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 hemophihia?” 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