Haemorrhagic Diseases

Purpura

Purpura is a symptom rather than a separate or distinct disease; it is shown as a leakage of blood into the skin and mucous membranes and elsewhere, with the formation of small purpuric patches of various sizes in the skin, which are of a bluish-red colour; the larger areas resemble bruises.

There are two main types of purpura: the primary form, and the secondary.

Primary Porpura

The primary form develops without any apparent underlying cause, and the symptoms are very variable. There is apparently some alteration, in the walls of the small capillary blood vessels, which allows the blood plasma and the blood cells to escape through into the tissues. The blood platelets are generally fewer in number than normal, and this also increases the tendency to bleeding, since the blood does not clot so easily.

The condition may be hereditary, or may develop later in life; women are rather more often affected than men. There is considerable bruising of the skin after very slight injuries, and severe loss of blood follows slight abrasions or the extraction of teeth. Anaemia is common when the haemorrhages are frequent or severe, but is also often present in minor degrees as a chronic symptom.

The loss of the blood plasma from the blood vessels is likely to give rise to surface weals and swelling similar to those seen in nettlerash; joint swellings with pain are also common, and there may be attacks of abdominal pain.

Since the condition is constitutional the disease is apt to be chronic; it may tend to become worse or there may be periods of improvement. When once the primary form of purpura has shown itself, attacks are liable to occur at intervals of any length of time. These attacks may always be of the same type in any individual patient, that is there may be a purpuric rash with nose-bleeds, or they may vary from time to time. A single attack may prove rapidly fatal, or there may be a succession of slight purpuric symptoms throughout life. The commonest age for the condition to show itself is from five to fifteen years.

Purpura Simplex is the mildest form, and is usually seen in children. Purpuric spots develop, generally on the legs, and may appear in crops. Diarrhoea is often present, with slight fever, and sometimes there are joint pains. The attack lasts for several weeks.

Purpura Rheumatica generally affects young men. At the onset the throat is sore, with a moderate rise of temperature. A purpuric rash develops, and there may be an urticarial eruption. Many of the joints become swollen and painful, and relapses are commonly seen at the same time each year. Each attack lasts for months but is seldom fatal, and there does not appear to be any connection with acute rheumatism.

In Henoch's Purpura there is the typical purpuric rash with abdominal colic, vomiting, and diarrhoea or constipation. Blood may be passed in the stools which look tarry. The condition may resemble acute appendicitis. Joint pains and relapses are common, and occasionally nephritis develops, with a high death-rate.

In Haemorrhagic Purpura, which is usually seen in girls, the onset is abrupt after a few days of slight malaise. There is a severe purpuric rash with haemorrhages from the nose, gums and other parts, and fever, joint pains, and vomiting; nephritis may develop, and the anaemia becomes more pronounced. Death may occur within a few days from general weakness, or improvement may set in within one or two weeks.

Treatment

The patient should rest in bed, and have a light diet. The limbs must be kept warm, and any bleeding must be checked as far as possible by adrenaline or cauterisation, etc. Drugs are of doubtful value, but iron tonics are given during convalescence.

Blood transfusions are given in serious conditions. The cortisone hormones will produce remissions in many cases, especially if given in conjunction with blood transfusion. In severe cases in adults the spleen may have to be removed.

The condition often improves as the patient gets older.

Haemophilia

In this familial disease there is a dangerous tendency to persistent bleeding, and when an accident or operation involving loss of blood occurs there is extreme danger to life, since the bleeding is difficult to control owing to a lack in the blood of a clotting substance, antiharmophilic globulin or factor VIII.

The condition affects males but is passed through the family by the female line. Daughters of haemophiliac fathers should not bear children if they marry; mortality is high among haemophiliacs however great the care that is taken.

Haemophiliacs must strenuously avoid all risk of loss of blood, as from dental extractions or minor abrasions. If bleeding occurs, fresh plasma or concentrates of the missing factor must be given promptly. Sometimes transfusions of whole blood may be needed.

Leukaemia

Leukaemia is a disease of the blood in which enormous numbers of partly developed white cells are formed, owing to disturbances in the work of the bone marrow, lymph glands or spleen. In myeloid leukaemia the bone marrow's output of white cells is at fault, whereas in lymphatic leukaemia the activity of the lymph glands and spleen seems to be uncontrolled. The white cells may be increased up to as much as 10,000 to 400,000 per cubic millimetre of blood, compared with a normal level of 7,000.

Both the myeloid form and the lymphatic form of the disease may occur as either an acute or a chronic condition. There is little difference in symptoms between the two acute types and the two chronic types of leukaemia; the outlook is very grave in all cases of leukaemia, and the chronic form is always fatal eventually.

Acute Leukaemia

In acute leukaemia the total white cell count is sometimes little changed, but many abnormal cells are present. The disease is very rapid in onset, being ushered in with vomiting, headache, small or large haemorrhages into the skin and from the gums, nose, womb and alimentary tract.

The patient quickly becomes anaemic, because accompanying the great increase in the white cells, the red cells become considerably reduced in number. The gums are spongy and the teeth loosen, the breath being very foul owing to the inflammation of the gums. The temperature is usually about 37-5°-39° C (100°-102° F).

The course of this disease is usually very rapid; the patient may die in a few days, and in any case seldom lives for more than a few months. Occasionally the symptoms subside, and the condition becomes more like that seen in chronic leukaemia.

Treatment is aimed at obtaining a remission of the disease for months or years.

Medical treatment is not universally established and many drugs, and combinations of drugs are used to obtain a remission of the disease. Such drugs include the corticosteroids, e.g., prednisolone, the cytotoxic drugs, and alkylating agents. One combinairon is used to attain remission and a different regime is used for maintenance.

X-ray therapy is used in certain cases.

In the lymphatic type of leukaemia excision of the spleen may be considered.

Supportive treatment often includes such measures as blood transfusions, and administration of antibiotics to ward off the infection to which the patient is prone.

Bone marrow transplantation may have a place in the further treatment.

Chronic Leukaemia

The cause of the disease is not known. It is important to make an accurate diagnosis between the acute and the chronic types of leukaemia since more effective treatment is available for the chronic form. Chronic leukaemia is commoner in men than in women and the symptoms are stow and insidious.

The patient becomes anemic, breathless, and weak, and he may notice that the abdomen is becoming larger, owing to a great increase in the size of the spleen. There may be various haemorrhages as in the acute disease. The glands of the armpits, neck and groins are often enlarged. A complete blood examination must be carried out to confirm the diagnosis in a suspected case.

Treatment

The patient need not as a general rule be confined entirely to bed, although he must lead a quiet life, with daily rest periods. In the later stages the patient is generally bedridden.

All forms of infection, such as colds or influenza, must be avoided. The disease is invariably fatal although the patient often lives several years (sometimes for eight to ten years), if treatment by X-rays, by such drugs as busulphan (Myleran), and chlorambucil, alone or in combination with prednisolone, is given. Blood transfusion is often beneficial when the anaemia is severe, and iron tonics should be given.

The diet should be varied and nourishing, and contain a liberal supply of fats, bacon and cereals. When the appetite is poor, a bitter tonic may be ordered.

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