Anupam Sachdeva1, Vinod Gunasekaran2, H N Ramya2, Jasmita Dass2, Jyoti Kotwal2, Tulika Seth3, Satyaranjan Das4, Kapil Garg5, Manas Kalra6, Rani S Sirisha7, Anand Prakash8. 1. Sir Ganga Ram Hospital, New Delhi, India. Correspondence to: Dr. Anupam Sachdeva, Director, Pediatric Hematology Oncology and Bone Marrow Transplantation unit, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi 110 060, India. anupamace@yahoo.co.in. 2. Sir Ganga Ram Hospital, New Delhi, India. 3. All India Institute of Medical Sciences, New Delhi, India. 4. Army Hospital Research and Referral, New Delhi, India. 5. Sawai Man Singh Medical College, Jaipur, India. 6. Indraprastha Apollo Hospitals, New Delhi, India. 7. Rainbow Children's Hospital, Hyderabad, India. 8. St Johns Medical College Hospital, Bangalore, India.
Abstract
JUSTIFICATION: Despite having standard principles of management of hemophilia, treatment differs in various countries depending on available resources. Guideline for management of hemophilia in Indian setting is essential. PROCESS: Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expert committee members reviewed the final manuscript. OBJECTIVE: To bring out consensus guidelines in diagnosis and management of Hemophilia in India. RECOMMENDATIONS: Specific factor assays confirm diagnosis and classify hemophilia according to residual factor activity (mild 5-40%, moderate 1-5%, severe <1%). Genetic testing helps in identifying carriers, and providing genetic counseling and prenatal diagnosis. Patients with hemophilia should be managed by multi-specialty team approach. Continuous primary prophylaxis (at least low-dose regimen of 10-20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response. Factor replacement remains the mainstay of treating acute bleeds (dose and duration depends on body weight, site and severity of bleed). Factor concentrates (plasma derived or recombinant), if available, are preferred over blood components. Other supportive measures (rest, ice, compression, and elevation) should be instantly initiated. Long-term complications include musculoskeletal problems, development of inhibitors and transfusion-transmitted infections, which need monitoring. Adequate vaccination of children with hemophilia (with precautions) is emphasized.
JUSTIFICATION: Despite having standard principles of management of hemophilia, treatment differs in various countries depending on available resources. Guideline for management of hemophilia in Indian setting is essential. PROCESS: Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expert committee members reviewed the final manuscript. OBJECTIVE: To bring out consensus guidelines in diagnosis and management of Hemophilia in India. RECOMMENDATIONS: Specific factor assays confirm diagnosis and classify hemophilia according to residual factor activity (mild 5-40%, moderate 1-5%, severe <1%). Genetic testing helps in identifying carriers, and providing genetic counseling and prenatal diagnosis. Patients with hemophilia should be managed by multi-specialty team approach. Continuous primary prophylaxis (at least low-dose regimen of 10-20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response. Factor replacement remains the mainstay of treating acute bleeds (dose and duration depends on body weight, site and severity of bleed). Factor concentrates (plasma derived or recombinant), if available, are preferred over blood components. Other supportive measures (rest, ice, compression, and elevation) should be instantly initiated. Long-term complications include musculoskeletal problems, development of inhibitors and transfusion-transmitted infections, which need monitoring. Adequate vaccination of children with hemophilia (with precautions) is emphasized.