Meet the editor

Dr. Pankaj Abrol is presently a professor and the head of the Department of Pediatrics, SGT Medical College Hospital & Research Institute, Gurgaon, India. He is a former senior professor and head of Pediatrics-II in the Postgraduate Institute of Medical Sciences, Rohtak, where he was Chief Pediatric Hematologist Oncologist, and in charge of Thalassemia & Hemophilia Day Care Centers. He is an active participant in the hemophilia train-

ing program in India and initiated free treatment of hemophilia with factors in the state of Haryana. He established a pediatric cancer unit after getting trained at Tata Memorial Hospital, Mumbai, India. He obtained the MBBS and MD (Pediatrics) degrees from the Institute of Medical Sciences, Banaras Hindu University, Varanasi. He is a resource person for pediatric oncology, thalassemia, and hemophilia at state as well as national level. He has many publications in the field and is a reviewer for various journals in pediatrics and pediatric hematology oncology. His exemplary services in the field of pediatrics and pediatric hematology oncology won him the Fellowship of Indian Academy of Pediatrics (FIAP). He has more than 35 years of experience teaching many graduates and postgraduates.

**Preface III**

**Chapter 1 1**

**Chapter 2 9**

**Chapter 3 35**

**Chapter 4 55**

Perioperative Management of Hemophilia A Using Recombinant Factor

*Diana Ornelas-Ricardo, Clara-Ibet Juárez-Vázquez and Hilda Luna-Záizar*

*by Atsushi Okamoto, Kenta Yamamoto, Go Eguchi, Yoshitaka Kanai,* 

Clinical Issues in Women with Inherited Bleeding Disorders *by Ana-Rebeca Jaloma-Cruz, Isaura-Araceli González-Ramos,* 

Introductory Chapter: Hemophilia

Genotype-Phenotype Heterogeneity in Haemophilia *by Muhammad Tariq Masood Khan and Abid Sohail Taj*

VIII in Patients Undergoing Major or Minor Surgery

*Terufumi Yamaguchi and Yasuhiro Maeda*

*by Pankaj Abrol*

Contents

## Contents


Preface

Hemophilia is a rare inherited disease due to a deficiency of coagulation factors VIII and IX. It was initially detected in the royal families of the UK, Russia, Spain, and Germany; hence, it is labeled a royal disease. Though known since biblical times, the term hemophilia was ascribed to Schonlein in the 1820s. Low levels of factor VIII were identified in 1947. Decreased levels of factor IX were shown in Hemophilia B in 1952. A lot of knowledge was acquired later and management progressed from plasma to FFP to cryoprecipitate to purified plasma-derived factors to recombinant factors—first, second, and third generation. There had been significant improvement in first aid measures for the management of multiple complications like arthropathy and the development of inhibitors. Improvement in treatment has seen various phases—from general therapy with plasma to specific therapy with recombinant factors. Now efforts are being made to prolong the half-

life of factors VIII and IX to genetic therapy by targeting the affected gene.

poor and needy patients.

postpartum.

It gives me immense pleasure to be associated with and edit a book on hemophilia, a disease for which research is making rapid advances. I have been associated with hemophilia for a long time and have organized hemophilia care in the state of Haryana in India and initiated steps for the free supply of factors VIII and IX for

This book covers various important aspects of hemophilia management. State-ofthe-art chapters have been written by various authors. The chapter on genotypephenotype correlation describes the changing concepts of multiple mutations in hemophilia and emphasizes the role of gene-environment interaction. In the chapter on the perioperative management of hemophilia A, authors have discussed intraoperative and postoperative factor replacement therapy in hemophilia A patients undergoing minor and major surgeries. Factor replacement in hemophilia is discussed in detail. Hemophilia A and B being X-linked disorders are by and large considered as strictly affecting the male population. The chapter on women's issues in inherited bleeding disorders highlights that a significant number of heavy menstrual bleeders can be hemophilia A and B carriers, in addition to more common causes of von Willebrand disease (VWD), platelet disorders, and some rare bleeding disorders. This can pose serious life threats, especially after surgery or

This book on hemophilia highlights the latest literature and many important aspects of this disease. A few decades ago, little was known about hemophilia. At present, we are able to save many lives and also alleviate morbidity and prevent disability due to rapid advances in first aid measures, physical therapy, and prophylaxis. Prophylaxis will undoubtedly prove the turning point in hemophilia management.
