Progressive Pernicious Anemia.
Synonyms.—Idiopathic Anemia; Essential Anemia; Corpuscular Anemia.
Definition.—A grave blood-disease, characterized by a progressive decrease, in the number of red corpuscles and by fatty degeneration of the various viscera, and a characteristic, lemon-yellowish decoloration of the skin.
Etiology.—Pernicious anemia depends upon an insufficient and defective formation of red corpuscles, and is found more frequently in middle life than in the young, and among the poor classes rather than the well-to-do, though it may be found in children and persons of wealth.
It is not a common disease, though in Switzerland it prevails more frequently than in any other country. Addison was the first to clearly describe pernicious anemia as an idiopathic disease, and though there have been many who have doubted that it could exist as a distinct lesion, we will have to admit that there are cases of anemia, when there is no appreciable cause,—cases that have not been preceded by tuberculosis, Bright's disease, malignant growths, renal, hepatic, or splenic affections, wasting diseases, hemorrhages, or chronic diarrheas.
Pregnancy and parturition may be associated with anemia. The course of pregnancy may be attended with so much nausea rind vomiting that the function of blood-making is seriously impaired, and anemia of a permanent character develops. Generally, however, the anemia develops post partum. Atrophy of the stomach has been regarded as a cause of anemia, the two being often associated, though Grawitz regards the atrophy the result, rather than the cause.
In rare cases, parasites may be the producing cause, by impairing nutrition and establishing toxins that result in cell destruction; the anchylostoma duodenalis and the bothriocephalus being the ones most frequently responsible for the disease.
Exhausting diseases and profuse hemorrhages may also figure as contributing causes, but after we exclude these we still find cases of pernicious anemia that can not as yet be accounted for. Quincke and Peters think that the increased hemolysis is due to the large amount of iron found in the liver at this time. Hunter called attention to the urine of anemic patients. He found it darker in color and containing pathological urobilin. His findings naturally strengthen the views of the last-named writers.
Pathology.—The skin presents a characteristic lemon-tint. There is but little emaciation. The fats are well preserved and of a yellow tinge, while the muscles may be pale or of a reddish color, resembling horse-flesh. The heart is flabby, and contains but little blood. There is fatty degeneration of the organ, the muscular fibril being replaced by fat. The spleen is but slightly enlarged, and shows a cloudy swelling or fatty degeneration. The liver and kidneys present the same characteristic changes, and in addition, there is an excess of iron. In the liver it is deposited in the outer and middle zones of the lobules while in the kidneys it is found in the convoluted tubules. Hemorrhages occur in the retina, skin, and other portions of the body. There is nearly always a change in the bone-marrow, it. becoming reddish, and soft in character. The stomach is usually diminished in size, with atrophy of the gastric tubules. The intestinal glandula share in the atrophy. Punctate hemorrhages occur in the brain, and the spinal cord, and posterior sclerosis of the cord is not infrequent.
Fatty degeneration of the various viscera, and even the intima of the smaller vessels, is perhaps the most constant lesion, if we except the blood changes.
The blood shows a marked diminution of the red corpuscles as well as other changes in these cells. It is pale, thinner, and does not coagulate readily. The red blood-corpuscles are often reduced to 1,000,000 per cubic millimeter, and in extreme cases to 500,000, while 143,000 has been recorded. The blood-disks are found widely separated, and not forming rouleaux.
These blood-cells are of various sizes and shapes, the giant cells predominating, though there is a wide range between the megalocytes and the microcytes. In form they may be spherical, oblong, dumb-bell shaped, or of other irregular shapes. There is also a reduction of hemoglobin. The leukocytes are diminished in number, but do not assume the peculiar shapes noticed in the red blood-corpuscles.
Symptoms.—The disease comes on so insidiously that the patient is unable to refer to the day that the disease arrested his attention. He, perhaps, has noticed for some time that his strength was failing, and that he was getting pale; that his appetite was poor, and that he tired readily on exertion. Among the earlier symptoms are shortness of breath, slight palpitation of the heart, dizziness, ringing in the ears, and headache. Dyspeptic symptoms are common, and nausea and vomiting, with diarrhea, are often present. The patient takes on a lemon-color tint, and the skin is waxy in appearance, and, though emaciation does not take place, the tissues lose their tonicity and become flabby.
Respiration is short and hurried on the slightest exertion, and there is sometimes pain, more often a sense of constriction of the chest. The pulse may be full, but is soft and easily compressed; generally, however, it is small and feeble. Hemorrhage may occur in the retina, giving rise to disturbed vision. There also may be bleeding from the nose, lungs, urethra, and uterus. As the disease progresses, the ankles swell, debility increases, and the patient takes to his bed.
In advanced cases there may be an irregular fever, anemic fever, though the temperature range is usually low. The lips and gums are pale and bloodless. The mind wanders, and he sinks into a half-torpid state. If we examine the heart during this stage we find the cardiac sounds feeble and hemic murmurs common, especially over the base of the heart.
Treatment may give rise to some encouragement, but a relapse occurs sooner or later, and the patient dies from exhaustion.
Diagnosis.—This is usually not very difficult, though it may be mistaken for malignant growths, kidney lesions, and various grave diseases. The age of the patient and the slow, insidious manner in which the disease begins, should arouse our suspicions. The increasing pallor changing to a lemon-tint, with waxy skin, the inelastic, doughy tissues, the absence of emaciation, the tired, wearied condition of the patient on the slightest exertion, the quick and hurried breathing, would suggest anemia. If retinal hemorrhages are found, they confirm the diagnosis. Micro-scopial examination of the blood not only reveals a marked decrease in the red blood-disks, 1,000,000 or less per millimetre, but also reveals large nucleated led blood-corpuscles, megalocytes and various sized and shaped corpuscles.
Cancer is apt to occur late in life; there is greater emaciation, more pain, and a local tumor can usually be outlined.
Prognosis.—All writers agree that pernicious anemia is a very grave disease, and that very few cases recover. Under the administration of arsenic, some cures are recorded, though a patient, seemingly cured, is apt to have a relapse within five years and die. A few permanent recoveries are recorded.
Treatment.—All schools of medicine agree that arsenic is the one remedy that promises greater relief than any yet tried. While administering this agent, we are stimulating the blood making organs, with the hope of getting a better elaboration of blood. Pure air, outdoor exercise, and an easily digested diet will assist materially in bringing about the desired result. If the patient complains of great weariness, rest in bed should be enjoined. Daily or triweekly injections of normal saline solution may prove of some value. Should recovery take place, the patient should, at the first indication of its return, resort to arsenic.