Hæmorrhagic Diseases

It is claimed that blood transfusion provides an efficient means of treatment in most conditions distinguished by symptoms of spontaneous hæmorrhage or by traumatic hæmorrhage which cannot be controlled. All such diseases have the common features that the coagulation time of the blood is abnormally prolonged, and it may be supposed that the transfused blood supplies some missing constituent, so that for the time the blood is enabled to coagulate more normally. Most of the evidence available shows that the claims made for transfusion are not exaggerated.

Jaundice.—It is well known how exceedingly dangerous an operation upon a jaundiced patient may be owing to the difficulty of obtaining hæmostasis. The coagulation time of the patient’s blood is not affected in a transient catarrhal jaundice, but in the chronic condition it has been shown to be three or four times the normal (223). In these circumstances it is found that a transfusion is of some use in shortening the coagulation time of the patient’s blood so that bleeding ceases, although sometimes, especially in cases of jaundice due to malignant disease in which the biliary obstruction has not been relieved by the operation, the effect is very transitory, and after two or three days the patient may again begin to bleed (215). No other method of overcoming this has yet been found to be more effective than transfusion, though the intravenous administration of calcium compounds is sometimes of value.

Hæmophilia.—Blood transfusion is of still greater value when the coagulation time of the blood is prolonged owing to a congenital deficiency, as in hæmophilia. It is unnecessary to discuss here in detail the precise nature of the deficiency. No definite conclusion has yet been reached, though it seems to be clear that the abnormality resides in the organic clotting complex, and not in the calcium content of the blood. Treatment, therefore, will aim at supplying the deficient substance, so that the coagulation time may be reduced to normal, whereupon the bleeding will cease. Various methods of bringing this about have been used. Horse serum or whole blood injected subcutaneously has often been found effective and sometimes even when used merely as a local application. Not infrequently, however, horse serum fails of its effect, so that no reliance can be placed upon it. Even when effective, the alteration in coagulation time is transitory, a fact which introduces an obvious objection to its use, for if the occasion should arise, as it easily may, for a repetition of the treatment, the patient may be exposed to the risk of severe anaphylactic shock.

Another form of treatment has been introduced by H. W. C. Vines, in which a slight anaphylactic shock is deliberately induced, the result of this being a fall in the coagulation time of the blood to normal. The mechanism of this change is at present unexplained. Again, the effect is transitory, but for a certain period afterwards a surgical operation may be safely performed upon a hæmophilic patient treated in this way. This method has not yet been extensively tested, and in any case it cannot be used in an emergency, for the patient must be sensitized by a preliminary injection and an interval of several days allowed to elapse before the anaphylaxis can be produced.

The efficiency of blood transfusion in the treatment of hæmophilia has been very often demonstrated, and seems at present to afford the most certain means that we possess of arresting the symptoms. Presumably the transfused blood supplies directly the deficient factor in the coagulation complex, and it has been shown by Bernheim (1917) that the transfusion even of quite a small amount of blood will almost immediately stop the bleeding. In addition to comparative certainty and rapidity in action, transfusion has the advantage that it will replace the blood which has been lost, for often the patient has reached a stage at which he is in danger of his life from actual anæmia. This treatment, therefore, will always be useful in an emergency, whether the patient be bleeding to death from a slight wound, or whether he be suffering from acute appendicitis and so is in need of an immediate operation. If transfusion does not at once stop the bleeding, the treatment can be repeated, so that the patient should not be allowed to die from loss of blood. In most cases the bleeding will eventually stop if the patient’s life can be prolonged. Even if the treatment be immediately successful, the transfused blood necessarily contains only a limited quantity of the substance necessary for the coagulation complex, and this gradually disappears. Again, therefore, the effect is transitory, so that transfusion is in no sense curative. It has been noticed that the tendency of a hæmophilic to bleed decreases as age advances, and it has been suggested by Ottenberg and Libmann that small quantities of blood should be injected into his veins at regular intervals of one to three months. It is possible that in this way he might be brought safely through the more perilous years of his life.

The proof of the effect of transfusion upon the coagulation time of the blood rests upon the evidence of a number of independent observers. Pemberton has recorded a case of a hæmophilic whose coagulation time before transfusion was estimated to be 23 minutes. Blood was given to the amount of 500 cc., and 5 minutes later the coagulation time was 3 minutes. Twelve hours later it was 8 minutes, and on the fourth day after transfusion it had risen again to 20 minutes.

Other observations have been made as follows:

Coagulation time.Minutes.
BulgerBefore transfusion82
1 day after transfusion10
8 days ” ”8
25 ” ” ”40
Minot & LeeBefore transfusion150
After ”normal
3 days after transfusion60
5 ” ” ”100
AddisBefore transfusion245
After ”24
25 days after transfusion200
After 8 cc. serum injected38

In treating jaundice or hæmophilia the transfusion may be performed by the method of choice described in Chapter VII of the present work. The addition of an anticoagulant to the blood does not render it any less efficient as a hæmostatic agent. In all cases the coagulation time of the patient’s blood is found to be reduced after transfusion, whether sodium citrate be used as an anticoagulant or not. The explanation of this may be found in the fact referred to on p. 120, that the citrate is very rapidly destroyed in the circulation, and so cannot for long influence adversely the hæmostatic properties of normal blood.

