Werlhof’s disease, also known as Werlhof’s disease and immune thrombocytopenia, is an autoimmune disease. The formation of antibodies against the body’s own blood platelets (thrombocytes) is characteristic of those affected. The disease requires extensive medical treatment and therapy.
What is Werlhof’s disease?
The general practitioner Paul Gottlieb Werlhof (1699-1767) first described Morbus maculosus haemorrhagicus as a blood disease in 1735. It was not until 1883 that it was diagnosed that the signs of the disease were generated by a reduction in blood platelets. See fun-wiki for What is AMC used for.
Morbus Werlhof, the Werlhof disease, like so many others, bears the name of its discoverer, Paul Gottlieb Werlhof. A characteristic of the disease is that the body mistakenly attacks its own platelets, leading to their rapid breakdown. This is why bleeding occurs in the untreated course.
Causes
Werlhof’s disease belongs to the group of autoimmune diseases: The immune system forms antibodies against the body’s own blood platelets, the thrombocytes. They are components of white and red blood cells and play an important role in blood clotting because the disc-shaped platelets can close wounds by cross-linking.
In the case of Werlhof’s disease, the number of platelets decreases significantly because the platelets overloaded with antibodies are broken down too quickly in the spleen. While the platelet count in the laboratory is on average 140 to 360 thousand/μl, the tendency to bleeding increases as the platelet concentration in the blood decreases. Petechiae are the typical appearance.
Petechiae are pinpoint sized hemorrhages from the capillaries of the mucous membranes or the skin. First, the petechiae are usually found on the lower leg or ankles. If the lack of blood platelets is the cause of the bleeding, it is called thrombocytopenic purpura.
If the proportion of thrombocytes decreases dramatically, this is referred to as primary immune thrombocythemia. This deficiency can assume life-threatening proportions. In addition, the trigger of the primary immune thrombocythemia is not known according to the current state of knowledge. Studies have shown that Werlhof’s disease often has to be diagnosed after infections and/or pregnancies.
In children, the disease occasionally occurs spontaneously, only to heal just as spontaneously after some time. This pleasing course can also be observed in adulthood, but only in the first year of illness. After a year Werlhof’s disease becomes chronic.
Symptoms, Ailments & Signs
In Werlhof’s disease, there is a sharp decrease in blood platelets (thrombocytes). As a result, the smallest injuries in the blood vessels can no longer be closed, so that blood leaks out of the arteries and veins. The clinical picture of Werlhof’s disease is correspondingly diverse.
In the initial phase, small punctiform bleeding, so-called petechiae, are among the characteristic symptoms. These can occur both on the skin and on the mucous membranes. The resulting bleeding into the skin or mucous membranes is about the size of a pin and is often mistaken for flea bites.
If the number of blood platelets continues to fall, the bleeding can spread and the individual petechiae coalesce to form flat skin lesions. Even minor injuries result in large bruises (hematomas). Severe nosebleeds and bleeding that can hardly be stopped in the case of minor cuts or abrasions also indicate a reduced number of thrombocytes in the blood.
Vaginal bleeding can also occur in women. Blood can also appear in the stool of both men and women. A pronounced Werlhof’s disease with bleeding in internal organs such as the spleen, liver, lungs or kidneys can also result in a life-threatening shock.
Diagnosis & History
A steadily and sharply decreasing number of blood platelets means that injuries to the blood vessels cannot be closed again. This leakage of blood generates various symptoms:
In the beginning there are the pinhead-sized petechiae. When they flow together, there is widespread bleeding. Bruising, profuse nosebleeds, blood in the urine and stool, vaginal bleeding, and vomiting blood are typical symptoms. Severe bleeding can result in acute shock symptoms.
If the organism is supplied with too little oxygen, whereby blood loss can be a significant cause in Werlhof’s disease, the body begins to prevent unimportant organs from being supplied with oxygen. This condition can become life-threatening if left untreated. If the brain, lungs, liver, spleen or other internal organs are affected, massive damage and functional disorders can also occur.
Werlhof’s disease can be identified on the basis of the blood count, because the number of thrombocytes is significantly reduced. After examining the bone marrow, however, the doctor will diagnose a significant excess of blood-forming megakaryocytes. In the course of diagnosis, other platelet diseases must therefore be ruled out.
Complications
Werlhof’s disease causes significant limitations and complaints in everyday life for the patient. In most cases, there is increased bleeding, which can also occur internally. Without treatment, life expectancy is significantly reduced because irreversible damage to the organs can occur.
It is also not uncommon for the affected person to experience nosebleeds and reduced resilience. The blood is also found in the stool or urine and can lead to a panic attack or sweating in many people. It is not uncommon for those affected to vomit blood. Likewise, the body is undersupplied with oxygen, which can also irreversibly damage the internal organs.
The liver and spleen in particular can lead to a life-threatening condition for the patient. The damage to the organs leads to further complaints and complications. Treatment of this disease takes place acutely with the help of drugs. Complications usually do not arise.
