Autoimmune Disorders that May Coexist with Myeloproliferative Neoplasms (MPNs)

by David Wallace

As some MPN patients are aware of firsthand, there are a number of autoimmune diseases that may run concurrently with the condition – while these disorders are often exacerbated by the weakened immune system of MPN patients, inflammation also plays a key role.

However, research has linked MPNs to a predisposition to autoimmune disorders.  In this article, we’ll take a look at some of the autoimmune conditions known to coexist with MPN, as well as the role of inflammation in MPN.  We will also take a brief look at research findings in regard to autoimmune disorders predisposing a patient to the disease.

About Inflammation

More and more, inflammation is being linked to autoimmune diseases even in otherwise healthy individuals– with our modern diet of processed foods and GMO’s, as well as our propensity for high-stress lifestyles, inflammation is now the norm.

For patients battling MPNs, the inflammation can be even more of an issue.

Chronic Inflammation in MPNs

Inflammation is highly problematic in Myeloproliferative Neoplasms (MPNs) in that it not only increases pain/discomfort for patients in terms of symptoms, it also plays a direct role in proliferation of the neoplastic clone.

Chronic inflammation is a common characteristic of MPNs, and it is suspected that it may be the cause of many of the chronic symptoms of the illness.  There is a gene known as the JAK2 – a mutation called JAK2V617F, is known as a common hallmark of MPNs.

While inflammation is the body’s natural resistance to disease, progenitor cells and hematopoietic stem cells that are mutated by JAK2V617F are resistant to inflammation, which allows them to continue to proliferate unhindered by the body’s defenses.

In other words, inflammation can cause cancer cells to multiply at a faster rate, thereby causing the disease to progress.  Inflammation has also been linked to anemia and bone marrow fibrosis, or the replacement of bone marrow with scar tissue, an irreversible condition.

For those MPN patients who are predisposed to chronic inflammatory conditions, it’s a vicious cycle – the inflammation brought about by MPNs stimulates the proliferation of the neoplastic clone, while this proliferation then drives inflammation, which in turn aggravates autoimmune disorders.

However, there is research that indicates patients who are diagnosed with certain autoimmune disorders may be at higher risk for developing MPNs.

Common Autoimmune Conditions

While there are a number of autoimmune conditions, some that are known to coexist with MPNs are:

autoimmune diseases coexist with MPNs

Psoriasis:  this is a chronic inflammatory disorder of the skin where T-lymphocytes, or specialized white blood cells cause the production of skin cells to speed up to the point where the patient is covered with itchy, scaling patches.

Crohn’s Disease:  this inflammatory bowel disease can affect any part of the gastrointestinal tract, from the mouth to the anus – it is debilitating and often devastatingly painful.

Lupus:  a chronic autoimmune disease in which the body’s immune system becomes hyperactive and attacks normal, healthy tissue. This results in symptoms such as inflammation, swelling, and damage to joints, skin, kidneys, blood, the heart, and lungs.

Localized Scleroderma:  this connective tissue disorder is rare, and manifests in cutaneous sclerosis, which can cause disfigurement such as sores and skin hardening – joint issues can also occur.

Autoimmune Hemolytic anemia (AIHA):  this group of blood disorders attacks the immune system, causing the body to attack its own red blood cells as though they were foreign bodies. Red blood cells house hemoglobin, which help carry oxygen throughout the body, and which can then lead to an insufficient oxygen supply.

Polymyalgia Rheumatica:  this inflammatory disorder causes both stiffness and pain in the muscles and joints.

Giant cell arteritis:  this condition is characterized by inflammation in the lining of the arteries, primarily in the head- it particularly affects arteries in the temples.  The condition can be painful and can cause symptoms such as headache, double vision, and flu-like symptoms.

Reiter’s Syndrome:  a chronic type of arthritis that evolves due to either inflammation of the eyes, or conjunctivitis or inflammation in the urinary or gastrointestinal system.

Aplastic Anemia:  this condition occurs when the body ceases making new blood cells of all three types– red, white and platelets. Also known as blood marrow failure, it causes there to be an inadequate amount of oxygen in the bloodstream.

Research into Comorbidity

There has been some investigation in the scientific community as far as the comorbidity of MPN and related autoimmune disorders.

A study by the Swedish Cancer Registry looked at a wide range of autoimmune disorders specifically in relation to myeloproliferative neoplasms – it was found that patients with a previous history of autoimmune dysfunction were 20% more likely to develop MPN Further evaluation indicated that patients who had been previously diagnosed with immune thrombocytopenic purpura, polymyalgia rheumatica or Crohn’s Disease were at 2 to 3 times the risk for MPN.  Research has also found that the most significant risk of developing MPN occurs in patients who have had a previous diagnosis of Reiter’s Syndrome, giant-cell arteritis, or aplastic anemia.

While the research is not yet conclusive, it appears as though autoimmune patients, including those with specific autoimmune conditions, are at a much higher risk of developing MPN, which brings us one step closer to understanding the source of the condition.

This article was reviewed by a MPN Specialist for accuracy.

References:

Swedish Cancer Registry Research –  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4619974/

Inflammation and MPN – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4641200/

Aplastic Anemia – https://www.mayoclinic.org/diseases-conditions/aplastic-anemia/symptoms-causes/syc-20355015

Psoriasis – https://www.mayoclinic.org/diseases-conditions/psoriasis/symptoms-causes/syc-20355840

Reiter’s Syndrome – https://www.ncbi.nlm.nih.gov/pubmed/18436339

Giant-Cell Arteritis – https://en.wikipedia.org/wiki/Giant-cell_arteritis

Autoimmune Hemolytic Anemia – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4326213/

Localized Scleroderma – https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Localized-Scleroderma-Juvenile

Lupus – https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4857264/ 

Sponsored by Incyte Corporation


 

About David Wallace

Founder of PV Reporter, a resource for Myeloproliferative Neoplasm (MPN) patients and caregivers. After being diagnosed with Polycythemia Vera (PV) in 2009, I utilized social media to connect with "informed patients" and develop a better understanding of emerging treatment options. My philosophy on patient care is straight forward - "educating the patient is essential, so the patient can guide their physician to meet his or her needs." PV Reporter is a comprehensive resource hub giving visitors vital tools to become "empowered patients."

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