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Lupus and Blood Disorders

It is estimated that 85% of lupus patients will experience some form of unhealthy blood condition. In this article, we will take a closer at the blood disorder risks, symptoms and treatments for individuals living with lupus.

 

Introduction

Individuals with lupus may be at a greater risk for developing blood disorders. Chronic inflammation, changes in blood due to SLE or even the very medications used to treat lupus can lead to these disorders. Some blood disorders may be almost asymptomatic, while others may pose more alarming symptoms, such as pain and shortness of breath. While conditions such as some types of anemia can be easily treatable with medications and even changes in diet, others, such as vasculitis, require more aggressive therapy.

Understanding blood and what complications may arise with lupus can help individuals recognize when things seem a little off. Having this information may help to take some of the fear and anxiety away in the event that an individual finds themselves facing yet another health complication.

What is blood?

Blood makes up about 10% of the weight of an average-sized adult (150-180 pounds) – this equates to about one-and-a-half gallons of blood. Adults who are bigger will have more blood, whereas babies only have about a cup of blood flowing through their bodies.

Blood is made up of the following:

  • Erythrocytes: These red blood cells contain the pigment hemoglobin, which gives blood its red color. Erythrocytes are also responsible for carrying and carbon dioxide and oxygen throughout the body, to and from organs and other tissues.
  • Leukocytes: These white blood cells are an essential part of the immune system and help to fight disease and infection.
  • Platelets: Also known as thrombocytes, platelets help blood clot. As with all blood cells, they form in bone marrow. Some individuals do not have enough platelets and may have to rely on donor platelets to recover safely from surgery, etc.
  • Plasma: Plasma is the fluid that carries the above blood components and others, such as, nutrients, throughout the body. Plasma alone makes up about 55% of the volume of blood.

As mentioned, leukocytes, or white blood cells, play an integral role in the immune system and immune response. There are five types of white blood cells with a complex relationship of functions. Each plays a vital role in keeping the body safe from infection and injury.  Here is a brief description of the most common:

  • Lymphocytes are an important and complex group and are often highlighted in research and in the media. There are two main types:  T cells and B cells.
    • T cells come in many varieties and can directly attack foreign invaders, regulate other lymphocytes.Some, called regulatory T cells (or Tregs) work to inhibit the immune response and prevent autoimmune disease!
    • B cells largely create antibodies and help activate T cells.
    • Note: A third special lymphocyte, called the natural killer cells, can attack virus-infected cells and tumor cells.
  • Neutrophils are the most numerous of the white blood cells and are the first line of defense against bacteria and fungi. They release cytokines and activate other cells of the immune system.
  • Monocytes can do many things, but most commonly break down bacteria by consuming them.
  • Basophils secrete histamine during an allergic reaction.
  • Eosinophils help with allergic responses and can also kill cancer cells.

Sometimes, these cells and the other components of blood can become out of balance. This often happens in individuals with lupus due to the nature of SLE, how the body tries adapt to the autoimmune response, overlapping conditions and even treatment plans.

What causes a blood disorder?

Scientists believe that the autoimmune response and the production of antibodies can damage the blood cells in individuals with lupus. Blood disorders can occur for various reasons depending on which cells are affected.

Low White Blood Cell Counts:  Over 80% of individuals with lupus may have abnormal white blood cell counts or more specifically, low lymphocyte counts. These low counts may be attributed to the following:

  • the production of antibodies that specifically target lymphocytes; and
  • immunosuppressants used to treat severe forms of lupus such as lupus nephritis.

Because the body should always be producing lymphocytes, there are usually enough to fight infection, and a true blood disorder may never develop. A healthcare practitioner will routinely check blood levels, however, to make sure everything looks normal.

Low neutrophil counts may be the result of:

  • Ethnicity: Individuals of Afro-Caribbean decent both with and without lupus often have low neutrophil levels.
  • Azathioprine: Sometimes known as Imuran, azathioprine is often prescribed for more severe lupus symptoms or after kidney transplant and may cause a dip in neutrophil counts.

