study

Abstract

Lymphomas or hepatocarcinomas related to blood-borne transmitted diseases are well-known malignancies in persons with haemophilia (PWH). However, rising life expectancy has increased the number of PWH suffering from other malignancies. This study aimed to collect cancer occurrence data in PWH followed in five European haemophilia treatment centres (Brussels, Geneva, Marseille, Montpellier and Paris-Bicêtre) over the last 10 years and to analyse some particular features of cancer occurring in PWH. In total, 45 malignancies were diagnosed in 1067 PWH. The most common malignancies were hepatocellular carcinoma (12/45) and urogenital tract tumours (9/45). Bleeding at presentation or changes in bleeding pattern was indicative of cancer in four patients. Three patients with mild haemophilia developed anti-factor VIII inhibitors after intensive substitution therapy prior to surgery or invasive procedures. There was no bleeding associated with chemotherapy or radiotherapy. A few bleeding complications occurred following invasive (3/39) or surgical procedures (2/27) as a result of insufficient hemostatic coverage or in spite of adequate substitution. No bleeding was noted after liver or prostate biopsies. Following cancer diagnosis, five patients were switched from on-demand to prolonged prophylaxis substitution. In the majority of cases, the standard cancer treatment protocol was not modified on account of concomitant haemophilia. Thus, oncological treatments are not contraindicated and should not be withheld in PWH assuming that adequate haemostasis correction is undertaken. As shown by our study results, a change in bleeding pattern in adult PWH should raise suspicion of a malignancy. Intensive substitution must be considered a risk factor for inhibitor development.

Incidence and survival of cancers among 1,054 hemophilia patients: A nationwide and 14‐year cohort study

“…As recombinant coagulation concentrates were commercialized, life expectancy increased in persons with hemophilia (PWH) ; however, health care providers are facing new challenges, in particular age‐related comorbidities . Malignancies, along with cardiovascular events and diabetes, have emerged as one of the important causes of morbidity and mortality in PWH . Most articles published in the previous decades focused on the epidemiology and outcome of blood‐borne cancers in this patient group, as the incidence of HCV and HIV infection were high among these patients .…”

Gastric Cancer, Hemophilia A and Angiodysplasia - An Unreported Association with Clinical Implications in a Patient with Digestive Hemorrhage

“…The incidence of malignancies in patients with hemophilia is higher, compared with general population, but most patients have virus-related cancers (22)(23)(24), because prevalence of hepatitis B, C and human immunodeficiency virus infection is higher, compared with the general population (23). Thus, hepatocellular carcinoma, leukemia and lymphoma are the most frequent types of cancers in patients with hemophilia (23,24).…”

“…Increased bleeding tendency should raise suspicion of underlying malignancy in an adult patient with hemophilia (22). Radical surgery and other forms of oncological therapy are not contraindicated in patients with hemophilia and malignancies (22).…”

“…Increased bleeding tendency should raise suspicion of underlying malignancy in an adult patient with hemophilia (22). Radical surgery and other forms of oncological therapy are not contraindicated in patients with hemophilia and malignancies (22). However, surgery of malignant tumors in patients with hemophilia is associated with increased risk of postoperative complications, including bleeding, despite proper replacement therapy (27).…”

Management strategies in patients with diffuse large B‐cell lymphoma and severe haemophilia

“…Four were reported to have bleeding complications, including three prior to diagnosis and one following a biopsy. Interestingly, no bleeding events occurred during chemotherapy or in the setting of subsequent cytopenias [5]. The transplanted lymphoma patients identified in the literature review had plasmablastic lymphoma and mantle cell lymphoma.…”