Lupus and thrombocytopenia: What to know

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Thrombocytopenia occurs because either:

• The body cannot make enough platelets.
• The body destroys the platelets it does make.
• The spleen holds on to too many platelets.

In the case of lupus, the body destroys blood platelets. This occurs because lupus is an autoimmune disease. In autoimmune diseases, the immune system mistakenly attacks healthy cells.

Experts think that thrombocytopenia affects 20–40% of people with lupus.

Thrombocytopenia is a common blood disorder associated with lupus.

A 2017 paper suggests that the earlier thrombocytopenia occurs in the disease course, the more severe the symptoms and the less positive the outlook.

For example, a 2016 study found that the risk of death in people with lupus was significantly higher if thrombocytopenia was severe. The risk almost doubled from 9% to 15%. People with lupus are more likely to die if they do not experience complete remission of thrombocytopenia during treatment.

In about 16% of cases, thrombocytopenia is the first indication that a person has lupus. It may even be present up to a decade before the person receives a diagnosis of lupus.

Research also suggests that certain immunosuppressant drugs that doctors use to treat lupus can cause thrombocytopenia. Examples include azathioprine and methotrexate.

Rarely, treatments for lupus that contain hydroxychloroquine may also cause thrombocytopenia.

Thrombocytopenia is a common blood disorder in people with lupus, but certain risk factors may make severe or chronic thrombocytopenia more likely.

A 2016 study involving 230 people with lupus and thrombocytopenia found that severe thrombocytopenia was more likely among those who:

• were older in age at the onset of lupus
• were assigned female at birth
• had renal, heart, or nervous system problems

Thrombocytopenia associated with lupus may not always cause symptoms, but there may sometimes be signs of a low blood platelet count, such as:

• bleeding gums or nose
• prolonged bleeding from cuts
• blood in the stool, urine, or vomit
• heavy periods
• a red, purple, or brown dotted rash called petechiae that can occur anywhere but typically appears on the arms, neck, and lower legs
• hematoma, which is a lump due to clotted blood under the skin
• frequent bruising
• rectal bleeding
• feelings of fatigue
• an enlarged spleen

If the symptoms of thrombocytopenia are mild to moderate, a person may not need treatment to manage the condition. However, a doctor may want to monitor the individual’s platelet count regularly. Children will usually improve without any treatment over the course of a few weeks or months.

The condition does tend to worsen over time, and it may become chronic or severe. Severe thrombocytopenia needs immediate treatment and careful management.

Medication

The initial treatment usually involves corticosteroids, such as prednisolone or methylprednisolone, or intravenous antibodies, known as immune gammaglobulin.

Follow-up treatment may include a combination of multiple medications, such as hydroxychloroquine, danazol, or azathioprine, to boost blood platelet count by reducing the immune system response.

Once the blood platelet count is back to a safe level, doctors usually recommend discontinuing treatment.

Surgery

Sometimes, people with chronic or severe thrombocytopenia will need to undergo surgery to remove the spleen, but this procedure does not work for everyone.

Living without a spleen can cause other complications, such as being more susceptible to infection. For this reason, people with lupus who develop thrombocytopenia should consider all options and side effects before embarking on treatment.

After a splenectomy, people should keep a close eye on their health and watch for signs of infection, such as a fever.

Other treatments

If surgery and medication do not help improve thrombocytopenia, a doctor may prescribe thrombopoietin receptor agonists, such as eltrombopag or romiplostim. These help boost platelet production.

Rarely, thrombocytopenia can cause severe bleeding, which may require hospitalization and a transfusion of donor platelet concentrates. The transfusion process will inject donor platelets into the person’s bloodstream.

People with thrombocytopenia should avoid some common drugs that may increase the risk of bleeding, such as aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs.

People with thrombocytopenia resulting from an autoimmune condition should avoid activities that may cause head injury to decrease the risk of bleeding in the brain.

Taking extra care when cycling, driving, and using sharp tools will also help reduce the risk of an injury that may cause bleeding.

Thrombocytopenia is a condition that commonly affects people with lupus. If the condition is mild to moderate, a person may not need any treatment.

However, severe thrombocytopenia is associated with a higher risk of mortality, and a person may require medication, hospitalization, or surgery.