PURPLE DISEASE: SYMPTOMS, CAUSES, TREATMENT - GEOGRAFÍA - 2025

The purple disease, also known as idiopathic thrombocytopenic purpura, is an autoimmune origin pathology characterized by the presence of a platelet disorder. It is usually classified within hematological disorders.

It is defined clinically by the presence of episodes of mucosal and cutaneous bleeding, menorrhagia, persistent bleeding due to trauma, petechiae, purpura, bruising, gingivorrhagia, ecchymosis, etc.

The origin of thrombocytopenic purpura is found in an autoimmune process that generates various antibodies against platelets and their production at the medullary level. Given the clinical suspicion, it is essential to perform various laboratory tests to confirm the diagnosis: hemogram, blood coagulation analysis, tissue biopsy, bone marrow aspiration, etc.

Currently there are various therapeutic approaches for the treatment of purpura disease: plasma exchange, administration of glucocorticoids, antiplatelet agents, splenectomy, etc.

History

Idiopathic thrombocytopenic purpura was first described by the German medical specialist Paul Gottlieb Werlhof in the year 1735. Due to this, in the initial moments it was known as Werlhof's disease.

Later, Paul Kznelson in 1916 published the first report in which he referred to a successful therapeutic approach for thrombocytopenic purpura, splenectomy.

Already in 1951, a group of researchers formed by Willaim, Harrington and Holigsworth associated the characteristics of this pathology with a disorder of autoimmune origin.

Characteristics of purple disease

Idopathic thrombocytopenic purpura is an autoimmune disease characterized by the presence of platelet penia and that usually appears during adolescence. It is a pathology whose basic clinical course leads to the development of bruises and hematomas, sudden or excessive bleeding, among other signs.

At a specific level, this pathology is defined as a type of thrombocytopenia, that is, an abnormal and pathological decrease in blood platelet levels.

Platelets are cells that are part of our blood material. Its essential function is the formation of clots and the repair of blood vessels that are injured and / or damaged.

These cells are specialized in maintaining the structure and integrity of the blood vessels in our body and, in addition, they help prevent and control bleeding by accelerating coagulation.

In optimal conditions, it is expected that we have platelet levels of 150,000 to 400,000 / mc. However, less than 150,000 can cause significant medical complications related to bleeding and blood clotting.

Thrombocytopenia or platelet penia can appear due to various factors:

• The bone marrow does not make enough platelets.
• Platelets are destroyed in the bloodstream.
• Platelets are destroyed in organs such as the liver and spleen.

In the case of idiopathic thrombocytopenic purpura, the absence of platelets is associated with the development of pathological autoimmune processes.

Statistics

Statistical analyzes indicate that thrombocytopenic purpura has an incidence of 1 case per 25,600-50,000 inhabitants in the general population each year.

In the United States, the incidence of this pathology is usually around 3.3 cases per 100,000 inhabitants in adulthood each year. For its part, the prevalence reaches 9.5 cases per 100,000 people.

It can affect anyone, although it has a prevalence ratio of 1.3; 1, being more frequent in the female sex. This disease can also appear in any age group, however, it is more common for its initial manifestations to appear in childhood.

Approximately 40% of the diagnosed cases correspond to patients less than 10 years of age. The prevalence being very high in the age group of 2 to 4 years.

In the pediatric population, thrombocytopenic purpura has an annual incidence of about 5.3 cases per 100,000 children. In the case of males, two age peaks have been identified. It especially affects those under 18 years of age and the elderly.

Symptoms

Thrombocytopenic purpura is a pathology that is mainly related to clinical manifestations of a hemorrhagic nature.

Although in some cases low platelet levels present as an asymptomatic medical condition, some of the signs and symptoms most associated with idiopathic thrombocytopenic purpura include:

Cutaneous and mucosal bleeding

Recurrent and spontaneous bleeding is one of the cardinal signs of this pathology.

People with idiopathic thrombocytopenic purpura are at serious risk of spontaneous bleeding and bruising. Although it can appear in any area, this symptom is more associated with bleeding from the gums or nose.

