हिमोफिलिया व रक्तस्राव विकार: छोट्या जखमेनंतर दीर्घकाळ रक्तस्राव, सांध्यांमध्ये सूज व दुखणे (विशेषतः मुलांमध्ये), दात काढल्यानंतर न थांबणारे रक्त – ही हिमोफिलियाची लक्षणे आहेत. योग्य फॅक्टर उपचाराने सांध्यांचे नुकसान टाळता येते.
हीमोफीलिया और रक्तस्राव विकार: छोटी चोट के बाद देर तक खून बहना, जोड़ों में सूजन और दर्द, दांत निकालने के बाद खून न रुकना – हीमोफीलिया के लक्षण हैं।
Haemophilia is an inherited bleeding disorder, mostly in boys, caused by a lack of clotting Factor VIII (haemophilia A) or Factor IX (haemophilia B). Other bleeding disorders include von Willebrand disease and platelet function disorders such as Glanzmann thrombasthenia.
Symptoms
- Prolonged bleeding after cuts, circumcision, tooth extraction or surgery
- Painful swelling of joints (knee, elbow, ankle) and muscles
- Easy bruising; heavy periods in women with bleeding disorders
Diagnosis
Coagulation tests (PT, aPTT), factor assays, von Willebrand tests, and inhibitor testing.
Treatment
- Factor replacement – on-demand and prophylaxis to prevent joint damage
- Newer therapies such as emicizumab (a weekly or monthly injection under the skin)
- Physiotherapy, management of "target joints" and safe planning of surgery and dental work
- Guidance on accessing factor concentrates through government haemophilia programmes