Symposium: What Is Hemophilia?: Hemophilia: Specific Entity or Syndrome?

TUI)IES OF HEMOPHILIA have, ins the past-, helped greatly to solve the mansy intricacies of the blood coagulation mechanism. More recently, howeser, they have also altered our s-cry (‘onscept of the disease arid stnggested that, ins-stead of a svehl defined entity, hemophilia ins fact represents a syndrome siit.h many different pathogensetic mechanisms. The questions “i’hat. is hemo-phihia? ” is thus-s timely arid has assumed theoretic as well a-s practical importance. Ann analysis of the evolution ins the knsosvledge of hemophilia may, perhaps, help to furnish ans answer. Some of the genetic arid clinical (‘haradteritic of hemophilia sun(’h as the immurnity of females to the disease, sihich they are ises-erthehess capable of transmitting to male-s as a sex-linked recessise trait, ’ 2 arid the occunrrensce of ses-ere bleeding manifestations ins joints arid deep ti-s-sires, either sponstanseouis or fobloivinig minor traunma,3 has-c beers knsosvns for os’cr a censtury or so. The ability of trarssfursions of fresh blood and plasma to (‘Onitrol promptly lint temporarily the bleeding of hemophilia is, on the contrary, a recent acqunisitions. Earlier, A. E. Wright4 recognized the delay of the hibood (‘lotting