The seeming paradox of using an anticoagulant in an endeavour to promote the coagulation of the blood is heightened by the work of Ottenberg, who has shown that the coagulation time may be reduced by the intravenous injection of sodium citrate alone. In this experiment 20 cc. of a 3 per cent. solution of sodium citrate were injected into a hæmophilic, whose coagulation time had been found to be 85 minutes. Ten minutes after the injection it was found to be 25 minutes. Two days later it had risen again to 85 minutes. This observation has not been confirmed but, if it be true, citrated blood is likely to be actually more efficient in the treatment of hæmophilia than untreated blood.

The amount of blood to be transfused in hæmophilia will vary with the age of the patient and according to whether he is suffering from acute anæmia or not. If hæmostatic effects only are wanted, 100 cc. of blood will be enough. If anæmia is also present, the dosage will be governed by the same considerations as have already been discussed in the section on the treatment of hæmorrhage.

Melæna Neonatorum.—Another hæmorrhagic condition in which blood transfusion is of the very greatest value is that known as melæna neonatorum. Severe hæmorrhage takes place from the bowel of an infant, sometimes only a few hours after birth. The cause is quite unknown, but it is found that absolute hæmostasis is usually brought about by blood transfusion. Horse serum has often been successfully used as in treating hæmophilia, but blood transfusion again has the additional merit that the blood which has been lost is thereby replaced. A single transfusion is usually enough, as the hæmorrhage does not tend to recur when once it has been stopped. For a newly born infant, even if in extremis, only a small quantity of blood is needed, so that a transfusion of 50 to 100 cc. is usually found to be enough. Bruce Robertson suggests that, as a good working rule, the amount should not exceed 15 ccm. per pound of body weight. The superficial veins of an infant are exceedingly small, so that the introduction even of a fine needle into the median basilic may be matter of the greatest difficulty. The best method of transfusing an infant, therefore, demands special consideration. A description of this will be found on p. 134 of the present work.

The value of transfusion for melæna neonatorum has not been very generally recognized, but a number of striking cases have been reported. Defibrinated blood had been used in 1873 by Sir Thomas Smith as described in Chapter I, but the first case in which whole blood was used was published by Lambert in 1908. Later, in 1910, Welch, and then Schloss, recommended the subcutaneous injection of serum or of blood, but these measures were clearly not so effective as the intravenous transfusion of blood, as has been testified by numerous observers (Lespinasse, Unger, Vincent, Graham, Bruce Robertson, Lapage, Hutchinson, etc.). The patients may be actually moribund, for a new-born infant can only afford to lose a relatively small amount of blood, but even then transfusion is often successful. Bruce Robertson reports that of a series of forty cases of hæmorrhagic disease of the new-born which were treated by transfusion, all recovered except four; of these two died from associated umbilical sepsis, one from intracranial hæmorrhage, and the fourth had already ceased breathing when the treatment was begun.

It has sometimes been stated that for transfusing an infant either parent can be safely used as blood donor, on the assumption that the serum reactions are not yet developed. This may sometimes be true, but the fallacies and possible dangers of this are explained in a later chapter.

A case was recently reported by R. D. Laurie, who, knowing that he himself belonged to Group IV, drew 20 ccm. of his own blood into a syringe containing five grains of sodium citrate in solution. This he injected into a vein in the infant’s arm; the small size of the vein he had chosen made this difficult, but the treatment resulted in the rapid recovery of the patient.

Purpura.—Of all the forms of hæmorrhagic diseases, the two already described, hæmophilia and melæna neonatorum, are the only ones for which blood transfusion is a really effective remedy. It is probable that under the somewhat general term “purpura hæmorrhagica” are grouped several conditions, all of very obscure origin, none of which are conspicuously benefited by transfusion. Many transfusions have been given for purpuric symptoms, chiefly in America. Several cases are reported by Bernheim, and twelve transfusions were given to seven patients by Peterson. In some of these the treatment produced a temporary improvement, but usually they relapsed after an interval of a few months. One of Bernheim’s patients appears to have owed his life for the time being to a transfusion, but he died subsequently during a recurrence. Two cases are reported by Graham. One was not benefited at all; the other improved for a time, but afterwards relapsed. In a serious case, therefore, transfusion may be worth trying; it has indeed been stated by Ottenberg and Libmann, observers with a wide experience of transfusion, that this treatment is “definitely curative” in severe cases of purpura. At the present time there is little to add on the subject, but it is possible that further advances will be made by proceeding on these lines.