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MPN Awareness Day
Sep 12th, 2019  

At AOP Orphan, we have been contributing to research on myeloproliferative neoplasms (MPNs) for many years.  On September 12th – the 7th Annual MPN Awareness Day – we especially want to help raising awareness of MPNs, since we believe that informing, connecting and advocating for those who are affected and involved is crucial for the well-being of patients. We will extend these efforts over the following months and bring into focus relevant information, advice and insights on all our communication platforms.

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What are MPNs? 

  • MPNs stands for myeloproliferative neoplasms
  • MPNs are a group of rare disorders of the bone marrow
  • MPNs are blood cancers that occur when the body produces too many red or/and white blood cells, or/and platelets.

     
Prof. Dr. Martin Griesshammer Director, Department of Hematology, Oncology, Hemostaseology and Palliative Medicine, Mühlenkreis clinics

“MPNs is a chronic haematological disease group caused by a stem-cell disorder. William Dameshek, an US haematologist, described this group of diseases for the first time approximately sixty years ago. He was looking at common symptoms and phenomena e.g. thromboses, constitutional symptoms, but also the unifying hyperproliferation of the bone marrow combined with splenomegaly and an increased number of thrombosis and bleeding events. 2016 WHO has renewed the definition of MPNs."

Prof. Dr. Martin Griesshammer

MPNs World Health Organisation Classification

The 2016 revision to the World Health Organization (WHO) classification of myeloid neoplasms includes the following myeloproliferative neoplasms (MPNs): 

  • chronic myeloid leukemia (CML), BCR-ABL1+
  •  chronic neutrophilic leukemia (CNL)
  • polycythaemia vera (PV)
  • primary myelofibrosis (PMF)
  • essential thrombocythemia (ET)
  • chronic eosinophilic leukemia not otherwise specified (NOS)
  • MPN, unclassifiable.

Source: The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Daniel A. Arber, Attilio Orazi, Robert Hasserjian, Jürgen Thiele, Michael J. Borowitz, Michelle M. Le Beau, Clara D. Bloomfield, Mario Cazzola and James W. Vardiman. Blood 2016 127:2391-2405.

Dr. Jean Jacques Kiladjian Professor of Clinical Hematology, Hospital Saint-Louis

"Under the umbrella of myeloproliferative neoplasms (MPNs) there are different diseases that belong to the family of malignancies. MPNs are completely different from solid cancers since MPNs are chronic diseases mostly. MPNs develop in the bone marrow of the patients and produce too many blood cells. According to the category of blood cells that are predominant, we can classify these diseases in different categories.

One is polycythaemia vera, there is mostly an increase of red blood cells but sometimes also along with too many white cells and platelets.

Then, if only platelets are increased, it’s usually essential thrombocythemia.

And you have the third one which is primary myelofibrosis that is a kind of a more heterogeneous disease group of patients with different aspects.

In general it’s always an overproduction of blood cells. “ 

What is PV and ET?

Click on the arrow icon below to find out more about Polycythaemia Vera and Essential Thrombocythemia, its causes, symptoms, and diagnosis. 

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Essential Thrombocythemia

Essential thrombocythemia (ET) is a disorder of the bone marrow characterized by an overproduction of platelets (thrombocytes). This overproduction of platelets can lead to an increased risk of blood clots (thrombus) and bleeding (hemorrhage). However, with a successful treatment, most patients with ET have a near normal life expectancy.

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Polycythaemia Vera

Polycythaemia Vera (PV) is a Rare Disease of the blood-building cells in the bone marrow primarily resulting in a chronic increase of red blood cells (erythrocytes), thereby decreasing the flowability of blood. Circulatory disorders such as thrombosis and embolism are possible consequences. With proper treatment, the majority of patients with Polycythaemia Vera have a near-normal life expectancy.