Conditions Related to Immune Thrombocytopenia: Autoimmune Diseases and More

Immune thrombocytopenia (ITP) can occur on its own or along with another health condition. Most people have primary ITP, which means no clear cause can be identified.

Working with your doctor to identify any related conditions is key to managing your ITP.

This article covers several conditions related to ITP and explains how they may affect your diagnosis, treatment plan, and prognosis (outlook).

Autoimmune Diseases and Immune Thrombocytopenia

ITP is generally divided into two types — primary and secondary.

Primary ITP has no identifiable cause. Secondary ITP develops because of another health condition. Causes may include an autoimmune disease, infection, immune deficiency, certain cancers, or some medications.

Some people are first diagnosed with primary ITP and later learn that another condition is involved. Studies have found that misdiagnosis can happen, but the reported rates vary depending on the group studied.

Several autoimmune diseases are linked to secondary ITP. Knowing the signs and symptoms of these conditions can help you get diagnosed and treated sooner.

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE), the most common form of lupus, is an autoimmune disease that can affect the skin, joints, and other organs.

Low platelet counts are fairly common in people with lupus. However, not every case of low platelets in lupus is caused by ITP. Some people who are first diagnosed with primary ITP are later diagnosed with lupus.

In some cases, ITP can be the first sign of SLE. Easy bruising and bleeding occur because the immune system attacks platelets. These blood cell fragments help your blood clot.

Lupus and ITP may also affect how well the body makes new platelets. In some people, platelet production is reduced even when the number of platelet-making cells is normal or increased.

Severe ITP is also linked to poorer outcomes in lupus. People with severe thrombocytopenia may have more severe symptoms and a higher risk of organ damage.

Symptoms of SLE that may occur alongside signs of ITP include:

• Fatigue (extreme tiredness that doesn’t improve with rest)
• A butterfly-shaped rash across the nose and cheeks
• Headaches
• Mouth or nose sores
• Joint pain and swelling
• Hair loss
• Low-grade fever

Lupus Medications

Some lupus medications can also cause thrombocytopenia. Depending on the drug, this may happen by reducing platelet production in the bone marrow or through other mechanisms. Examples in lupus include:

• Azathioprine
• Cyclophosphamide
• Mycophenolate mofetil

Autoimmune Thyroid Disease

Autoimmune thyroid disease develops when the immune system attacks the thyroid gland. This can cause the thyroid to become overactive or underactive.

Hashimoto’s Disease

Hashimoto’s disease causes a drop in thyroid hormones, leading to hypothyroidism. As thyroid function slows, symptoms such as the following can develop:

• Fatigue and sleepiness
• Dry skin
• Hair loss
• Muscle and joint pain
• Swelling in the neck
• Sensitivity to cold

Another symptom of Hashimoto’s disease is heavy or irregular menstrual bleeding. This can also be a sign of ITP.

Studies suggest that Hashimoto’s disease and ITP may occur together more often than expected. Researchers think shared immune system or genetic factors may play a role, but the exact reason for this risk is not yet clear.

Graves’ Disease

Graves’ disease, a common cause of hyperthyroidism, is also associated with ITP. In one study of 248 people with ITP, 13 had Graves’ disease.

Signs of Graves’ disease include:

• Fatigue and trouble sleeping
• Losing weight despite an increased appetite
• Sensitivity to heat
• Palpitations (fast heartbeats)
• Tremors in the hands
• Nervousness and irritability
• Bulging eyes

Sjögren’s Disease

Sjögren’s disease is an autoimmune disorder that affects the glands that make tears and saliva. The most common symptoms are dry mouth and dry eyes. Some people also have dry skin, rashes, and swollen, painful joints.

Some studies have found that ITP can occur in people with Sjögren’s disease, but the reported rates vary. ITP may develop before, at the same time as, or after Sjögren’s is diagnosed.

Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) develops when the immune system makes antibodies that target proteins linked to certain fats in cell membranes. These antibodies can raise the risk of blood clots.

Blood clots can be life-threatening, so it’s important to watch for symptoms such as:

• Shortness of breath or chest pain
• Abdominal pain
• Pain in the neck, jaw, back, arms, or legs
• New swelling and discoloration in an arm or leg

APS can also cause low platelet levels. In many people, the decrease is mild. APS is best known for increasing the risk of blood clots and pregnancy complications. However, the risk of bleeding may rise if platelet counts become very low or if blood thinners are being used.

Researchers think several immune system processes may contribute to low platelet counts in APS. These may include increased platelet destruction and increased platelet use as blood clots form.

Other Conditions Linked to Immune Thrombocytopenia

In addition to autoimmune diseases, several other health conditions are linked to ITP.

Chronic Infections

Infections can activate the immune system. In some people, this immune response may also damage platelets and contribute to ITP.

Some infections linked to ITP can be chronic (long-lasting) or remain inactive in the body and reactivate later. Examples include:

• Cytomegalovirus — A common virus that may cause fever, sore throat, and fatigue and can remain inactive in the body after infection
• Helicobacter pylori — A bacterial infection that can cause stomach ulcers
• Hepatitis C — A viral infection that affects the liver, causing inflammation
• Human immunodeficiency virus (HIV) — A viral infection that attacks immune cells called CD4 T cells
• Varicella zoster — The virus that causes chickenpox, stays inactive in the body, and may later reactivate as shingles

Certain Types of Cancer

Blood malignancies (cancers) can be associated with ITP, such as:

• Chronic lymphocytic leukemia (CLL)
• Small lymphocytic lymphoma (SLL)

From 1 percent to 5 percent of people with CLL and SLL have ITP. Researchers think abnormal immune cells, including T cells that attack platelets, may contribute.

Many people with blood cancers have low platelet counts for reasons other than ITP. A sudden drop may be caused by ITP, an infection, a medicine reaction, or a clotting problem. Doctors use blood tests, a medical history, and sometimes a bone marrow evaluation to find the cause.

Chemotherapy often lowers platelet counts by reducing platelet production in the bone marrow rather than by causing ITP. Rarely, immune-related low blood cell counts can develop after a stem cell transplant. Your doctor will monitor your platelet counts during treatment.

Treatments for ITP and low platelet counts due to cancer are very different. You’ll work closely with your doctor to find the cause of your thrombocytopenia and the best way to manage it.

Immune Deficiencies

Immune deficiencies, also called immunodeficiencies, can contribute to ITP. In these conditions, the immune system doesn’t work properly, making it harder for the body to fight infections.

People with immune deficiencies are more likely to have repeated infections. They’re also at higher risk of thrombocytopenia, including ITP.

Research suggests that ITP related to immune deficiencies may be more difficult to treat and may be resistant to treatments.

Join the Conversation

On myITPteam, people share their experiences with immune thrombocytopenia, get advice, and find support from others who understand.

What ITP-related conditions do you have? Share your story in the comments below.