In keeping with Blood Cancer Uncensored’s overall goal of providing support for all blood cancer patients and support people, this is an attempt to pull multiple “user friendly” links together to give the basics of the MPNs which compose a select group of our members. This is because I have noticed a huge quest for information from our members and perhaps it is a bit time consuming to take it all in. All questions about any of the information you see here should be directed back to your doctor. This article is primarily provided to give a quick reference guide which includes descriptions, symptoms, diagnosis, and treatment options as of the date of publishing. Because the links to the research are posted here, hopefully this publication will grow with the knowledge and expanding treatment options and will stand the test of time. Please do NOT attempt to diagnose yourself by reading this article and it’s accompanying links. This research is meant to enhance what you already know and to put it in one resource. If you discover any better resources, please feel free to comment at the end of the paper (Discus) or use our email box bloodcanceruncensored@gmail.com .

“Chronic myeloproliferative disorders are a group of slow-growing blood cancers in which the bone marrow makes too many abnormal red blood cells, white blood cells, or platelets, which accumulate in the blood. The type of myeloproliferative disorder is based on whether too many red blood cells, white blood cells, or platelets are being made. Sometimes the body will make too many of more than one type of blood cell, but usually one type of blood cell is affected more than the others.^[1]

There are 6 types of chronic myeloproliferative disorders: chronic myelogenous leukemia (CML), polycythemia vera, primary myelofibrosis (also called chronic idiopathic myelofibrosis), essential thrombocythemia, chronic neutrophilic leukemia, and chronic eosinophilic leukemia. Chronic myeloproliferative disorders sometimes become acute leukemia, in which too many abnormal white blood cells are made.^[1] ” (source: https://rarediseases.info.nih.gov/diseases/9319/chronic-myeloproliferative-disorders AND https://www.cancersupportcommunity.org/myeloproliferative-neoplasms?msclkid=9dcf82b569491aea337de20edd880d8a#myelofibrosis)

Polycythemia vera (PV)

This condition occurs when your body–specifically your bone marrow–makes too many red blood cells. This causes to your blood to thicken, which makes you at risk for blood clots, amongst other life-threatening problems. The condition is know as PV, because the term “polycythemia” and “vera” are hard to pronounce! It is pol-e-sy-THEE-me-uh-VEER-uh). Please use the link highlighted below for information about symptoms, diagnosis, and treatments as to date. Check out the advice given on itching. That is a known side effect with many types of blood cancers and disorders (see this article for some interesting perspective on itching). A very detailed summary of PV is in the next link.

https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/diagnosis-treatment/drc-20355855 AND https://medlineplus.gov/ency/article/000589.htm

Outlook

Close monitoring is very important. You will have to have blood draws frequently to prevent clots and other complications. The long term outlook is life expectancy of about 20 years. Please see https://www.healthline.com/health/polycythemia-vera/prognosis-and-life-expectancy#outlook for more details.

Essential Thrombocythemia (ET)

This next blood cancer is called Essential Thrombocythemia (throm-bo-sy-THEE-me-uh). Like PV, it is rare and in this condition your body fails to produce enough platelets, which, in turn, can severely effect blood clotting. The key to this condition is to properly describe your symptoms to your doctor, as the symptoms can be subtle and mimic other conditions. It occurs more often women, and seems to target folks over 50 years old. Unfortunately, there is no cure at this time. In this condition, genetic testing is very important and is diagnosed by noting unusual changes in the JAK2 gene. Anomalies in this gene are noted in the other cancers in this group.

Please use the links below to explore more details about ET.

https://www.cancersupportcommunity.org/myeloproliferative-neoplasms?msclkid=9dcf82b569491aea337de20edd880d8a#essential-thrombocythemia-et AND https://medlineplus.gov/ency/article/000543.htm

Myelofibrosis (MF)

With Myelofibrosis, the body produces too many blood cells, and this overtasks your bone marrow. This then causes scar tissue in the bone marrow. There are two types: Primary Myelofibrosis (also known as chronic idiopathic myelofibrosis), which develops on its own, and Secondary Myelofibrosis, which can branch out from Polycythemia Vera or Essential Thrombocythemia. The scarring then prohibits the body from producing its blood cells normally. Of the MPNs, it is a fairly common blood cancer of which there are a variety of treatments, including a potential cure called allogenic stem cell transplantation. Genetic testing is one way of determining whether you have this condition or not. Please see the following link for more information on this condition.

