HEMOPHILIA 299 



although the left heart is frequently enhirf^ed, there is usually no in- 

 creased blood pressure demonstrable; furthermore, conditions of 

 high blood pressure, such as nephritis, do not cause hemophilia. The 

 theory of ''hydremic plethora" is also without good foundation. 



The most natural place to look for the fundamental fault is in the 

 blood, but speaking strongly against this is the occasional occurrence 

 of "local" hemophilia; e. g., in this type of hemophilia wounds of the 

 skin may behave as in normal individuals, whereas any injury of the 

 mucous surfaces is followed by pronounced hemophilic bleeding ;^^ 

 in other cases the hemophilic bleeding is limited to regions above the 

 shoulders; in still another class the bleeding is always from one organ, 

 e. g., the kidneys. Nevertheless, a great deal of investigation of the 

 blood has been done, at first chiefl}' with negative results. There are no 

 characteristic changes in the cellular elements of the blood, beyond 

 the changes common to all secondary anemias, excepting possibly a 

 decrease in the number of w^hite corpuscles with a relative increase in 

 the number of lymphocytes as observed by Sahh; the platelet count 

 is normal. No constant alterations in the salts of the blood have 

 been found, calcium usually being normal ;^^ and the proportion of 

 water, fibrinogen and the several other proteins, the alkalinity, and 

 the osmotic pressure of the serum all seem to be normal. Metabo- 

 Hsm is unchanged, except possiblj^ for calcium loss in some cases.** 

 Since bleeding is normally stopped principally by coagulation, a de- 

 ficiency in fibrin or its antecedents might be expected, but most 

 studies on this point have shown a normal amount of fibrinogen in 

 the blood of hemophilics, the frequent formation of large tumors of 

 clotted blood at the bleeding points supporting the experimental 

 evidence that the blood contains an abundance of fibrinogen. The 

 "bleeding time" following punctures in the skin is not excessive. As 

 to the rate of clotting, Sahli," who avoided a number of errors made 

 in earlier investigations, found that in the intervals between the at- 

 tacks of hemorrhage the rate of the coagulation of the blood is con- 

 stantly much slower than normal. During an attack of bleecUng the 

 coagulation time approaches the normal; indeed, it may be faster 

 than normal; apparently this is due to a reaction on the part of the 

 organism to the loss of blood. If blood is collected directly from the 

 site of bleeding the coagulation time is very rapid, because of the ac- 

 cumulation of fibrin ferment from the clot over which the escaping 

 blood flow^s. Yet in spite of the normal coagulability of the blood and 

 the rapid clotting after the blood escapes from the vessel, bleeding 

 continues for long periods before it can be stopped. As he found no 

 general change in the properties of the blood to account for the bleed- 



" Abderhalden, Ziegler's Beitr., 1904 (35), 213. 

 " Ivlinger and Berg, Zeit. klin. Med., 1918 (85), 335, 406. 

 " Kahn, Amer. Jour. Dis. Children, 1916 (11), 103; Laws and Cowie, ibid. 

 1917 (13), 236; Hess, Bull. Johns Hopkins Hosp., 1916 (26), 372. 



