Antihaemophilic Globulin and Plasma Thromboplastin Component Deficiencies Associated with a Qualitative Platelet Disturbance

Platelet Coagulant Activities and Clinical Severity in Haemophilia

“…Contradictory evidence (Feissly and Fried 1924;Patek and Stetson 1936) indicated that a plasma factor, subsequently shown to be factor VIII (Quick 194 7; Brinkhous 194 7), was responsible for the apparent superiority of normal compared to haemophilic platelets in coagulation systems. Although it is now accepted that haemophilia is not a platelet disorder, occasional reports of platelet dysfunction in patients with haemophilia (Holburn et al 1957;Djerassi et al 1957;Eliakim 1962;Call et al 1969) have given rise to the suggestion that a qualitative platelet defect may occasionally be associated with haemophilia (Owen et al 1971).…”

Familial Multiple Coagulation Factor Deficiencies

“…Previous reports of patients with combined Fac tor VIII and Factor IX deficiencies can be divided into two groups: proven [14,33,61,65,66,73,75,84,114,126,130,131,133], and possible but unproven [15,30,56,85,102,112,122,123] cases. The diagnosis of dual deficiencies in proven cases was established by crossmatch studies with plasma from individuals with known Factor VIII or Factor IX deficiencies or by quantitative specific factor assays.…”

“…In addition to the cases of proven combined Fac tor VIII and Factor IX deficiency, there are a num ber of reports of patients claimed to have simul taneous deficiencies of the two factors, but with laboratory data inadequate to establish conclusively the diagnosis [15,30,56,85,102,122,123]. Diagnoses in these reports are based on the thromboplastin generation test (TGT) or prothrombin consumption test, without confirming crossmatch studies or specific factor assays.…”