CASE 2: EDWARD B.
Edward B. is a 64-year-old male with mild hemophilia A (FVIII 30%) that was diagnosed in childhood. He experiences bleeding episodes only infrequently and treats them on demand with factor concentrate. He visits his hemophilia treatment center (HTC) regularly for checkups. He recently presented to the emergency department of a local hospital with chest pain at rest. Although there is no family history of cardiovascular disease, Edward takes irbesartan 150 mg daily for stage 1 hypertension (BP 150/95) and atorvastatin 20 mg daily for borderline high cholesterol. Based on results of a stress test, which reveals significant narrowing in the left anterior descending artery, he is scheduled for an angiography and percutaneous coronary intervention (PCI).
The advent of HAART (highly active antiretroviral therapy) has significantly improved the survival of HIV-infected hemophilia patients. Up to one-third of patients live 20 to 25 years with the virus.10
However, an overwhelming majority of older hemophilia patients must also contend with the comorbidity of HCV infection, which is associated with reduced HCV clearance, leading to increased hepatic inflammation and accelerated progression to cirrhosis and liver failure.10, 15
Up to 98% of patients treated with large pool plasma-derived factor concentrates pre-1985, before development of viral inactivation, are infected with HCV, and 80% of these patients developed a chronic form of the virus.8
HCV is a major cause of chronic liver disease, liver failure, liver transplantation, and other complications in hemophilia patients and a leading cause of death.8, 9
HAART, beneficial for extending the lives of HIV-infected hemophilia patients, is also a contributor to liver disease in this cohort, and its use is associated with end-stage liver disease (ESLD) in HCV/HIVcoinfected patients.10
Cirrhosis, a potential complication of HCV, develops in approximately 20% to 30% of infected patients, with a rapid rise in incidence 15 years postinfection.9
Evidence suggests that patients in this cohort are also at risk for thrombocytopenia. A landmark study conducted by Eyster et al (1985) reveals that the platelet disorder is strongly associated with liver disease, which was a leading cause of death in the study population of hemophilia patients observed for 5 or more years.23
With increasing age, hemophilia patients will develop malignancies. These patients have the same cancer mortality rates as the general population, with the exception of hepatocellular carcinoma, the leading cause of death in this cohort.8, 10, 18
However, survival increased from 1992 to 2004, with the greatest improvement seen in those with localized disease and in patients receiving therapy.15
Older patients with hemophilia are also at increased risk for HIV-associated non-Hodgkin lymphoma. However, use of HAART has resulted in substantial reductions in incidence and mortality rates associated with this malignancy.15
Based on results of a CDC-sponsored surveillance study, patients with hemophilia have a 50-fold increased risk of death from renal disease compared to the general population.20
Risk factors include HIV infection, hypertension, and kidney bleeding in chronic renal disease.15
HIV infection, too, is associated with a variety of acute and chronic renal diseases, such as HIV-associated nephropathy and immune complex glomerulonephritis.15
Nephrotoxicity of HAART and HCV coinfection also contribute to renal disease in HIV-positive hemophilia patients.15