 

Low Red Blood Cell Counts:  Some individuals with lupus may experience low red blood cell counts. This condition may be due to the following:

  • the destruction of red blood cells by antibody production;
  • decreased red blood cell production due to inflammation throughout the body;
  • excessive bleeding; and
  • iron, vitamin B12 and/or folate deficiencies.

 

Low Platelet Count:  Low platelet count may be caused by the presence of antibodies that affect platelet production and how blood clots. This disruption is often caused by antiphospholipid syndrome (APS). Severe APS can cause pregnancy complications, heart attacks and even stroke.

Other Underlying Health Conditions Which May Cause Blood Disorders

  • Renal failure: Researchers have found that chronic renal failure may cause conditions such as anemia.
  • Immune mediated bone marrow failure syndromes (BMFS): Though rare, BMFS can occur when there are changes to the production of red and/or white blood cells and platelets in bone marrow. These changes may cause conditions such as acquired aplastic anemia.
  • Infection: A condition such as chronic anemia may be caused by infection in the body. Once the infection is properly treated, the anemia will usually clear.

Diagnosing Blood Disorders

A number of healthcare practitioners, including a hematologist who specializes in the study of blood and blood diseases, can diagnose and treat blood disorders. A healthcare team should work together to properly address any conditions, complications and manage treatment plans.

A practitioner will conduct a physical exam and may order a blood test called a complete blood cell count (CBC) to check for changes in blood cell production and any corresponding disorders. A CBC can detect the numbers of red blood cells, white blood cells, hemoglobin, and platelets that are present. This lab test can also be used to determine the proportion of red blood cells to plasma in the blood, or hematocrit.

Depending on the severity of the blood disorder, a healthcare practitioner may also order the following lab work:

  • bone marrow biopsies;
  • tests that look for connective tissue disorders as the underlying cause;
  • tests that check for organ damage;
  • tests that can rule out hemolysis or the rapid destruction of red blood cells in the spleen or other parts of the body;
  • scans such as MRI, CT, EKG, SPECT or PET.
  • angiogram;
  • X-ray; and
  • urine tests.

 

Common Blood Disorders of Lupus

While this list is not inclusive, these three are the most common types of blood disorders that may become risks for individuals with lupus.

Anemia:  Approximately half of individuals with lupus may become anemic. An individual may become anemic when they do not have enough red blood cells to adequately carry oxygen throughout the blood to other tissues and organs.  There are several types of anemia including:

  • Iron deficiency anemia is the most common type of anemia and occurs when there is not enough iron in the body to make the hemoglobulin needed for red blood cells.
  • Vitamin deficiency anemia is usually caused from folate and vitamin B12 deficiencies, two nutrients needed for red blood cell production.
  • Anemia of chronic disease or anemia of inflammation can affect individuals with lupus, rheumatoid arthritis (RA), kidney disease and/or Crohn’s disease.
  • Aplastic anemia is rare, but life-threatening and is the result of the body being unable to produce enough red blood cells due to conditions like infections and autoimmune disease.
  • Bone marrow disease anemia often accompanies leukemia and myelofibrosis.
  • Hemolytic anemia happens when red blood cells are destroyed so quickly that the body cannot keep up its production of the cells.
  • Thalassemia anemia:An inherited disorder, thalassemia occurs when the body does not make enough hemoglobin, causing red blood cells to become oxygen-deficient.

Sickle cell anemia is an inherited disease that runs in families and predominately affects individuals of African or Hispanic descent. In sickle cell anemia, the red blood cells become sickle-shaped which can dramatically impede blood flow throughout the body. It can be extremely painful and cause severe fatigue, jaundice, delayed growth in children and can harm many tissues of the body. Blood and bone marrow transplants may cure some of the disease, and there are some drug treatments that can improve quality of life, especially for those unable to receive a transplant.

Symptoms of anemia can range from mild to severe and include:

  • fatigue;
  • paleness;
  • irregular heartbeat;
  • chest pains;
  • headaches;
  • shortness of breath;
  • dizziness; and
  • cold hands and feet.