In severe cases, surgery or an organ transplant may be necessary to keep the person alive. This may also reduce the patient’s life expectancy.
When should you go to the doctor?
If the affected person suddenly suffers from rapid formation of bruises or discoloration of the skin, there is cause for concern. A doctor is required in the event of dizziness, increased blood loss, circulatory disorders or a decrease in physical resilience. If there is a lack of concentration, heavy bleeding from minor injuries, a repeated taste of blood in the mouth or blood in the excretions, a doctor’s visit is necessary.
An inner weakness, a general malaise or a feeling of illness should be examined and treated. Bruises, abnormalities in the female menstrual cycle or uncontrolled bleeding from the vagina are warning signals from the organism. There is a health problem that needs to be diagnosed and treated as soon as possible. In the case of changes in the mucous membranes, skin pallor or increased sensitivity to cold stimuli, a doctor should be consulted.
Since the disease can lead to bleeding of the organs and thus to a life-threatening condition, a doctor’s visit is recommended at the first signs. If an acute health-threatening condition occurs, an ambulance is required. An ambulance must be called and, at the same time, first-aid measures must be initiated by those present to ensure the survival of the person concerned.
Treatment & Therapy
Once the doctor has diagnosed Werlhof’s disease, various treatment methods are used. The first priority is stopping acute bleeding. For this purpose, high-dose corticosteroids are used as part of the “first-line therapy”. This shock therapy, which has side effects, ends after a relatively short time and can result in a thorough regression of the symptoms of the disease after just a few weeks.
Children with a mild course of the disease do not necessarily have to be subjected to this therapy. Werlhof’s disease usually occurs briefly as a result of viral infections or occasionally after mumps, measles or rubella vaccinations, and then heals on its own. Chronic courses in adults are further treated with antibodies, so-called immunoglobulins, for example biological rituximab or other immunosuppressants. This is to fight the autoimmune disease.
Manifest clinical pictures are counteracted with the surgical removal of the spleen. The advantages of healing Werlhof’s disease must be weighed against the possible complications of the operation. Despite removal of the spleen, recurrences occur and the risk of death is one percent.
Outlook & Forecast
In general, the prognosis for adult sufferers of Werlhof’s disease is favorable and the overall cure rate is between 70 and 80 percent. For an accurate prognosis, however, a distinction must be made between acute and chronic forms.
In most cases of acute Werlhof’s disease, complete remission (= reduction in symptoms) occurs within a month. This applies in particular to affected children. In the case of chronic courses, on the other hand, spontaneous remission occurs only in rare cases (less than 5 percent). This value improves considerably under steroid therapy and rises to 25 percent, and even to two-thirds with the addition of further therapy measures. About 0.4 percent of adults with severe chronic Werlhof’s disease before the age of 40 die of an intracerebral hemorrhage (bleeding in the brain tissue). This probability increases with age and is 1.2 percent for the 40 to 60 year olds and 13 percent for the over 60 year olds.
In addition, recurrences (recurrence of symptoms) often occur in Werlhof’s disease. In these cases, the specialists often consider surgical removal of the spleen (splenectomy). But even a spleen removal does not completely neutralize the risk of recurrence.
Prevention
Since the exact cause of Werlhof’s disease is still being researched and the disease is also an autoimmune disease, preventive measures are not yet known.
Aftercare
In most cases, those affected by Werlhof’s disease have no special or direct follow-up measures available, so ideally those affected by this disease should see a doctor very early on to prevent the occurrence of other complications and symptoms. Since this is a genetic disease, it cannot usually be completely cured.
Those affected should therefore seek advice, especially if they wish to have children, so that the disease does not recur in their descendants. As a rule, those affected by Werlhof’s disease are dependent on taking various medicines. A correct dosage and regular intake should always be observed, and a doctor should be consulted if you have any questions or are unclear.
Many of those affected are also dependent on regular checks and examinations of their internal organs. In this way, other damage can be detected and treated early. It is not uncommon for contact with other people affected by the disease to be very useful, as this can also lead to an exchange of information. This information can make everyday life easier for the patient.
You can do that yourself
Werlhof’s disease cannot be treated by the patient themselves. However, targeted measures and a change in lifestyle can alleviate the symptoms and symptoms of the autoimmune disease. The typical skin changes can be covered with make-up or with the right clothing. This is particularly necessary in the later stages of the disease, when the bleeding and scarring have already spread to the entire arm and neck. If severe hematomas suddenly occur, they can be cooled with the help of compresses or cold packs.
In the long term, the sick have to change their diet. This makes the immune system more resistant to infections. In the case of reddened eyes, bleeding gums and vomiting blood, natural remedies such as aloe vera, sage, devil’s claw or ginseng can help in addition to the medication prescribed by the doctor.
If the measures mentioned do not alleviate the symptoms and symptoms of Werlhof’s disease, the doctor must be consulted again. The medication may need to be adjusted or there may be another condition that needs to be diagnosed. Visiting a self-help group makes it easier to deal with the disease and can thus contribute to a better quality of life.
