Blood Disorders and Lupus
“You cannot talk about lupus and not talk about blood. The two are intricately entwined.” - Don Thomas, Jr. M.D. The Lupus Encyclopedia, p. 147
Let’s call a spade a spade. If you would like to blame one system in the body as the instigator of developing lupus, it would have to be the blood. That is where it all starts, and for many living with the disease, that is where many issues resound. In fact, abnormalities in the blood cells are some of the most common problems seen in people with systemic lupus.
You may be wondering why? Why is there (as the old hymn state) such “power in the blood?” As you may be aware, lupus is a chronic inflammatory disease that is caused by the immune system (your body’s natural defense system of white blood cells) attacking your own cells by mistake. Those overactive white cells traveling through your blood and lymph systems can be a catalyst for inflammation and issues. Additionally, though there is no single blood test to determine a lupus diagnosis, the blood is very important in the process of steering your diagnosis. It can help your doctor determine disease manifestations, and through analysis, support potential treatment.
Around 85% of lupus patients are believed to experience issues associated with the blood, presenting in antibody abnormalities, clotting problems, low red and white counts, and swelling in the blood vessels. In this blog, we will explain the most common ways in which it affects the blood and how hematologists (specialists in blood disorders) can work with your rheumatologist to evaluate and treat lupus blood disorders.
The blood makes up about 7-8% of the human body's weight. Blood contains three main components: red blood cells, white blood cells, and platelets. Blood cells are primarily produced in the bone marrow, where new blood cells are being created to replace the old cells that
have run their course and are being removed by organs such as the spleen. Red blood cells, or “erythrocytes” have the heavy responsibility of carrying oxygen around the body. They also contain an iron-rich protein called hemoglobin that carries oxygen from the lungs to the rest of the tissues in the body.
Hemoglobin is also responsible for giving blood the wonderful "red" hue when oxygen binds to it. When the oxygen is used up by the tissues, the hemoglobin that now lacks oxygen binding gives off a bluish-purple hue. That is why your veins, just underneath the skin, appear bluish in color. Unoxygenated hemoglobin also causes the bluish-purple color of the fingertips and toes that can occur in many patients with Raynaud's phenomenon during the cold or stressful moments.
White blood cells, or “leukocytes” are an essential asset to the body's immune defense. They are the ground soldiers that fight against cancer cells, viruses, bacteria and other unwanted infectious diseases and foreign materials. And lastly, platelets are your internal "BAND-AIDS®" that help blood clot when your skin is cut. Plasma is another blood component containing electrolytes, glucose, hormones and various nutrients.
Anemia is one of the most common blood disorders in lupus patients. Anemia affects up to half of all people living with lupus at one point or another. Anemia is defined as having too little hemoglobin in the blood. There are many reasons that someone with SLE may develop it, and it is very important to figure out why someone is anemic in order to treat it appropriately. There are several types of anemia, the two most common to be found in SLE
patients are iron deficiency anemia and anemia of chronic disease. However, vitamin deficiency anemia, aplastic anemia, bone marrow disease anemia, hemolytic anemia, sickle cell anemia, and thalassemia anemia are other variations of the disease as well. Most SLE patients with anemia have mild anemia (hemoglobin of 10 or higher) and do not feel bad at all. If you are suffering from anemia, unusual fatigue and weakness are two of the first symptoms you may experience. Other symptoms include paleness, irregular heartbeat, chest pains, dizziness and cold hands and feet. It can be temporary or long-term and ranges from mild to severe. Treatments vary from taking supplements for mild cases to more invasive medical procedures (like a blood transfusion or bone marrow transplant) for severe cases. Iron deficiency anemia can be helped by sticking to a healthy, iron-rich diet. If you have an inherited anemia like sickle cell or thalassemia, talk to your doctor about any future risks.
30-50% of people living with SLE will develop antiphospholipid antibodies (aPLA's). The presence of these antibodies in the blood can potentially cause an increase in blood clots. In fact, 50% of people with aPLAs develop increased clotting issues over time. These issues can lead to deep venous thrombosis (DVT), blood clots in the lungs (pulmonary embolism, PE), blood clots in the arteries of the brain (strokes), heart attacks, and increased pregnancy risk complications such as miscarriage. When someone has aPLAs along with blood clots or pregnancy complications, it is called antiphospholipid syndrome (APS).
Just to be clear, blood clotting is not always a bad thing. The body's blood is usually in a liquid state, and when a person experiences an injury or has surgery, blood thickens and clots (through a process called hemostasis) and stops the spot that is bleeding. However, with lupus, sometimes the hemostasis process is too active and a blood clot forms where it shouldn't be.
