HEMOPHILIA 435 



signs of the disease in the form of severe hemorrhages from the gums. Some- 

 times in girls, although quite rarely, there may be no evidence of the disease 

 throughout infancy and childhood, the first symptoms appearing at the time 

 of puberty and recurring at each menstrual period, the menses being unusually 

 profuse and persisting for a long time. Even after an easy and uncomplicated 

 labor occasionally severe, even fatal, hemorrhages occur, so that Kehrer has 

 proposed the interruption of pregnancy in hemophilic women by artiiicial 

 labor. 



The most obvious and important sign in the clinical picture of hemophilia 

 is the appearance of well marked hemorrhages either without an assignable 

 cause or as the result of very slight external injuries. In about 50 per cent. 

 of the eases the mucous membrane of the nose is the seat of the hemorrhages ; 

 next follow bleeding from the gums and intestinal hemorrhages (each about 

 12 per cent,), then pulmonary, gastric and renal hemorrhages (each about 6 

 per cent.). Most frequent are the hemorrhages from the skin and mucous 

 membrane, next from the Joints, to which I shall later refer explicitly, and 

 finally, metrorrhagia. Apart from arthritic hemorrhages, hemorrhages from 

 the serous membranes without assignable cause are quite rare. On the other 

 hand, hemorrhage from the conjunctiva is occasionally noted, apparently 

 occurs without cause, and may sometimes be so severe as to result fatally. 

 This was seen in the case of two brothers, both bleeders, who were quite 

 young. Occasionally the hemorrhages are so profuse that death takes place 

 in a few hours. It is often astonishing to observe what enormous amounts 

 of blood hemophiliacs may lose, and how rapidly they recover from this. In 

 Cohen's case the patient lost a pound of blood (358 grams) per hour, and in 

 another case of hemophilia, within eleven days twenty-four pounds of blood 

 were lost. Occasionally syncope, the result of cerebral anemia whereby blood- 

 pressure falls to a minimum, causes a cessation of hemorrhage. With an 

 increase of the blood-pressure, hemorrhage begins anew. The disproportion- 

 ately rapid regeneration of the blood must be referred to the previously men- 

 tioned increase of the hematopoietic function of the bone-marrow in hemo- 

 philia (Fischer) which, however, is of a somewhat hypothetical nature. 



It is customary in hemophilic bleeding to differentiate between spontaneous 

 and traumatic hemorrhages. All hemorrhages are designated as spontane- 

 ous for which no plausible reason can be assigned. But it must also be 

 considered, in the study of the hemorrhagic diathesis, that there are condi- 

 tions in which the organism of the hemophiliac reacts by hemorrhages to 

 very slight influences — influences of such an insignificant character that the 

 person who suffers a lesion scarcely notes it at all or but very slightly. With- 

 out doubt some of the hemorrhages which in this diathesis have been looked 

 upon as spontaneous are in reality traumatic. We differentiate in these 

 hemorrhages (spontaneous as well as traumatic) two forms: The superficial 

 and the interstitial. 



Spontaneous hemorrhages are occasionally preceded by prodromes such as 

 cardiac palpitation, congestion of the head, vertigo, tinnitus aurium, anxiety, 

 nausea and even vomiting. Prior -to very severe hemorrhages, decided red- 

 ness of the face is often noted'; in the visible arteries rapid and strong pul- 