Treatments for anemia may include:

  • oral iron tablets;
  • vitamin B12 injections;
  • oral folic acid;
  • immunosuppressants like prednisone;
  • cyclophosphamide;
  • cyclosporine; and
  • intravenous immunoglobin (IVIG).

Sickle cell anemia is much harder to treat and may require blood transfusions, hydroxyurea and pain-relieving narcotics.

 

Thrombosis:  Blood is made mostly of water. In a healthy, uninjured individual, blood easily flows throughout the body. When a person becomes injured or requires surgery, blood becomes thicker as it clots in order to stop excessive bleeding. Individuals with SLE are at greater risk of developing blood clots (thrombosis), which if dislodged and travel to other parts of the body are called emboli. Thrombosis is often associated with the production of antiphospholipid antibodies (APA) or antiphospholipid syndrome (APS).

Types of thrombosis include:

  • pulmonary embolus which is a blood clot that ends up in the lungs;
  • deep vein thrombosis which is a blood clot that occurs in the legs or arms; and
  • stroke which is when a blood clot travels to the brain.

Symptoms of thrombosis may include:

  • severe headache;
  • throbbing in the arm or leg;
  • swelling in one leg, rarely both;
  • swollen, sore veins; and
  • red, warm skin and localized pain.

It is imperative that if any individual experiences any of the above symptoms that they seek medical help immediately.

Thrombosis treatments may include anticoagulants such as heparin and warfarin. Individuals who sit a lot at home, work, school or on plane flights are also asked to move about at intervals in order to prevent blood clots from forming.

 

Vasculitis:  Vasculitis is an autoimmune lupus overlap condition and occurs when the lining of the blood vessels become inflamed. There are several types of vasculitis depending on which vessels of the body are affected. This inflammation can damage the walls of blood vessels and change blood flow.

General symptoms of vasculitis include:

  • fever;
  • loss of appetite;
  • inexplicable weight loss;
  • fatigue; and
  • aches and pains.

Symptoms are also dependent on where the vasculitis is located and may include the following:

  • stomach pain;
  • mouth sores;
  • blurred vision;
  • brain fog;
  • protein in urine;
  • persistent sinus infections and congestion; and
  • skin lesions or discoloration.

Treating vasculitis may include:

  • corticosteroids;
  • anti-rheumatics/immunosuppressants; and
  • over-the-counter pain and/or anti-inflammatory medications such as acetaminophen and ibuprofen.

Less-Common Blood Disorders in Individuals with Lupus

Though much less common, the following blood disorders have also been reported in individuals with SLE:

  • Leucopenia: A drop in white blood cell count that often has seemingly no origin and may be a side effect of medication.
  • Thrombocytopenia: A drop in platelets caused by the production of antibodies that attack and destroy them.
  • Neutropenia: A drastic drop in white blood cells that may leave an individual vulnerable to infection;
  • Acute lupus hemophagocyte syndrome: Primarily found in people from Asian descent, this is a rare blood complication of SLE that is characterized by fever and a sudden drop in blood cells and platelets.
  • Lymphomas: Individuals with SLE have a greater risk for developing lymphatic system cancers such as Hodgkin’s disease and non-Hodgkin’s lymphoma (NHL).
  • Pancytopenia: Bone marrow failure.
  • Leukocytosis: A significant increase in white blood cell count due to steroid use, surgery, stress or infection.

Treatments for these conditions may include:

  • corticosteroids;
  • immunosuppressants;
  • rituximab;
  • belimumab;
  • hydroxychloroquine (Plaquenil);
  • mycophenolate mofetil; and
  • Intravenous immunoglobulin (IVIG)

 

In Conclusion

Though blood disorders are common and some can be very serious, most are treatable when caught early.  This makes it imperative for early diagnosis and treatment. Individuals with lupus should be proactive and seek the advice from a healthcare practitioner if any new symptoms develop. Playing an active role in one’s own healthcare management does not only support physical wellbeing, but may boost confidence and a foster a sense of safety, security and control as well.

