Edited by Muhammad Tariq Sohail and Tahir Shamsi pp. 278 Lahore, Pakistan: Ferozsons, 2010 ISBN: 978-969-0-02272-1
Major advances in haemophilia therapy have had a very positive impact on results in the more affluent countries which are able to afford coagulation factor concentrates. Life expectancy is virtually normal and the introduction of recombinant concentrates has eliminated the risk of transmission of pathogens such as the viruses of hepatitis and HIV. Prophylactic administration of factor VIII two or three times a week prevents recurrent haemarthrosis and the development of crippling deformities which would otherwise inevitably develop by early adulthood. By contrast, the situation in poorer countries remains dire. This is primarily due to the high cost of the necessary blood products. The World Federation of Hemophilia (WFH) estimates that some two thirds of the estimated 340 000 patients with haemophilia still receive no proper treatment.
Professor Sohail has assembled an impressive panel of 62 contributors from all over the world. Most are members of the very active Musculoskeletal Committee of the WFH and have very extensive practical experience of the management of the disease. This authoritative text is very much a practical handbook, covering every aspect of haemophilia care, in a logical sequence. Many surgeons in Europe will rarely face the complications which are regularly seen in poorer countries, including contractures, ankylosed joints and pseudotumours. The surgical aspects of these topics are covered in detail, as well as conservative modes such as physiotherapy and orthotics. As a consequence of the lack of proper blood products, orthopaedic surgeons in the developing world have been forced to develop innovative therapies; it is interesting to see that many such procedures are now increasingly adopted in the developed world. The techniques described in detail include synovectomy using radionuclides and chemicals such as rifampicin. A point the authors make repeatedly is that patients should be treated in dedicated specialist centres by a multidisciplinary team in order to guarantee the requisite collective experience in treating this rare condition. Guidance is also provided on the selection and appropriate dosage of coagulation factor concentrates.
The pitfalls of operating on patients with inhibitors to factor VIII are rightly emphasised. Up-to-date literature citations at the end of each chapter will help readers to find more detailed information. This comprehensive and authoritative book contains a wealth of practical advice and information. It will be of interest to haematologists and physiotherapists as well as orthopaedic surgeons. Although it is focused on the developing world, the many surgeons working in rich countries, who work abroad to support the numerous ongoing WFH international programmes, would be well advised to read this book.