Purpura

 

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Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Kylie Smith, Lucinda hampton, Kim Jackson, Elaine Lonnemann and WikiSysop  

Definition/Description

Purpura is a hemorrhagic condition that occurs when not enough normal platelets are available to plug damaged vessels or prevent leakage from even minor injury to normal capillaries. Purpura is characterized by movement of blood into the surrounding tissue (extravasation), under the skin, and through mucous membranes, producing spontaneous ecchymoses (bruises) and petechiae (small, red patches) on the skin [1]. Purpura measure between 4 and 10 mm (millimeters) in diameter. When purpura spots are less than 4 mm in diameter, they are called petechiae. Purpura spots larger than 1 cm (centimeter) are called ecchymoses [2]. There are many different types of purpura. Their classification depends on the appearance or cause of the condition.

Idiopathic thrombocytopenic purpura (ITP), also known as primary immune thrombocytopenic purpura and autoimmune thrombocytopenic purpura, is defined as isolated thrombocytopenia with normal bone marrow and in the absence of other causes of thrombocytopenia. ITP has two distinct clinical syndromes, with typical manifestation of an acute condition in children (spontaneous resolution within 2 months) and a chronic condition in adults (lasting longer than 6 months) [3].

Prevalence

Frequency

• The incidence of ITP in adults is approximately 66 cases per 1,000,000 per year
• An average estimate of the incidence in children is 50 cases per 1,000,000 per year [3].

Age

• Peak prevalence occurs in adults aged 20-50 years.
• Peak prevalence occurs in children aged 2-4 years.
• Approximately 40% of all patients are younger than 10 years [3].
  
  

Characteristics/Clinical Presentation

Physical signs and symptoms include:

• Easy or excessive bruising (purpura)
• Superficial bleeding into the skin that appears as a rash of pinpoint-sized reddish-purple spots (petechiae), usually on the lower legs
• Bleeding from the gums or nose
• Blood in urine or stools
• Unusually heavy menstrual flow [4]
• Non-blanchable lesions [5]

Idiopathic thrombocytopenic purpura can occur in anyone at almost any age, but these factors increase the risk: [4]

• Your sex. Women are two to three times more likely to develop ITP than men are.
• Recent viral infection. Many children with ITP develop the disorder after a viral illness, such as mumps, measles, influenza or a respiratory infection.
  
  

Associated Co-morbidities

ITP is associated with a considerable number of medical conditions including hematological diseases, dermatological conditions, oral conditions, viral infections (such as Hepatitis C, HIV, measles, and mumps), gastrointestinal, bleeding, and autoimmune disorders. One possible cause of associated signs and symptoms could be due to an adverse drug reaction. However, further research is needed to identify the cause and extent of the association between these conditions and ITP [6,7]

Medications

A detailed description of The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia can be viewed here [7]:

http://www.bloodjournal.org/content/117/16/4190?sso-checked=true

The primary focus of a pharmaceutical approach to the treatment of ITP is to raise the individual’s platelet count. They may also be asked to stop taking medications that impair platelet function, such as aspirin, blood thinners, and ibuprofen [8].

Corticosteroids

The doctor may start with a corticosteroid medication, which can help increase the platelet count by decreasing the activity of the immune system. It usually takes about two to six weeks for their platelet count to return to a safe level. When it does, the doctor will discontinue the drug [8].

Intravenous immunoglobulin

If your purpura is causing severe bleeding, your doctor may give you an intravenous medication called intravenous immunoglobulin (IVIG). They may also give you IVIG if you need to increase your platelet count rapidly before surgery. This treatment is usually effective in increasing your platelet count, but the effect is usually only in the short term. It can cause side effects such as headache, nausea, and fever [8].

Other drug therapies

The latest drugs used to treat purpura are romiplostim (Nplate) and eltrombopag (Promacta). These medications cause bone marrow to produce more platelets, which reduces the risk of bruising and bleeding. Potential side effects include:

• headaches
• dizziness
• nausea
• joint or muscle pain
• vomiting
• increased risk of blood clots
• acute respiratory distress syndrome

Biologic therapy, such as the drug rituximad (Rituxan), can help decrease the immune system response. It’s mostly used to treat patients with severe thrombocyotopenic purpura and patients for whom corticosteroid treatment isn’t effective. Side effects may include:

• low blood pressure
• sore throat
• rash
• fever [8]
  
  

Diagnostic Tests/Lab Tests/Lab Values

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Etiology/Causes

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Systemic Involvement

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Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

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Differential Diagnosis

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Case Reports/ Case Studies

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Resources

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Recent Related Research (from Pubmed)

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References

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