Hemophilia.

Synonyms.—Bleeder's Disease; Hemorrhagic Diathesis.

Definition.—A hereditary disease characterized by frequent and sometimes uncontrollable hemorrhage, either external or interstitial, and occurring either spontaneously or from slight traumatism.

Etiology.—This constitutional defect is in some unaccountable manner transmitted from mother to son, and while the daughters of such a mother are not bleeders, they in turn transmit the same constitutional defect to their male offspring, though the children of a bleeder escape the disease. The disease is confined almost entirely to the male sex, the proportion being thirteen to one. It is found in all classes, and, strange to say, its victims are frequently large, vigorous, and well developed. It usually appears before the second or third year, though it may be delayed to the tenth year or even later.

Pathology.—The pathology of hemophilia is not well understood. In some cases the walls of the capillaries are unduly thin. Enlargement of the joints, especially of the knees, is common. Coagulation of the blood is delayed, and this peculiar condition may be due to a perversion of some of its essential constituents. Hemorrhages may occur in and about the capsules of the joints, and inflammation of the synovial surfaces may occur. In one of my cases this was a marked feature.

Symptoms.—The general health is generally remarkably good, with the exception of an occasional attack of synovitis or rheumatism. The essential symptoms, then, are frequent prolonged hemorrhages, either spontaneously or traumatic.

Spontaneous hemorrhages are more apt to occur during the early years of life, and after maturity this tendency may subside and bleedings only occur as the result of a traumatism; thus one of my patients had frequent and alarming hemorrhages, occurring from the gums and nose, up to the age of twenty-five years, since which time (he is now forty-eight) the only severe hemorrhages have been the result of injuries.

The slightest bruise will be attended by severe interstitial hemorrhage; in one instance, the result of a fall, the leg, from the knee to the ankle, became a dark-purple plum-color. In some cases the bleedings only occur as the result of an injury; thus the brother of the case just referred to has had several prolonged and dangerous hemorrhages, the result in one case from the extraction of a tooth, the patient bleeding at short intervals for nearly two weeks, and another severe bleeding from a slight injury to the lip. The two brothers, the only children of the family, have never suffered from anemia, notwithstanding the great loss of blood, owing, I think, to their splendid appetites and digestion.

The bleeding may occur from the nose, mouth, kidney, or urethra, and not infrequently into the joints. The bleeding consists of an oozing from the capillaries, and may last for days. Frequent attacks of rheumatism are the most common affection to disturb the otherwise healthy state of a bleeder.

Diagnosis.—Knowing the history of a hereditary disposition, a persistent capillary oozing would render the diagnosis very easy. Without such a history, our diagnosis would be made by a prolonged and almost uncontrollable hemorrhage, without sufficient cause to account for it.

Prognosis.—A large per cent of bleeders die young, perhaps fifty per cent before the tenth year, and every year lived after that age favors a gradual change for the better and a final disappearance of the tendency to hemorrhage.

Treatment.—Perfect quiet must be enjoined and all excitement overcome. Internally the first trituration of carbo veg. has served me well in one persistent bleeder. Oil of cinnamon and erigeron in five to ten drop doses will also be found to give good results, while gallic acid in from five to ten grain doses may be tried.

Locally, firm compression may prove successful, and tannin, perchloride of iron, chloraseptic, and adrenalin may be tried in turn, though I prefer the dry tampons of wool or asbestos, since the ferruginous applications are apt to cause such a hard clot that in its removal a fresh hemorrhage is exched. Circumcision should not be performed on a bleeder, nor any other surgical operation, unless absolutely necessary; the extraction of a tooth being attended with danger.

The Eclectic Practice of Medicine, 1907, was written by Rolla L. Thomas, M. S., M. D.