A system of practical medicine. By American authors. Vol. 3

by Unknown

¹⁷⁷ Trans. State Med. Soc. N.Y., 1877.

The PATHOLOGY of the disease is unknown. No doubt two circumstances combine in hæmophilia—congenital fragibility of the vessels and a defect in coagulability of the blood—but whereon these depend we are as yet entirely ignorant. There is no evidence of the nature of the anatomical changes in the vessels which permits of their ready laceration, and none on the nature of the alteration of the blood which prevents the normal thrombus formation in a wound; and in the absence of information on these points theories must necessarily be unsatisfactory, and their discussion, in a work of this practical nature, profitless.

The DIAGNOSIS presents no difficulty in members of a bleeder family, in whom slight joint trouble and petechiæ are as much manifestations of the disease as the more severe hemorrhages. In a large majority of cases the tendency becomes manifest at an early date. The spontaneous umbilical hemorrhages of infants are, as a rule, to be excluded, being dependent upon, or associated with, jaundice or syphilis or a mycosis (Weigert¹⁷⁸). The hemorrhagic diathesis may develop in children or members of a healthy family and prove fatal, and the question in such cases always comes up, Are they instances of hæmophilia? There seems to be a desire to limit this term to cases of an hereditary nature only; but when a child shows a marked tendency to multiple hemorrhages, spontaneous or traumatic, which tendency persists and is not merely transitory, and particularly if there are joint troubles, I think that under these circumstances we have a genuine case of hæmophilia; and such a child, if he—it is more likely to be a male—survives and marries, may be the founder of a bleeder family. These are the congenital in contradistinction to the hereditary cases. In the histories of the bleeder families we frequently come back to the origin in a person born of a healthy stock in which there have been no hemorrhagic tendencies. On the other hand, single severe uncontrollable hemorrhages in children or adults are not to be ranked as hæmophilia unless there have been other features pointing to the existence of the diathesis. The literature abounds in cases of this kind, many of which are described as hæmophilia. In doubtful cases it is very difficult to decide, as in a case of Forscheimer brought before the Academy of Medicine of Cincinnati.¹⁷⁹ In the review of American literature we have excluded all cases in which the hereditary or congenital characters were not well marked.

¹⁷⁸ Cohnheim's Pathologie, i. 382.

¹⁷⁹ Cincinnati Lancet and Clinic, 1884.

It may be useful to put down here for the guidance of the practitioner the varieties of bleeding commonly met with, and which must not be confounded with hæmophilia:

(1) The umbilical hemorrhages of infants, due to jaundice or to syphilis hæmorrhagica neonatorum, etc.

(2) Purpura simplex, seen often in debilitated, rarely in healthy, children, usually confined to the legs, and in some cases I have seen it associated with rheumatic pains or swellings in the knees and ankles.

(3) Peliosis rheumatica, an affection which in the large interstitial hemorrhages and the joint swellings touches hæmophilia in a curious way. It too may show itself in several members of the same family.

(4) Purpura hæmorrhagica, Morbus maculosus Werlhöfii, a grave disease, characterized by extensive cutaneous ecchymoses, mucous hemorrhages, but not dependent on any local disease, or, so far as is known, on any specific poison. The bleedings in scurvy may be mentioned here, but there could be little difficulty in determining their nature.