 

References

 

Anemia. (2019). Mayo Clinic. Retrieved July 12, 2020 from https://www.mayoclinic.org/diseases-conditions/anemia/symptoms-causes/syc-20351360

Aplastic anemia. (2019). Mayo Clinic. Retrieved July 12, 2020 from https://www.mayoclinic.org/diseases-conditions/aplastic-anemia/symptoms-causes/syc-20355015

Blood basics. (2020). American Society of Hematology. Retrieved July 12, 2020 from https://www.hematology.org/education/patients/blood-basics#:~:text=Red%20Blood%20Cells%20(also%20called,45%20percent%20of%20its%20volume.

Complete blood count (CBC). (2019). Mayo Clinic. Retrieved July 12, 2020 from https://www.mayoclinic.org/tests-procedures/complete-blood-count/about/pac-20384919#:~:text=A%20complete%20blood%20count%20(CBC,blood%20cells%2C%20which%20carry%20oxygen

DVT (deep vein thrombosis). (2019). National Health Service. Retrieved July 17, 2020 from https://www.nhs.uk/conditions/deep-vein-thrombosis-dvt/

Fozya, B. (2013). Hematological disorders in patients with systemic lupus erythematosus. The Open Rheumatology Journal, 87-95. doi: 10.2174/187431290130701087. Retrieved July 12, 2020 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3816272/

Giannouli, S., Voulgarelis, M., Ziakas, P., & Tzioufas, A. (2006). Anaemia in systemic lupus erythematosus: From pathophysiology to clinical assessment. Annals of the Rheumatic Diseases, 65(2), 144-148. Doi: 10.1136/ard.2005/041673. Retrieved July 17, 2020 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1798007/

Hemolytic anemia. (2020). Johns Hopkins Medicine. Retrieved July 12, 2020 from https://www.hopkinsmedicine.org/health/conditions-and-diseases/hemolytic-anemia#:~:text=Hemolytic%20anemia%20is%20a%20disorder,blood%20cells%2C%20you%20have%20anemia.

Human body facts. (2020). Science Kids. Retrieved July 12, 2020 from sciencekids.co.nz/sciencefacts/humanbody/blood.html

Iron deficiency anemia. (2019). Mayo Clinic. Retrieved July 12, 2020 from   https://www.mayoclinic.org/diseases-conditions/iron-deficiency-anemia/symptoms-causes/syc-20355034

Lupus and blood disorders. (2014). Lupus UK. Retrieved July 12, 2020 from https://www.lupusuk.org.uk/wp-content/uploads/2015/10/8.-Blood-Disorders.pdf

Sickle cell disease. (2020). National Heart, Lung, and Blood Institute. Retrieved July 17, 2020 from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease

Systemic lupus erythematosus. (2014). Cleveland Clinic. Retrieved July 12, 2020 from my.clevelandclinic.org/health/diseases_conditions/hic_Systemic_Lupus_Erythematosus

Thalassemia. (2019). Mayo Clinic. Retrieved July 12, 2020 from https://www.mayoclinic.org/diseases-conditions/thalassemia/symptoms-causes/syc-20354995

Vitamin deficiency anemia. (2019). Mayo Clinic. Retrieved July 12, 2020 from   https://www.mayoclinic.org/diseases-conditions/vitamin-deficiency-anemia/symptoms-causes/syc-20355025

What are white blood cells? (2020). University of Rochester Medical Center. Retrieved July 12, 2020 from https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=160&ContentID=35#:~:text=White%20blood%20cells%20are%20also,cells%20as%20your%20immunity%20cells.

 

Author: Liz Heintz

Liz Heintz is a medical research writer who received her BA in Communications, Advocacy, and Relational Communications from Marylhurst University in Lake Oswego, Oregon. She most recently worked for several years in the healthcare industry. A native of San Francisco, California, Liz now calls the beautiful Pacific Northwest home.

All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at [email protected] for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus.

All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.

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