This results in a thrombus (fancy word for blood clot). Sometimes the clot detaches and travels somewhere else in the body (called an embolus). Traveling blood clots like this can be dangerous. Blood clots in the veins can travel to the lungs (pulmonary embolism), legs (DVT), arms, and brain (stroke). As mentioned above, thrombosis in lupus is often (but not always) associated with the presence of antiphospholipid antibodies. Several different blood tests are used to detect antiphospholipids: the anticardiolipin test, the lupus anticoagulant test, beta-2 glycoprotein-1 antibody, false positive syphilis tests, and anti-phosphatidylserine antibodies (the role of the latter two with blood clot formation is not well define). Before starting a family, it is so important to have the initial three done to determine if APS antibodies are present and if you are at an increased risk for developing lupus thrombosis and pregnancy complications.
Unfortunately, women can be unaware they have this issue and may experience pregnancy loss because of it. However, with the right tests and information better outcomes are definitely possible. Low dose aspirin every day can help prevent blood clots from forming and women with lupus and APS can and do have successful pregnancies.
Lupus patients who experience blood clots may also need to be on blood thinners such as warfarin or heparin for the rest of their lives to prevent them from forming.
Vasculitis can occur in any body organ but most commonly affects the skin in lupus patients. It is defined by as inflammation and damage to the walls of the blood vessels. It can disrupt the blood flow in the arteries, veins, and capillaries. Vasculitis can be mild or disabling and, in severe cases, fatal. Vasculitis can occur in lupus when the immune system mistakenly attacks the blood vessels. Lupus is the cause of the vasculitis in most causes. However, rarely, someone can have an overlap syndrome with a lupus-unrelated vasculitits, such as giant cell arteritis, polyangiitis with granulomatosis, Henoch Schönlein purpura, or polyarteritis nodosa. It can also occur due to an infection, reaction to a medication, or another disease or condition (like Sjögren's Disease).
The symptoms of vasculitis can vary depending on where the inflammation is. Doctors usually diagnose vasculitis by doing a complete blood workup and sometimes a biopsy or angiography. Treatment is directed toward decreasing inflammation of the blood vessels and may include glucocorticoids (prednisone or others) or immunosuppressants.
In thrombocytopenia, antibodies attack and destroy blood platelets. This can happen in 10-25% of people with SLE (and can also occur in those with APS), causing platelets to decrease in number. Most patients have mildly low platelet counts and no problem at all. The symptoms include bruising and bleeding from the skin, gums, nose, or intestines. What is interesting is that some people actually will develop thrombocytopenia before developing SLE. When this happens, it is called immune thrombocytopenic purpura (ITP), formerly called idiopathic thrombocytopenic purpura. 10% of people with ITP will develop SLE during their lives. Treatment for thrombocytopenia includes high doses of steroids, immunosuppressants, Rituxan, Benlysta, and IVIG are sometimes used as well.
Leukopenia and Neutropenia:
The most important types of white blood cells in SLE are neutrophils and lymphocytes. Neutrophils help fight off bacterial infections, while lymphocytes play numerous roles, including fighting off viruses and overseeing overall immune function. A drop in the number of neutrophil white blood cells is called neutropenia, whereas a decline in the number of lymphocytes is called lymphopenia. When the complete white blood cell count is low, it is called leukopenia. Patients with SLE often experience neutropenia, but the condition is usually harmless unless the reductions are so drastic that the individual becomes vulnerable to infections. Viral infections, certain medications, cancer, other autoimmune disorders or congenital disorders, or lupus itself can cause this drop. Lupus medications, including azathioprine and cyclophosphamide, can lower cell counts as well, so extra lab tests may be requested to monitor during treatment with these agents. If counts go too low, the prescribed drug is usually stopped briefly, or the dosage is reduced. Usually, there is no additional treatment for low white cell counts. Infections in lupus are more often related to alterations in the body's immune system that are not reflected in routine blood counts.
Acute Lupus Hemophagocytic Syndrome:
Primarily found in those with Asian backgrounds, this is a rare SLE blood complication characterized by fever and a sudden drop in blood cells and platelets. It is generally of short duration and is treated with high-dose corticosteroids, cyclosporine, and high-dose intravenous immunoglobulin.
Lymphoma is a group of cancers that affect the cells of the immune system. SLE patients and people with other autoimmune disorders have a greater risk of developing lymph system cancers such as Hodgkin’s disease and non-Hodgkin’s lymphoma (NHL). The risk is also higher in those with primary Sjögren’s disease (formerly called primary Sjögren's) and also in those with rheumatoid arthritis compared to the general population.
Blood involvement with lupus can occur with or without other symptoms. It is important that lupus patients have their blood checked regularly to detect any problems.
As a lupus patient of 32+ years, I have experienced my fair share of blood complications. It can be a lot, from thrombocytopenia as a child to anemia in my adult years, to constant monitoring of my low white cells. But, my blood is a part of me, and though it may be an instigator and, at times, a troublemaker, it has taught me what to look for and to stay vigilant of any new symptoms.
So at least I know my grief hasn’t been in “vein.” (pun intended)