Progressive Pernicious Anemia.
Synonyms:—Essential anemia; idiopathic anemia; corpuscular anemia; Biermer's anemia.
Definition:—A serious disease of the blood in which there is rapid progressive disintegration of the red blood corpuscles, accompanied with a tendency towards fatty degeneration. There is not only a decrease in the number of red cells, but there are alterations in the character of the cells and serious changes in the marrow of the bones. There is a slight pathological distinction between idiopathic anemia and Biermer's anemia.
Etiology:—There is a direct destruction of the red blood corpuscles or an insufficient supply of these corpuscles or defects in the morphological processes by which the corpuscles are formed. The condition may occur at any time of life among both males and females. It is more common between the ages of twenty and forty-five years, occurs rarely in young children, is not uncommon among those who are fortunately situated and who have the best of surroundings, although the conditions named under chlorosis plainly exercise a causative influence. Idiopathic anemia is described by Addison as a form of this disorder which occurs with no discoverable cause, either ante-mortem or post-mortem.
At other times the disease follows certain infectious disorders, either immediately or after a short interval, or it occurs in conjunction with tuberculosis, or hepatic or splenic disorder, or Bright's disease or other chronic disease of the kidneys; or it may be the result of disease which exercises a serious depleting influence upon the system, as chronic diarrheas, hemorrhages, or malignant disease. It also occurs after confinement, or during the course of pregnancy, when there has been serious disarrangement of the function of the blood making organs, which on their part may result from chronic disease of the stomach or of the gastrointestinal tract. Occasionally it is found after death that the disease was caused by parasites of various characters. The oxyuris vermicularis, the ankylostomum duodenale, the bothriocephalus latus are those most frequently found. It is thought to occur also from toxemias of various kinds.
Symptomatology:—It is often impossible to determine the time when this disorder could be said to have started. It is insidious in character and usually of slow development. The patient is feeble for a long time and indisposed to any physical exertion; has weakness, faintness, vertigo, and finally increasingly severe headaches. There seems to be an increase of weight, but the patient will state that it is not "healthy" fat. With this increase there is a peculiar paleness which is slowly on the increase. Finally there is shortness of breath, irregular action of the heart or palpitation on little exertion, with ringing in the ears, or a roaring in the head, and perhaps temporarily disordered vision. Disorder of the stomach is progressive also, beginning with slight nausea, loss of appetite, imperfect digestion and diarrhea. The tongue is pale and bloodless, as well also as the gums, lips and mucous membranes of the mouth. It is thick and flabby and often heavily coated. There is a characteristic pearly appearance to the sclerotic coat of the eye, which is diagnostic. With the increase in the weight of the patient there is puffiness of the face and a swollen condition of the ankles, and the tissues, while increased in size, lack tone, and are deficient in the power of resistance or of muscular contractility. They are flabby and loose and the pallor of the skin increases to a waxy or lemon colored paleness.
The weakness is rapidly progressive and prostration soon appears. The patient is too weak to sit up and the tone of voice and manner of speaking indicate extreme feebleness. The mental processes are slow, the mind becomes feeble, and there is a slight tendency to delirium or wandering of the mind. Every muscular exertion causes rapid and feeble respiration and increases the action of the pulse. There may be hemorrhages as the disorder progresses, either from the lungs, stomach or from the nose, occasionally from the womb or from the kidneys. The temperature is variable. Occasionally it is elevated and there is a mild fever, but usually it is normal or subnormal and the extremities are cold. The heart sounds are feeble and hemic murmurs are discoverable over the base of the heart. The pulsation of the carotids is very distinct and of a peculiar jerky and expansile character.
Diagnosis:—The fact that anemia occurs in middle life and more frequently in men than in women, the peculiar yellowish and cheesy appearance of the skin, which is very flabby, and the increase of weight, with the extreme pallor, are all suggestive phenomena. An examination of the blood will show the red cells to be distinctly diminished in number, a condition which is not true in chlorosis. The red corpuscles are nucleated and often increased in size and there are present a large number of megaloblasts. The progressive character of the disease, the increase in all the serious symptoms, the tendency to prostration and to hemorrhage, will all point to this disorder.
Prognosis:—The prognosis is always grave. Cases that improve for a while under treatment, or that are apparently restored, are liable to relapses, with a fatal result. The disease runs its course to a fatal termination in from three or four months to a year, or at the farthest to a year and a half.
Treatment:—While we can give these patients but little encouragement and the results of the treatment of the disease, are usually unsatisfactory, it is obligatory upon us that every case is treated with the utmost care. All measures which are calculated to restore tone and vitality to the patient, to improve the functional operations of the vital organs, must be adopted and a course of life pursued which provides the very best of hygienic surroundings for the patient. Out of door air and sunshine and a good digestion are the most important. The food should be selected with reference to its nutritional qualities and ease of digestion. I am in favor of continuing the use of artificial digestives in these cases during the entire progress of the treatment. Symptomatic indications should be met according to the knowledge and judgment of the physician, with specific remedies.
The beneficial influence of arsenic upon this disease is exceedingly difficult to explain, but it is universally recognized as an efficient remedy. It seems to retard the disintegrating processes and stimulate the functional activity of the organs which are engaged in the blood making processes. It is given in small doses at first, not to exceed four or five drops of Fowler's solution, three or four times daily. This is increased one drop each day, up to the point of toleration. If the condition of the patient is such that he can take a large quantity of this remedy without injurious influences, his chances of recovery seem to be correspondingly increased. Patients have been known to take from eighty to one hundred minims of the solution in the course of twenty-four hours without markedly bad effect. A few years ago bone-marrow was used as a restorative and it was thought for a while that it would prove a dependable remedy, but its popularity has not greatly increased.
It is important in most cases that the patient should have some preparation of iron, and occasionally there will be considerable improvement from the use of this remedy. It is a good plan to push the arsenic to its full effect, then to give the patient a course of iron, omitting the arsenic, and ultimately to resort again to the arsenic treatment. If the patient should seem to be restored and a fair condition of health secured, it is wise to resort to the arsenic treatment occasionally, for a short period, in anticipation of a possible relapse The use of the tincture of iron in conjunction with stomach tonics, or artificial digestives, may be resorted to with good results. Diarrhea should always be controlled, as well as other depleting complications. For this purpose geranium, gallic acid, or the subsulphate of iron or aromatic sulphuric acid, are all available. The use of intravenous injections of the normal salt solution, or this substance introduced occasionally by hypodermoclysis, or the injection and retention per rectum of a full quantity of the same solution is of much importance. This must not be neglected.