Blood material can also be seen in waste, such as urine.

In the most severe cases where the platelet count is minimal, the affected people are at risk of suffering fatal bleeding (gastrointestinal, internal, intracranial bleeding, etc.).

Ecchymosis

In the medical field, ecchymosis is the term that is usually used to refer to the presence of bruises, commonly known as bruises. These lesions are defined as an accumulation of blood material at the skin level.

The etiological causes of bleeding under the skin are widely heterogeneous: injuries and trauma, immune disorders, allergic reactions, medical treatments, skin aging, etc.

They can take various presentations or clinical forms, from defined and localized hematomas to large affected skin areas.

Petechiae

Petechiae are a type of disorder related to subcutaneous bleeding. Visually, they are usually identified as a reddish stain, similar to blood. They usually reach an extension of a few millimeters and on a few occasions of centimeters.

Its appearance is due to the escape of a reduced amount of blood from a capillary or blood vessel located under the superficial layers of the skin.

This type of medical finding, along with others, are usually indicators of serious pathologies. They can indicate a vasculitis, thrombopenia, infectious process, etc.

Purple

It is a skin disorder characterized by the appearance of purple lesions in different locations of the skin or in the mucous areas of the body.

Like the previous ones, they are due to blood leaks under the superficial layers of the skin. It usually has an approximate extension of about 4 to 10 mm.

When the purpura reaches an amplitude greater than 4 mm they are called petechiae and if it exceeds one centimeter it is called ecchymosis.

Gingivorregia

This term is used to refer to hemorrhagic episodes that appear suddenly in the gums, at the oral level.

Although it is usually a very frequent medical condition in those frequent in emergency medical services, on some occasions it can be related to serious pathologies, such as carcinomas.

Menorrhagia

Blood clotting abnormalities and bleeding episodes can also disrupt menstrual cycles. In women affected by idiopathic thrombocytopenic purpura, menstrual periods defined by heavy and / or long-lasting bleeding may be observed.

It is a pathology that requires treatment and medical intervention as it can cause various complications such as anemia or episodes of acute pain.

Epistaxis

Epistaxis is the medical term used to refer to nosebleeds.

Other complications

In addition to the signs and symptoms described above, other more serious manifestations may appear that put the survival of the affected people at risk.

The most common are massive internal bleeding or central nervous system bleeding.

Causes

In most of those affected, thrombocytopenic purpura is associated with autoimmune disorders that cause a destruction of platelets.

Ongoing investigations have not yet been able to identify the etiological cause of this immune process, which is why it is often referred to an idiopathic disorder.

In other people, the clinical course of thrombocytopenia purpura may be associated with some risk factors:

• Sex: it is a pathology about three times more common in women than in men.
• Infectious processes: especially in children, an infectious process of viral origin is usually identified prior to the development of purpura. The most common are mumps, measles, or respiratory infections.
• Pregnancy: it is possible that due to pregnancy there is a significant decrease in the level of platelets.

Diagnosis

Faced with suspicious clinical signs and symptoms, an analysis of the family and individual medical history and a complete clinical examination is necessary.

Laboratory tests will be essential: hemogram, blood coagulation analysis, tissue biopsy, spinal aspiration, etc.

Treatment

The most common therapeutic approaches in thrombocytopenic purpura include:

• Administration of corticosteroids: Some types of corticosteroids such as prednisone can increase platelet levels by suppressing the activity of the immune system.
• Administration of intravenous immunoglobulins: it is used as an emergency treatment in cases of severe bleeding or a surgical procedure to rapidly increase the levels of platelets in the blood.
• Administration of thrombopoietin receptor agonists: some drugs such as romiplostmi or eltrombopag help prevent bleeding and bruising.
• Administration of immunuspressants: they inhibit the activity of the immune system to increase platelet levels. Some of the most common are rituximab, cyclophosphamine, or azathioprine.
• Antibiotics: their use is restricted to cases in which it is possible to identify an etiological cause associated with infectious processes.
• Surgery: in some patients, specialists recommend the removal of the spleen to improve symptoms or increase the platelet count.

References

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