Fatigue, frequent infections, unexplained bruising – these are not always just “part of lupus.” They may indicate a low blood cell count, a possibly serious condition called pancytopenia.  What is pancytopenia, its causes, symptoms, diagnosis and treatment options for those living with lupus?  Read on to learn more about this often overlooked condition.

• Introduction: Lupus and Blood Cell Counts
• How Lupus Affects the Blood
• The Symptoms, Diagnosis, Treatments and Living with Pancytopenia
• In Conclusion

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Introduction:  Lupus and Blood Cell Counts

Lupus is a complex autoimmune disease that can affect nearly every part of the body. Joints, skin, kidneys, brain, and heart are the mostly commonly talked about organs. Less visible, but just as important, is lupus’s effect on the blood. One of the most serious blood-related complications is pancytopenia – “pan” meaning “all;” “cyto” meaning “cell, and “penia” meaning “low.”

Pancytopenia is the medical condition when all three major types of blood cells are reduced:

• Red blood cells, which carry oxygen and prevent anemia-related fatigue.
• White blood cells, the very diverse type of blood cells that normally help fight infections, but can also drive autoimmune diseases like lupus.
• Platelets, which allow blood to clot and prevent excessive bleeding.

When all three are low at the same time, the body becomes more vulnerable to exhaustion, infections, and bleeding complications.

Pancytopenia occurs in 10% to 20% of those living with lupus at some point in the course of their disease.

By comparison:

• Anemia, low levels of red blood cells, occurs in about 50–70% of people with lupus;
• Leukopenia, low white cells, in 40–60%;
• Thrombocytopenia, low platelet count, in 20–40%.

Though less common, it can be very serious, so it is good to learn more about it!

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How Lupus Affects the Blood

Blood abnormalities are common in lupus, and they can occur for several reasons:

1. Because lupus is an autoimmune disease, the immune system mistakenly attacks the body’s own tissues, including blood cells. Autoantibodies may target red cells, white cells, or platelets, causing them to be destroyed faster than the body can replace them.
2. Lupus-related inflammation can suppress the bone marrow, where blood cells are produced. When the marrow is affected, production of new blood cells slows down and fewer circulate in the blood stream.
3. Medications used to treat lupus, such as immunosuppressive drugs, can sometimes lower blood counts as a side effect.
4. Infections, which people living with lupus are more prone to suffer, can also temporarily reduce blood cell levels.

Often, pancytopenia results from a combination of these factors rather than a single cause.

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The Symptoms, Diagnosis and Treatments of Pancytopenia

Symptoms

Pancytopenia is frequently under-recognized, because symptoms like fatigue or infections are often attributed to lupus itself rather than to falling blood counts. This is why routine blood monitoring is such a critical part of lupus care.

• Low red blood cell levels can cause fatigue, weakness, shortness of breath, or dizziness.
• Low white blood cell levels may increase the risk of frequent or severe infections.
• Low platelet levels may lead to easy bruising, nosebleeds, bleeding gums, or prolonged bleeding from cuts.

Diagnosis

Pancytopenia is usually detected through a complete blood count (CBC), a standard blood test that is regularly given to those living with lupus. If pancytopenia is found, clinicians may order additional tests to determine the cause, such as:

• Tests for general lupus activity because if low cell counts occur during flares, then lupus autoantibodies may be attacking blood cells directly.
• Checking for infections, in particular Epstein-Barr virus, HIV, hepatitis or some severe bacterial infections.
• A careful review of medications for possible issues, including chemotherapies, alcohol and some antibiotics.
• In some cases, a bone marrow biopsy in case another condition, such as blood cancers or aplastic anemia.
• Rarely, checking for an enlarged spleen, which can trap and destroy too many blood cells.

Identifying the underlying cause is essential because the type of treatment will depend on what is driving each blood count to be so low.

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Treatments and Living with Pancytopenia

The treatment of pancytopenia depends upon the underlying problem … or problems.

• If active lupus is causing the autoimmune destruction of blood cells, increasing or adjusting immunosuppressive therapy, corticosteroids or other immune-modulating medications, may be necessary.
• If medications are contributing to pancytopenia, your treating physician may adjust doses or consider alternatives. In cases of severe anemia or low platelets, transfusions or growth-stimulating medications may be used.
• Infections need to be treated quickly to make sure that they do not become serious.
• Regular blood monitoring is necessary to allow healthcare providers to catch changes early and to make any necessary changes to treatments before complications become severe.

 

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In Conclusion

Pancytopenia from lupus can be frightening, but when it is recognized early and treated appropriately, many living with lupus continue to live full, active lives.  As with any aspect of lupus, education, routine monitoring and open, honest communication with your healthcare team are the keys to achieving your best outcomes!

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References

Ameer, M. A., Tariq, M. A., Zain, S., Kabir, A., & Khawaja, M. (2024). A rare cause of pancytopenia in systemic lupus erythematosus (SLE) in a young patient. Cureus, 16(6), e63032. https://doi.org/10.7759/cureus.63032

Bashal, F. (2013). Hematological disorders in patients with systemic lupus erythematosus. The Open Rheumatology Journal, 19(7) http://dx.doi.org/10.2174/1874312901307010087

Cervera, R., Khamashta, M.A. & Hughes, G.R.V. (2009). The Euro-Lupus Project: epidemiology of systemic lupus erythematosus. Annals in Rheumatic Diseases. 68(9). 1379–1382.

Fayyaz, A., Igoe A. & Kurien, BT. (2015). Haematological manifestations of lupus. Rheumatology (Oxford), 54(1). 2–11.

Hahn, B.H. (2019). Dubois’ Lupus Erythematosus and Related Syndromes. 9th ed. Elsevier

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Author: Greg Dardis, MS      

Professor Dardis was the Chair of the Science Department at Marylhurst University and is currently an Assistant Professor at Portland State University and the American College of Healthcare Sciences.  His focus has been human biology and physiology with an interest in  autoimmunity.  Professor Dardis is also a former President of the Board of Directors of Kaleidoscope Fighting Lupus.

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