https://www.cancersupportcommunity.org/myeloproliferative-neoplasms?msclkid=9dcf82b569491aea337de20edd880d8a#myelofibrosis AND https://www.cancersupportcommunity.org/article/myelofibrosis-treatment-and-side-effects AND https://medlineplus.gov/ency/article/000531.htm

Outlook

Median survival rate is 3.5-5.5 years and treatment options are narrow. The only known cure is allogeneic stem cell transplantation and the requirements for an individual to qualify for it are difficult and it has very risky side effects. Very few people meet the requirements. The only FDA approved drug for treatment currently is ruxolitinib (Jakafi), yet more studies are being conducted. There are other medications being used to control this condition and you can learn more about this here: https://www.epainassist.com/blood-diseases/what-is-the-life-expectancy-of-someone-with-myelofibrosis

Chronic Myeloid Leukemia (CML)

Chronic myeloid leukemia is a slow growing cancer in which the main problem is overproduction of white blood cells created in the bone marrow, resulting in high blood cell counts. This is the blood cancer which is identified by genetic testing for an abnormal chromosome called the “Philadelphia chromosome”. Check out the story behind the discovery of the Philadelphia Chromosome here: https://www.foxchase.org/about-us/history/discoveries-fox-chase-research/philadelphia-chromosome/philadelphia-chromosome. The interesting part of this chromosome is that you are not born with it! It is a genetic anomaly that occurs in the blood-forming stem cell in bone marrow where chromosomes 9 and 22 become fused and then becomes a new gene called BCR-ABL. CML is also known as chronic myelogenous leukemia, chronic myelocytic leukemia or chronic granulocytic leukemia.

There are three phases and proper diagnosis determines treatment. Please see this link for more details https://www.pennmedicine.org/cancer/types-of-cancer/leukemia/chronic-myeloid-leukemia/cml-treatment AND https://medlineplus.gov/ency/article/000570.htm

Outlook

The following gives insight to the variety of factors considered when treating this rare blood cancer. Examples include:

• age
• phase
• pre-existing health issues
• platelet counts
• bone damage from the illness
• spleen size

Life expectancy depends on what phase you are in and your age, but a statistic from Healthline.com states that 65% of folks diagnosed are still alive after 5 years. Source: https://www.healthline.com/health/chronic-myeloid-leukemia/outlook-life-expectancy#outlook

Chronic Neutrophilic Leukemia (CNL)

This is an extremely rare blood cancer. In CNL, neutrophils, a type of white blood cell, are overproduced and accumulate in the peripheral blood and the bone marrow. Signs, symptoms and complications of CNL result from the overproduction of neutrophils.

Diagnosis is based on complex genetic profiling since it is so rare, yet it does respond treatment-wise to the JAK inhibitors (source: https://onlinelibrary.wiley.com/doi/full/10.1002/ajh.25688).

Please see also https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq#section/_255

Outlook

This brochure explains it very well. https://www.lls.org/sites/default/files/National/USA/Pdf/Publications/FS30_CNL%20Fact%20Sheet_FINAL.pdf

Chronic Eosinophilic Leukemia, NOS (CEL)

Chronic eosinophilic leukemia (CEL) is a rare myeloproliferative neoplasm. It causes an over-production of eosinophils in the bone marrow. The eosinophils are the white blood cells that react to infections and allergic reactions. In this condition, the eosinophils over-produce and can pile up in bone marrow and tissues inside of the body. This condition is not diagnosed with genetic testing but is often diagnosed from routine bloodwork. This cancer is slow growing and may turn into acute myelogenous leukemia (AML). A stem cell transplant may be a treatment option for some people with chronic eosinophilic leukemia. Treatment includes interferon alfa, corticosteroids, and chemotherapy drugs (example: Hydrea, Cytoxan, Procytox, Oncovin).

