Myeloproliferative neoplasm

The term myeloproliferative disorder or myeloproliferative disorder (often " MPS " abbreviated, Greek μυєλός, myelos "Mark ", meaning the bone marrow and proliferation is Latin for proliferation ( cell ) ) was coined in 1951 by the American hematologist William Dameshek and refers to a group of blood diseases. In the English-speaking language area abbreviated MPD for myeloproliferative diseases or chronic myeloproliferative diseases for CMPD.

Historical coinage

Dameshek summarized under this term 5 hematological diseases together:

  • Polycythemia vera ( rubra) (PV )
  • Essential thrombocythemia (ET )
  • Osteomyelofibrosis / Osteomyelosclerosis (OMF or OMS)
  • Chronic myeloid leukemia ( CML)
  • The acute Di Guglielmo syndrome ( erythremia )

In today's parlance, only the first four diseases under this term are summarized. The Di Guglielmo syndrome is, however, classified under the acute myeloid leukemia (AML ) (AML FAB M6).

All four diseases have in common that they are based on a malignancy of blood-forming cells of the myeloid series. The boundaries of the above-mentioned diseases from each other is often not easy, because the clinical manifestations often overlap. Occasionally, it can not be clearly assigned to one of the four diseases mentioned a myeloproliferative disorder, then one speaks simply of " MPS ". It comes in all cases to an increased proliferation of myeloid cells. The leukocyte count in the blood is increased. In PV the red blood formation ( " erythropoiesis " ) is primarily increased in the ET platelet formation ( " thrombopoiesis " ) with the other diseases more the white myeloid hematopoiesis ( " engraftment "); in the OMF also there is a pronounced connective tissue remodeling (fibrosis) of the bone marrow. It is typical except for the blood changes splenomegaly (enlargement of the spleen) and often hepatomegaly ( enlarged liver ). All MPS bear the risk of transitioning into acute myeloid leukemia (or myeloid " blast crisis " ) in itself.

Classification by the WHO classification of 2001 and 2008

The WHO classification of haematological malignancies in 2001 under the term of chronic myeloproliferative diseases ( CMPD = chronic myeloproliferative diseases ) the following diseases together:

  • Polycythemia vera ( rubra) (PV )
  • Essential thrombocythemia (ET )
  • Idiopathic myelofibrosis ( osteomyelofibrosis / Osteomyelosclerosis ) (IMF, OMF or OMS)
  • Chronic myeloid leukemia ( CML)
  • Chronic Neutrophilenleukämie ( CNL )
  • Chronic eosinophilic leukemia / hypereosinophilic syndrome (CEL / HES)
  • Unclassifiable chronic myeloproliferative disorders

In the WHO classification of 2008, the concept of CMPD has been replaced by the concept of myeloproliferative neoplasms.

World Health Organization
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