Indan Journal of Medical Research Indan Journal of Medical Research Indan Journal of Medical Research Indan Journal of Medical Research
  Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login  
  Home Print this page Email this page Small font sizeDefault font sizeIncrease font size Users Online: 385       

   Table of Contents      
BOOK REVIEW
Year : 2013  |  Volume : 138  |  Issue : 3  |  Page : 436-437

Hemophilia and hemostasis: A case-based approach to management


National Institute of Immunohaematology (ICMR), 13th Floor, New Multistoried Building, K.E.M. Hospital Campus, Parel, Mumbai 400 012, India

Date of Web Publication8-Oct-2013

Correspondence Address:
Kanjaksha Ghosh
National Institute of Immunohaematology (ICMR), 13th Floor, New Multistoried Building, K.E.M. Hospital Campus, Parel, Mumbai 400 012
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


Rights and PermissionsRights and Permissions

How to cite this article:
Ghosh K. Hemophilia and hemostasis: A case-based approach to management. Indian J Med Res 2013;138:436-7

How to cite this URL:
Ghosh K. Hemophilia and hemostasis: A case-based approach to management. Indian J Med Res [serial online] 2013 [cited 2019 Sep 15];138:436-7. Available from: http://www.ijmr.org.in/text.asp?2013/138/3/436/119391

Hemophilia and hemostasis: A case-based approach to management, 2 nd ed., A.D. Ma, H.R. Roberts, M.A. Escobar, editors (Wiley-Blackwell, UK). 2013. 224 pages. Price: not mentioned.

ISBN 978-0-470-65976-2


Blood coagulation is an important but complex biological phenomenon. Defects in blood coagulation have two important facets- haemorrhage and thrombosis. Both these pathologies can be caused by genetic and acquired mechanisms. Most common genetic condition leading to disorder in blood coagulation and haemorrhage is haemophilia. However, there are many other genetic defects of various coagulation factor genes and platelet related genes associated with thrombohaemorrhagic phenomenon.

The present book is in its second edition and the authors are leading experts in the field. The get up of the book is nice and the book is compact and handy.

The subject matter of inherited bleeding and thrombotic disorders and their management has been presented through 60 well selected cases. The language is lucid and easy flowing. As the individual cases have been discussed, the advantage of reading this book is that it can be read in any order. Each case management is complete by itself, and so is the investigation. Authors have been careful to include routine day-to-day investigations to arrive at the diagnosis. Special diagnostic tests have also been mentioned.

Important complications of haemophilia which affect 15-30 per cent of haemophilia A as well as 1-3 per cent of haemophilia B population have been discussed well and the management of bleeding in such patients has also been discussed. One would have expected a chapter on how major emergency operations in haemophilia patients with very high levels of inhibitor can be conducted safely; however, such a case was not discussed. A few cases have been discussed where more than one inherited conditions of bleeding disorder exist. Often the cases are not only difficult to diagnose but these also pose substantial problem in their management. Certain unusual cases like amyloid related factor X deficiency, heparin like substance in monoclonal gammopathy as a cuase of coagulation defect and a chapter on acquired Von Willebrand disease syndrome (AVWS) have enriched this book. Photographs are in black and white and are clear and relevant. As haemophilia and Von Willebrand disease are relatively common congenital bleeding disorders, enough data in the world literature exist on the biology of these diseases and management. Rare congenital bleeding disorder is a different game altogether. These disorders are uncommon (<1x10 6 ) and are often concentrated in those parts of the world where consanguineous marriages are common. Factor V deficiency, prothrombin deficiency, factor VIII deficiency, factor X deficiency have been discussed from the management perspective as individual cases.

Reproductive option in haemophilia patient is an important chapter, particularly for people with haemophilia (PWH) in developing countries where management options are restricted. Hence, carrier detection and prenatal diagnosis form important components of the management. Mild/moderately severe PWH with discrepant factor VIII levels may not have been picked up in developing countries because chromogenic assays are not routinely used for factor VIII level determination.

Thrombosis and thrombophylaxis in haemophilia patients are becoming increasingly relevant and difficult management decisions as haemophilia patients are living longer and are now developing heart disease, atherosclerosis, malignancy, etc. They also need hip/knee replacement therapy where thromboprophylaxis is also relevant. Familial thrombophilia is an important addition in this book so also Bayesian technique of risk assessment in bleeding disorder.

There are very few shortcomings in this book. One would have liked to see some more discussion on modern thrombin inhibitors and factor Xa inhibitors for management of thrombophilia. Hereditary platelet defects and some of the important challenges on blood coagulation have not received much attention though discussion on bleeding in patients with prostatic malignancy has been included. A chapter on pharmcokinetics of factor concentrates and their application in the management of difficult cases in haemophilia is appreciated but recent development of long acting clotting factors and prophylaxis in this condition has not received attention.

Overall, this is an extremely well written book suitable for consultants, trainees and, postgraduates, and is a must for all medical college libraries.




 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article

 Article Access Statistics
    Viewed456    
    Printed10    
    Emailed0    
    PDF Downloaded223    
    Comments [Add]    

Recommend this journal