Check out this link for more information:

https://www.cancer.ca/en/cancer-information/cancer-type/leukemia/leukemia/chronic-eosinophilic-leukemia/?region=on#:~:text=See%20your%20doctor%20if%20you%20have%20these%20symptoms%3A,throat%20or%20on%20the%20hands%20and%20feet.%20 AND https://www.cancer.gov/types/myeloproliferative/patient/chronic-treatment-pdq#section/_258

Outlook

Unfortunately, this condition is an aggressive blood cancer and the life expectancy is about 2 years. Please keep in contact with your specialist frequently for best results. See https://www.pathologyoutlines.com/topic/myeloproliferativeCEL.html and https://www.leukaemiacare.org.uk/support-and-information/information-about-blood-cancer/blood-cancer-information/leukaemia/chronic-eosinophilic-leukaemia/

Diagnosing Blood Cancer in General

First and foremost, if you are grappling with the concept that something is off with your body (abnormal fatigue, rashes, headaches, weight loss, blurry vision, for example), please make an appointment with your generalist/primary care physician immediately. Ask for blood testing to be done to search out anomalies. Then, if no obvious answers are provided, ask for a referral to a hematologist who is trained in what tests to run and will order more specific bloodwork/blood draw tests for you.

You will notice that for each kind of blood cancer, since the symptoms are so similar, that diagnosis usually starts with a referral to a hematologist or a a thoracic surgeon (for biopsy) following an examination by your general practitioner. You are filled with wonder and anxiety as they order tests like “flow cytometry”, bone marrow biopsies, and JAK2 testing. If you pay attention when your blood is drawn, they will take at least five vials. You feel like a lab rat, wondering what they will pinch, pull, or snip at next. But the science behind the genetic testing and the reasons for the biopsies and palpating of your lymph nodes are necessary for proper diagnosis. While you are being evaluated, we recommend that you try not to search “Dr. Google” too much, as this can be anxiety provoking and unclear. There is both good and bad information on the web!

When it is all said and done, there are so many different kinds of blood cancer; your doctor needs to be specific (see Adrian’s article on blood cancer varieties) so do not be surprised if it is taking a long time. It is nearly impossible to not ask yourself, “What did I do wrong?” when handed a blood cancer diagnosis. The short answer and the long is: YOU DID NOT DO ANYTHING WRONG. You and I live in an imperfect world with so much illness and the cards are stacked against most of us that we would escape this world without having some form of a chronic or acute illness. Of course we didn’t ask for it, but it has landed on us, so we can only join together and hold each others hands through it.

“The tests in this section can help work out whether someone has blood cancer.

They also help diagnose what type of blood cancer it is, such as leukaemia, lymphoma, myeloma or any other type.

Having these tests does not necessarily mean you have blood cancer. Many of these tests are used to diagnose a range of health conditions.

If you do have blood cancer, you may continue to have these tests before, during or after treatments, to see how you’re doing.

Having tests can be worrying. If you have any questions, or just need someone to talk to, contact our Support Services Team.” https://bloodcancer.org.uk/understanding-blood-cancer/tests-diagnosis/

Information about clinical trials is available from the NCI website. Also: ClinicalTrials.gov From the National Institutes of Health

Remember, you should never self-diagnose. If you are having symptoms, we recommend you discuss your symptoms with your GP/ Regular Medical Doctor and they will do a thorough examination and order blood testing. If you are diagnosed with one of these, we realize it is a rare category of blood cancer, but after having Blood Cancer Uncensored as a group for a over a year, our community is slowly growing. Get to know one another. Use the Facebook group to chat or connect on messenger. There is knowledge in numbers!

Specialists in the MPNs

https://patientpower.info/myeloproliferative-neoplasms/living-with-myeloloproliferative-neoplasms/finding-an-mpn-specialist-seeking-a-second-opinion AND https://www.mpnresearchfoundation.org/Find-a-Hematologist/ (folks in the United States) AND https://www.nice.org.uk/guidance/conditions-and-diseases/blood-and-immune-system-conditions/blood-and-bone-marrow-cancers AND https://www.cancerresearchuk.org/about-cancer/other-conditions/myeloproliferative-neoplasms (for folks in the United Kingdom)

Need Support? Check out these links too!

https://bloodcancer.org.uk/support-for-you/talk-blood-cancer/

https://copingmag.com/myeloproliferative-neoplasms/

https://www.cancercare.org/support_groups/tagged/myeloproliferative_neoplasms