Hemophilia Research Today is a free monthly online journal that collates and summarizes the latest research about Hemophilia, including details on genetics, causes, symptoms, blood transfusion.

Hemophilia and cancer: a new challenge for hemophilia centers.

Franchini M, Lippi G, Montagnana M, Targher G, Zaffanello M, Salvagno GL, Rivolta GF, Perna CD, Tagliaferri A

Immunohematology and Transfusion Center - Department of Pathology and Laboratory Medicine, University Hospital of Parma, Parma, Italy. massimo.franchini@azosp.vr.it

The improved life expectancy of hemophilia patients due to the advances in hemophilia care and factor replacement therapy has permitted to hemophiliacs to reach an older age. As a consequence, age-related diseases, such as cardiovascular disorders and cancer, have been increasingly recognized in such patients. In particular, the management of cancer in people with inherited hemorrhagic disorders represents a new challenge for physicians working in hemophilia centers. The few published literature data document that there is a close relationship between hemophilia and neoplasia. Indeed, the congenital bleeding tendency may influence the cancer in different ways, by interfering with its clinical presentation, diagnosis and treatment. These aspects, along with the epidemiology of cancer in hemophiliacs will be discussed in this review.

Published 25 May 2009 in Cancer Treat Rev, 35(4): 374-7.
Full-text of this article is available online (may require subscription).

Articles on Hemophilia published 5 May 2009:

Bacterial endocarditis and septic arthritis in a patient with severe hemophilia A: a case report.   J Pediatr Hematol Oncol, 31(5): 344-5.

Central venous access devices are commonly used in the care of pediatric patients with hemophilia. Bacteremia associated with this type of venous access is common. We describe a patient with severe hemophilia A who had development of septic arthritis and endocarditis secondary to multiple episodes of bacteremia associated with a central venous access device. Endocarditis and septic arthritis in patients with hemophilia are rare infectious complications but should be considered in patients with ... [Abstract] [Full-text]

Articles on Hemophilia published 17 April 2009:

Successful treatment of canine hemophilia by continuous expression of canine FVIIa.   Blood, 113(16): 3682-9.

Continuous expression of activated factor VII (FVIIa) via gene transfer is a potential therapeutic approach for hemophilia patients with or without inhibitory antibodies to human factor VIII (FVIII) or IX (FIX). Here, we investigate whether gene transfer of an engineered canine FVIIa (cFVIIa) transgene can affect hemostasis in a canine model of hemophilia, a good predictor of efficacy of hemophilia treatments. Purified recombinant cFVIIa exhibited 12-fold higher tissue factor-dependent activity ... [Abstract] [Full-text]

Articles on Hemophilia published 16 April 2009:

Inhibitors of factor VIII in black patients with hemophilia.   N Engl J Med, 360(16): 1618-27.

BACKGROUND: Black patients with hemophilia A (factor VIII deficiency) are twice as likely as white patients to produce inhibitors against factor VIII proteins given as replacement therapy. There are six wild-type factor VIII proteins, designated H1 through H6, but only two (H1 and H2) match the recombinant factor VIII products used clinically. H1 and H2 are found in all racial groups and are the only factor VIII proteins found in the white population to date. H3, H4, and H5 have been found only ... [Abstract] [Full-text]

Articles on Hemophilia published 17 February 2009:

Immunosuppressive treatment for acquired haemophilia: current practice and future directions in Germany, Austria and Switzerland.   Ann Hematol, 88(4): 365-70.

Acquired haemophilia is an autoimmune disorder characterised by autoantibody formation against coagulation factor VIII. Immunosuppressive treatments including steroids, cytotoxic drugs, rituximab or combinations thereof have been used to eradicate autoantibodies. Very few prospective studies exist evaluating the use of these treatments. Here, we performed a survey among 73 physicians from 57 haemophilia treatment centres in order to describe current practice patterns and critical issues for ... [Abstract] [Full-text]

Articles on Hemophilia published 2 February 2009:

The treatment of hemophilia A: from protein replacement to AAV-mediated gene therapy.   Biotechnol Lett, 31(3): 321-8.

Factor VIII (FVIII) is an essential component in blood coagulation, a deficiency of which causes the serious bleeding disorder hemophilia A. Recently, with the development of purification level and recombinant techniques, protein replacement treatment to hemophiliacs is relatively safe and can prolong their life expectancy. However, because of the possibility of unknown contaminants in plasma-derived FVIII and recombinant FVIII, and high cost for hemophiliacs to use these products, gene therapy ... [Abstract] [Full-text]

Articles on Hemophilia published 21 January 2009:

Phenotypic correction of murine hemophilia A using an iPS cell-based therapy.   Proc Natl Acad Sci U S A, 106(3): 808-13.

Hemophilia A is caused by mutations within the Factor VIII (FVIII) gene that lead to depleted protein production and inefficient blood clotting. Several attempts at gene therapy have failed for various reasons-including immune rejection. The recent generation of induced pluripotent stem (iPS) cells from somatic cells by the ectopic expression of 3 transcription factors, Oct4, Sox2, and Klf4, provides a means of circumventing the immune rejection barrier. To date, iPS cells appear to be ... [Abstract] [Full-text]

Articles on Hemophilia published 19 January 2009:

Preimplantation genetic diagnosis of haemophilia.   Br J Haematol, 144(3): 303-7.

Preimplantation genetic diagnosis (PGD) aims to increase the number of options available to couples who could have a child affected with haemophilia and reduce the anxiety these couples often associate with reproduction. The female partner must undergo an in vitro fertilization cycle, and the eggs or embryos are then biopsied. Embryos which are unaffected by haemophilia can then be transferred to the uterus. The clear advantage of this technique is that the woman knows from the very beginning ... [Abstract] [Full-text]

Articles on Hemophilia published 5 November 2008:

Ultrasound and computed tomography findings of spontaneous intramural hemorrhage of renal pelvis and ureter in patient with hemophilia a.   Urology, 72(5): 1030-2.

Intramural renal pelvic and ureteral hemorrhage is seen most commonly in patients treated with anticoagulant therapy. However, spontaneous intramural hemorrhage of the ureter seen in patients with hemophilia is a rare entity and has been reported only in 2 cases. Computed tomography is a valuable imaging method in the diagnosis and follow-up. We report the ultrasound and computed tomography findings of spontaneous intramural renal pelvic and ureteral hemorrhage in a patient with hemophilia A. [Abstract] [Full-text]

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