Introduction:
Hematologic disorders affect the whole body as they involve the main circulating component - the blood. The manifestations of such disorders can be appreciated clinically in various systems like the cardiovascular and excretory systems. One such important hematologic disorder is thrombocytopenia, with a reduced blood count of platelets (thrombocytes).
What Is Immune Thrombocytopenia?
This condition was previously called the idiopathic thrombocytopenic purpura. This is an autoimmune condition with an altered immune response in the body. The body’s immune system understands its platelets as foreign bodies (antigens) and, hence, tries vigorously to eliminate them from the body. In the due process, several antibodies are produced to target the so-called antigens (platelets). As a result of this action, platelets are destroyed, which leads to a downturn in the number of platelets in the body. This condition is called immune thrombocytopenia. The reason for this action by the immune system is not elicited yet. However, several genetic, environmental, and personal factors are considered to be part of the factors responsible for the causation of immune thrombocytopenia.
What Are the Clinical Manifestations of Immune Thrombocytopenia?
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Skin can show purplish spots (petechiae) scattered throughout the body. This is because underlying bleeding spots (hemorrhagic sites) are under the skin. When these bleeding spots are large, they are seen as ecchymosis.
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Purpura are seen as reddish to purplish bleeding areas in the skin and mucous membrane.
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The timing for normal clotting is prolonged. This can cause several complications during surgery or after a traumatic event due to excessive bleeding from the affected sites.
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Mucous membranes in the body are highly susceptible to bleed readily. This is seen clinically as nosebleeds or bleeding of gums in the oral cavity.
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Women may complain of excessive bleeding during their menstrual period (menorrhagia).
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There can be bleeding within the gastrointestinal (GI) tract. If the bleeding is in the upper part of the stomach or the GI tract, the stools are seen as black (tarry stools). This condition is called malena. Sometimes, people tend to vomit blood due to internal bleeding (hematemesis).
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Patients with immune thrombocytopenia are highly prone to develop bleeding within the brain and cranial cavity (intracranial hemorrhage). This can be a dangerous condition as it can lead to loss of blood volume and result in shock and death of the individual.
How Does Spontaneous Oral Purpura Occur in Immune Thrombocytopenia?
Purpura can occur anywhere in the body with thrombocytopenia. However, the oral tissues are commonly seen as they have a delicate and closely packed network of fragile blood vessels. The oral cavity is also a highly vascularized area. Activities with mild friction, like chewing, brushing, or speaking, can also create bleeding spots in the oral cavity due to their fragile nature. The platelets are required to seal a damaged blood vessel in the oral cavity. In immune thrombocytopenia, this does not occur as there is a dysregulation in the hemostatic mechanisms in the body.
Consequently, even minor trauma to the oral mucosa can result in bleeding from these fragile blood vessels, leading to the characteristic appearance of purpura. In immune thrombocytopenia, specifically, the autoimmune-mediated destruction of platelets further exacerbates the risk of bleeding, including oral purpura. Such purpuric lesions can be appreciated clinically as bleeding spots in the gums, lips, and palate. Patients may complain of unstoppable bleeding, especially after brushing. The buccal mucosa can also show petechial spots in some cases.
How to Diagnose Immune Thrombocytopenia?
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Clinical features like easy bruising, bleeding from mucosal sites like the nose and oral cavity, and petechiae, purpura, or ecchymosis should be checked thoroughly in the patient. Tiny bleeding spots are visible on the skin. This can help in including immune thrombocytopenia as a part of the differential diagnosis.
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Confirmatory diagnosis of the condition is possible only through laboratory hematological conditions. Hematological investigations will show reduced platelet numbers in the body. This occurs when the count goes to less than one lakh cells (100,000) per cubic millimeter.
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A peripheral blood smear of the obtained sample can show the platelets are of improper quality in the case of thrombocytopenia. This is due to structural deformity of the platelets in the circulating blood. However, in cases of immune thrombocytopenia, the structure of platelets looks normal because there is no damage to the platelet structure. This helps differentiate immune thrombocytopenia from other causes.
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Other serological tests will detect the presence of antiplatelet antibodies.
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Laboratory investigations also include performing kidney and liver function tests.
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In some cases, a bone marrow aspiration and biopsy are ordered by physicians to evaluate the synthesis and differentiation of blood cells within the bone marrow. This procedure is specifically reserved for individuals with atypical clinical features, inadequate response to treatment, or suspicion of other concurrent hematologic disorders.
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Laboratory investigations will be done to rule out all other causes of thrombocytopenia, like viral infection, neoplasm, or autoimmune disorders. This will help confirm the cause of thrombocytopenia, and a treatment plan can be made accordingly.
How to Treat Spontaneous Oral Purpura in Immune Thrombocytopenia?
Spontaneous bleeding or purpura involving the mucosa in the oral cavity is due to underlying thrombocytopenia (an abnormal immune response). Treating the underlying thrombocytopenic condition can help treat the purpuric lesions.
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When the bleeding due to immune thrombocytopenia is severe and life-threatening, blood transfusions are done to increase the thrombocyte count and achieve optimal hemostasis. This is reserved for essential cases as the probability of incidence of transfusion reactions is very high.
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Corticosteroids are drugs that suppress the immune action in the body, and hence, they are given in this case.
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Intravenous immunoglobulins are given in high doses to modulate the immune response in the body and increase the platelet count.
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Another class of drugs given are immunosuppressants. Rituximab, Azathioprine, and Cyclosporine are commonly given for immune thrombocytopenia.
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Thrombopoietin receptor agonists like Eltrombopag stimulate platelet production by mimicking the action of thrombopoietin (a hormone responsible for producing platelets). In this way, the number of platelets is increased, and the risk of bleeding is substantially reduced.
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Hemostatic agents like Tranexamic acid mouthwash and other local application methods are employed to arrest spontaneous bleeding in the oral cavity.
Conclusion:
Spontaneous oral purpura is a clinically significant symptom of immune thrombocytopenia. Treating this involves local and systemic drug usage to modulate the underlying cause of bleeding. Educating patients about gentle brushing and avoiding mechanical trauma to oral soft tissues is essential to prevent purpuric lesions in immune thrombocytopenia.
