A common question that I see asked by newly diagnosed systemic mastocytosis (SM) patients is, “Is systemic mastocytosisMastocytosis Rare disease caused by the buildup of mast cells. Cutaneous mastocytosis primarily affects the skin and is more common in children, while systemic mastocytosis affects internal organs and is more common in adults. a cancer?”
The answer should be cut and dry: All subvariants of systemic mastocytosis, from the most common indolent (slow progressing) to mast cell leukemiaMast cell leukemia The most severe subtype of SM, caused by the rapid buildup of immature mast cells in the bone marrow and blood. Mast cell leukemia accounts for less than 5% of SM cases and has a poor prognosis. (poor prognosis), are defined as a blood cancer with varying severities. It is worth mentioning that the World Health Organization classifies all forms of systemic mastocytosis as a blood cancer (known as a myeloproliferative neoplasmMyeloproliferative neoplasm SM used to be classified as a type of myeloproliferative neoplasm, a group of rare blood cancers characterized by an overaccumulation of blood cells in the bone marrow.) driven by a mutation of the KIT gene.
But when this question is asked, there is a palpable tension in how the SM community answers it that I found worth investigating. The most common misconception that I have noticed is that many patients think mast cell leukemia is the only subvariant of SM that is considered a cancer. Another common misconception is that the classification of cancer varies depending on which country you are in.
Read more about SM’s cancer classification: “Why is SM considered a type of cancer?”
What is the cause of this divide amongst patients?
Due to its rarity, systemic mastocytosis is commonly unrecognized and highly misunderstood by both the general population and medical providers alike. The root cause of this cancer classification confusion boils down to misinformation, a common challenge that comes with a rare disease.
Realizing the consequences of this misinformation inspired me to ignite a dialogue amongst patients to help them recognize where they are being steered off the road. I discovered that there are many reasons why labelling SM as a cancer is far from simple, and some of those reasons are emotionally driven.
Sign up here to get the latest news, perspectives, and information about SM sent directly to your inbox. Registration is free and only takes a minute.
Doctors themselves may not know
Misunderstanding is often followed by misinformation, especially when the misunderstanding comes from a highly regarded medical professional. It is understandable that the facts become blurry when you trust that your doctor knows best. While they may be brilliant, not every doctor knows everything. And many may not explain that SM is a cancer.
Over time, many SM patients come to realize that there is a dangerous lack of knowledge of this disease due to its rarity; this is why I stress the importance of knowledge and advocacy when living with SM.
You are allowed to challenge a doctor’s knowledge, especially when it affects your access to resources. Whatever the doctor’s reason, whether it be protecting their patient’s mental health or simply not being up to date on information, they should not be denying what SM is.
The word benign
While it can be incredibly debilitating, indolent systemic mastocytosis (ISM) is defined as clinically benign, meaning that it presents without organ dysfunction and has a normal to near-normal life expectancy. As an ISM patient who experiences debilitating symptoms and has had a brush with death from this disease, I find the word “benign” a huge understatement. It wasn’t until I gained an understanding of ISM’s clinical classification a cancer that I found the word “benign” to be less nonsensical. Using the word benign to describe ISM can only seem appropriate when viewed in terms of how the disease affects life expectancy — and even so, that comes with the caveat of life-threatening anaphylactic reactions. The way that I can wrap my head around ISM being labelled benign is to view it as a severe disease with a likely benign progression.
In terms of cancer progression, SM is clinically benign unless it has progressed to a sub variant that does cause organ dysfunction, and potentially shortens life expectancy. I believe that ISM’s clinically benign prognosis is what drives the misconception of what systemic mastocytosis technically is — after all, ISM is the most common nonadvanced subtype, and it’s what most patients are familiar with.
The personal component of denial
Caring for palliative patients as a nurse and losing loved ones to cancer, I can relate to those who have witnessed and have been a part of a person’s harsh battle with an aggressive cancer. While there are few shared experiences between these cancers and SM — such as medical trauma and unpredictability — I would never compare someone else’s cancer diagnosis with my SM diagnosis.
Considered a “chronic” blood cancer, nonadvanced SMNonadvanced SM In these subtypes of SM, mast cells accumulate in the body but do not usually cause severe organ damage. Nonadvanced SM includes the subtypes indolent SM and smoldering SM. doesn’t fit the typical idea of cancer. I think that’s part of why many patients with SM reject the “cancer” label.
I can completely understand the emotional component behind denying its classification as a cancer. Those who have witnessed and experienced the horrors of an aggressive cancer know it’s a severe battle.
It may seem unfair, but severity is not what determines if a disease is cancer or not. Typically, cancer is a disease that you either conquer or lose the battle to; nonadvanced SM is a disease that you submit to. Living with SM more resembles living with a chronic illness than living with a typical cancer, furthering its distinction. I will add that the newer tyrosine kinase inhibitors, although not a cure, are leaning more towards “fighting” SM clinically rather than only blunting symptoms.
Why does the cancer classification matter?
In my opinion, the classification of what SM is gives us a home, a recognized place. Most of the world doesn’t know what a mast cellMast cell A type of white blood cell produced in the bone marrow. They help defend against infections and play a key role in allergic reactions. In SM, mast cells become overactive and build up throughout the body. is; it makes me feel like an alien that nobody knows what to do with. The classification of cancer makes resources available and the challenges that we face recognizable. I want to be able to know and say what my disease is, because it affects my life every single day. If SM was its own disease with no recognized place to put it, we would likely be left out to sea: there would be less progress in treatment and research.
I admit that in most situations, I do not go around telling people that I have cancer. It is too complicated to explain how it’s not what they know about cancer. I find that explaining the definition to people leads to minimizing my disease and disbelief about its burden, so I stay mysterious in those insignificant situations.
I believe that for systemic mastocytosis to become recognized, the information of this complicated diagnosis needs be streamlined. That will require the union and advocacy of its patients. Then maybe there will be a day that SM patients can say “I have systemic mastocytosis” and have their plight be recognized.
