HEMOPHILIA 433 



portion of the femur is serrated and irregular, the cavity normally existing 

 between the two condyles has been obliterated; the same is also true of the 

 intercondylar eminences of the tibia which are irregular and broadened. Still 

 more advanced lesions are seen in older patients; the space in the joint car- 

 tilage disappears entirely, the tibia seems dislocated toward the femur out- 

 wardly. Occasionally adhesions may be noted between the femur and the 

 tibia. The condition of the capsule cannot be accurately determined on 

 account of the effused blood. 



The microscopic investigations of the vascular changes in hemophilia, made 

 by Buhl and Birch-Hirschfeld, deserve attention. 



The former found in a preacher, aged seventy-four, suffering from chronic 

 dermatoses and presenting hemophilic symptoms, an immoderate growth of 

 the capillary vessel loops with a great increase and subsequent transformation 

 of their nuclei. Buhl himself does not believe this anomaly to be peculiar to 

 hemophilia. Birch-Hirschfeld examined the heart and the large vessels, a 

 portion of the spleen, the kidney and the skin of a child, aged one year, who 

 had succumbed to congenital hemophilia, and remarks concerning these organs : 

 " In the heart and in the vessels connected with it I discovered no changes. 

 It may be stated that the vessels of the heart, considering the age of the child, 

 were quite of normal size. In the heart muscle there were but slight indica- 

 tions of fatty degeneration. The valves, as well as the intima of the larger 

 vessels, were delicate and normal in structure; the same was also true of the 

 muscularis and media of the arteries. In the kidneys the cortical canals were 

 swollen and there was slight granular cloudiness of the epithelium. There 

 was nothing noteworthy in the spleen except moderate hyperplasia of the 

 stroma of the pulp. But I must mention — with reserve — a finding which I 

 noted in the capillaries and the transitional vessels of other organs, particu- 

 larly of the liver and kidneys. In some areas the endothelia were obviously 

 enlarged, their nuclei swollen; here and there were granular deposits in the 

 protoplasm. In some rather poor preparations which were stained with silver, 

 the epithelial arrangement appeared to me irregular, disarranged, with occa- 

 sional wide spaces between the endothelia. I do not lay special stress upon 

 this condition. In delicate changes of this kind it is not always possible to 

 determine how much is artifact, and it must not be forgotten that such changes 

 are, perhaps, not rare in the last stages of other chronic cases." Kidd de- 

 scribed in the finer vessels of the subcutaneous connective tissue and of the 

 muscles an increase of the endothelia with dropsical swelling of the muscularis 

 and proliferation of their nuclei — changes which Legg in another case was 

 unable to recognize. I should like to mention the simultaneous occurrence 

 of hemophilia with multiple sarcomata. 



Obviously pathological anatomy affords no clue for the comprehension of 

 the clinical picture. The belief that blood formation is periodically increased, 

 which is mentioned by some authors, has but little to support it, and the same 

 is true of the hypothesis that the overfilling of the narrow, perhaps friable, 

 vascular system of hemophiliacs by a hypertrophied heart through which newly 

 formed blood is occasionally forced in large amounts leads to a rupture of the 

 markedly distended capillaries. 

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