The propensity to bleed varies greatly in its intensity in different sub jects. In the lowest degree it may show itself merely in the shape of ecchymoses in the skin. In a higher grade the patient may complain of spontaneous haemorrhage from the mucous membranes. In its most pro nounced form a tendency to every kind of bleeding is observed. The mucous membranes may pour out blood without obvious cause ; slight in juries may give rise to copious extravasation into the tissues ; petechim may appear in the skin ; and obstinate and painful swellings may attack the joints.
The haemorrhage usually occurs at a time when the patient appears to be in unusually good health, for it is at these times that there is a plethora of the smaller vessels. The bleeding may be preceded by signs of excite ment or irritability of temper, and it is said that there is sharpening of the senses of hearing and of sight. Epileptiform convulsions have been noticed in one case by Boier.
If the bleeding be spontaneous, it occurs in the child usually from the nose ; but may be also noticed from the inside of the cheeks and lips, and from the gums, especially during dentition. In less common cases blood is also poured out from the mucous membrane of the stomach and bowels, and may be vomited up or discharged by stool. As a rule, the younger the child the more likely is the haemorrhage to come from the nose or mouth. It is only towards puberty that hmmatemesis or raelna becomes common. Renal haemorrhage is rare. Once started, the loss of blood may be continuous and copious, so as to be arrested with the greatest difficulty ; or may cease for a time and then return. Sometimes from one source is quickly followed by a similar effusion from another, until the pa tient dies worn out by the constant discharge. When bleeding from one source alone ends in death, the hemorrhage occurs usually from the nose.
In addition to the spontaneous haemorrhages, slight wounds or blows may produce a copious effusion. Little cuts or scratches bleed obstinately ; slight blows upon the body may be a cause of serious extravasation ; and in certain subjects even the rising of a blister may fill the bleb with blood_ instead of serum. In such patients the extraction of a tooth, the applica tion of a leech, or the prick of a pin may induce bleeding which for a long time resists the most powerful styptics, and may even destroy the life of the patient in spite of the most energetic measures for its suppression.
The tendency to bleed, even in the case of the same child, is subject to curious variation. A slight injury which at one time gives rise to exces sive hemorrhage, at another is followed by no ill consequences ; and a child in whom repeated hemorrhages from the nose or mouth are a source of anxiety may bear the removal of a tooth without unusual bleeding fol lowing the operation. Thus Dr. Wickham Legg has reported the case of a boy, aged eight years, who was subject to frequent from the nose and gums. This child could bear the extraction of a tooth or a
cut on the finger without much loss of blood.
In all cases the source of the bleeding is capillary. The occurs as a constant oozing, which may last for hours, days, or weeks ; and it is astonishing to note the enormous quantity of blood which may be thus poured out by the most trifling wound. In the case of traumatic bleeding the haemorrhage usually begins some hours after the infliction the injury. It often does not cease until the patient becomes faint, and even then is liable to renewal when consciousness returns. By this means the child may be reduced to a state of profound anemia, and only slowly regains his colour and strength.
The petechile and subcutaneous haemorrhages which occur in hamio philia are very similar to those noticed in cases of purpura. They are com mon on the buttocks and limbs of infant bleeders, but the face usually es capes. Trifling blows may produce copious effusions. In some cases the blood infiltrates extensively through the areolar tissue of a limb, and death may even ensue from this inward bleeding. In other cases circumscribed collections of blood may be noticed, forming tumours of various sizes.
One of the most curious features of the disease in its higher grade is the joint affection to which these patients are so subject. The articula tions attacked are usually the larger ones, and in the majority of cases it is the knee which suffers ; but the ankles and hips, the shoulders and el bows are liable to be affected. The joint becomes swollen and tender, and the swelling usually increases until the ends of the bones can no longer be felt. It is accompanied by pain which is increased by movement, and there is a rise of temperature. Sometimes fluctuation may be detected. The swelling is said to be due, in some cases, to a simple effusion into the joint ; but it is more commonly the consequence of articular haemorrhage. It may occur either spontaneously or as the result of a trifling The symptom persists for a variable time, and it may be months before the joint returns to its ordinary dimensions. Several joints may be attacked in succession, or the joint affection may alternate with some form of visible hniorrhage. Blood tumours sometimes rise on the sides of a diseased joint. Thus M. Poncet has recorded the case of a boy, aged sixteen, whose right knee had been painful, stiff, and swollen for two years. Some time previously a small swelling had formed on the inner side of the Lame. This had turned black, and then had burst, giving rise to obstinate rhage. The boy was very subject to profuse bleedings from the nose, and eventually died in consequence of repeated from wounds made by the application of the actual cautery to the diseased joint.