Haemophilia: a sketch of our present knowledge of the disease, with results in eight cases of the effect of normal serum on the blood coagulation time

Abstract

1. The transmission of the disease in all the cases is according to the usual rule - through the female line.2. The Prolific tendency of the disease is well marked in all the family pedigree charts.3. There is an absence of symptoms in early infancy.4. No prodromel symptoms were ever complained of.5. The frequent nocturnal onset of symptoms is well seen in these cases.6. The joint symptoms are shown to occur at an earlier age than is generally stated - five years instead of twelve to fourteen years - they are very rapidly recovered from. In the foregoing cases the knee joints were most frequently ffected, and next the elbow joints. X ray photographs may be useful in diagnosis.Facts noted about the Blood:7. Blood Counts were done on fully 40 occasions. The Red. Counts and Haemoglobin showed nothing special to note. The White Counts, however, in no case shoved a leucopaenìa, es stated by Wright , but were found to be all normal or slightly raised.8. The differential Counts (300 cells being counted in each film) likewise show no departure from the normal, and no evidence was seen, in either the blood of the patients themselves, or that of their. non-haemophilic relatives, of a polymorphonuclear leucopaenia, stated by Wright to be a constant feature of the disease.9. The Blood Pressure was normal.10. The Viscosity of the Blood in the majority of the cases was below normal, and in only one case was it above normal; as held to be the usual rule by Neil.11. The local applications to bleeding surfaces are shown to be of little use. Local applitions of normal horse serum are equally disappointing12. The Coagulation of haemophilic blood shows no material difference from that of normal blood, except that it takes place in a relatively much longer time. Experiments shored that each successive specimen of blood taken from the same puncture wound increased in rate of coagulation, till it became instantaneous, and this delay is supposed to be due more to a qualitative than to a quantitative change in the blood.13. The series of blood coagulation estimations, (of which about 500 were done) taken at each sitting, were found to vary considerably in time, and to a greater extent than could be accounted for by experimental error. Those taken from the control and from the "non-bleeder" relations of the patients were not found to vary to an appreciable extent.14. In so- called "Spontaneous Haemophilia", normal serum has been found to give excellent results, but in true hereditary haemophilia, it never brought about a sudden fall in coagulation time. In the severe cases, it had some slight effect, but in the milder cases appeared to be inert. an the whole, therefore, normal serum, as a therapeutic agent in haemophilia, was found to be disappointing, and its action on the blood coagulation uncertain, slight and transient.