430 THE HEMORRHAGIC DIATHESES 



into the joints which occur while the patients are in bed, and when we are 

 unable to detect the slightest external cause. In such instances we are almost 

 always dealing with joints which have repeatedly been the seat of effusions 

 of blood. 



Most bleeders perish in infancy, a large proportion before the eleventh 

 year. Barely do they reach old age, seventy and above. The hemophilic 

 predisposition diminishes with advancing years. 



If we study the reports up to the present time, we are convinced that the 

 true causes of hemophilia are absolutely unknown. Even of the two etiologic 

 factors most frequently and invariably mentioned — an abnormal composition 

 of the vascular walls (which is said to consist in great fragility) and the 

 insufficient power of coagulability of the blood, to which I shall again refer — 

 we do not possess a single positive proof which will bear criticism. 



Heredity assumes such importance in the etiology of this disease that it 

 is well to point out that a highly nervous temperament on the part of the 

 ancestors has often been noted. Of course, the reports of the patients and 

 their relatives must be accepted and utilized with extreme caution ; yet a num- 

 ber of well-authenticated cases are on record which certainly prove the neuro- 

 pathic predisposition of the ancestors of bleeders. 



PATHOLOGICAL ANATOMY 



The anatomical examinations in fatal cases of hemophilia have, up to the 

 present time, shown nothing that is characteristic or invariable ; in particular, 

 very little has been revealed to explain the tendency to spontaneous hemor- 

 rhages, except in the case of the joints. Here, by the tireless researches of 

 surgeons, we are permitted a deeper insight into the mechanism of arthritic 

 hemorrhages. 



The number of autopsies by experts on the cadavers of bleeders is very 

 small ; consequently the number of anatomico-pathological findings is also very 

 scant. In general the following is reported: The cadaver is conspicuously 

 anemic; the skin is waxy pale. In cases in which death has occurred from 

 hemorrhage after trauma, petechise, ecchymoses, suffusions of blood and signs 

 of injury are found upon the skin itself with comparative frequency. The in- 

 ternal organs throughout are extremely anemic. The small amount of blood 

 flowing from severed vessels is decidedly watery. Of the abdominal organs, 

 in which there are often signs of preceding hemorrhages, the spleen is par- 

 ticularly interesting; W. Koch maintained — as we have seen — that it is in- 

 variably enlarged; it has occasionally been found enlarged, but is usually 

 normal. This enlargement of the spleen in hemophilia has sometimes been 

 deemed especially important, particularly as proving the infectious nature 

 of the disease; but the knowledge that this finding is inconstant has led to 

 the rejection of the theory. 



The blood-vessels of the skin which are superficially situated have been 

 often described, the arteries as well as the veins. The muscle of the heart 

 frequently, but by no means invariably, shows fatty degeneration. Its size 

 has in some cases been found normal ; in other cases it is hypoplastic, and in 

 still others hypertrophic, especially as regards the left ventricle. In a number 



