HEMOPHILIA. 819 



CHAPTER V. 

 HAEMOPHILIA (HJEMOKBHAGIC DIATHESIS). 



ETIOLOGY. The names haemophilia, haemorrhaphilia, are applied 

 to a congenital haemorrhagic diathesis distinguished either by the un- 

 usual obstinacy of traumatic haemorrhage or a tendency to sponta- 

 neous bleedings. Hitherto no abnormity capable of accounting for the 

 symptoms has been detected either in the blood or the vascular walls 

 of the patient, although in a small number of cases the walls of the 

 blood-vessels have been found to be remarkably thin and delicate. In 

 most cases of haemophilia the disease is hereditary, that is, the patient 

 descends from a family one or more of whose members in preceding 

 generations have suffered from the same affection. There are instances 

 in which it has been transmitted through four generations ; others in 

 which a generation has been "skipped," the grandchildren having 

 haemophilia, but not the children. It is rare for every member of a 

 family to inherit this dangerous disorder, and the daughters seem to 

 remain free from it with especial frequence. There also are well- 

 authenticated observations which leave no doubt of the occurrence of 

 congenital haemophilia not dependent upon hereditary predisposition. 



SYMPTOMS AND COUKSE. Until the discovery is made that a tri- 

 fling wound will give rise to an irrepressible and dangerous loss of 

 blood, there is no symptom to warn the patient of his perilous con- 

 dition. Some observers, indeed, claim that this class of patients are 

 distinguishable by the remarkable delicacy of their complexion, their 

 superficially situated and conspicuous veins, blonde hair, and blue eyes, 

 and, in one family that I know of, this description is equally applicable 

 to the whole of them, including the female members, who are exempt 

 from the disorder. Other observers, again, state expressly that the 

 patient's appearance presents no perceptible characteristics. 



The accidents which give rise to these alarming haemorrhages are. 

 generally extraction of a tooth, a puncture, a small cut or laceration, 

 and it would almost seem as if such injuries were more dangerous 

 than severe wounds. The blood oozes out as if from a sponge, al- 

 though no bleeding vessel is discoverable ; all attempts to stanch the 

 bleeding are in vain, and it persists for days. The blood, which at 

 first is normal, gradually grows thin and watery, forming small, loose 

 coagula. At last the complexion of the patient acquires a waxy pallor, 

 the lips lose their color ; syncope and other signs of exsanguinity oc- 

 cur, and the patient may perish in a few days. More commonly, how- 

 ever, the bleeding ceases, and the patient, in a state of utter exhaus- 

 tion, slowly recovers from the effects of his enormous loss of blood. 



