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Hereditary plasma clotting factor disorders and their management [Note 1. Treatment of Hemophilia Monograph Series, Number 4. World Federation ...]
Author(s) -
Kasper Ck
Publication year - 2000
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1046/j.1365-2516.2000.00042.x
Subject(s) - clotting factor , citation , medicine , boulevard , library science , family medicine , pediatrics , history , computer science , archaeology
Classic hemophilia is a sex-linked genetic disorder resulting in deficiency of plasma factor VIII (FVIII) coagulant activity. The level of FVIII is similar in all affected males in a given family. Hemophilia is often defined as severe, moderate or mild, according to the FVIII level. Patients with severe hemophilia have about one percent (one FVIII unit per deciliter) or less and tend to bleed frequently on minimal or unrecognized trauma, especially into joints or muscles. Patients with moderate hemophilia have about two to four FVIII U/dL and hemorrhage with moderate trauma. Persons with mild hemophilia have about 5 to about 30 FVIII U/dL and hemorrhage with severe trauma or at surgical operations. The average normal level of FVIII is 100 U/dL; the normal range is about 40-180 U/dL. Normal ranges differ according to ABO blood group for unknown reasons. The normal range extends down to about 40 U/dL in persons with type O red blood cells and down to about 5060 U/dL in persons of A, B or AB type red blood cells.