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Myeloproliferative Neoplasms

Justin T Ertle, MD and James Evan Davidson, MD Reviewed 06/2022
 


BASICS

DESCRIPTION

  • Myeloproliferative neoplasms (MPNs) are a group of clonal disorders that share a common cell of origin in the pluripotent hematopoietic stem cell.

  • This topic focuses on chronic myelog...

DIAGNOSIS

PHYSICAL EXAM

  • Constitutional symptoms are common, though a minority are asymptomatic.

  • All MPNs may share the following symptoms (3): 

    • Constitutional: fatigue, weakness, night swea...

TREATMENT

Management strategies include observation, phlebotomy/transfusion, medications, and stem cell transplantation. 

GENERAL MEASURES

  • PV:

    • Phlebotomy is first line for PV, with goal hema...

ONGOING CARE

PATIENT EDUCATION

  • Avoid situations increasing their risk of thrombosis, such as prolonged immobility.

  • With splenomegaly, patients may be advised to avoid high-contact sports and other activ...

REFERENCES

1
Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405.
2
Titm...

ADDITIONAL READING

Spivak JL. Myeloproliferative Neoplasms. N Engl J Med. 2017 Jun 1;376(22):2168-2181.  

SEE ALSO

Leukemia, Chronic Myelogenous; Polycythemia Vera 

CODES

ICD10

  • D47.1 Chronic myeloproliferative disease

  • C92.10 Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission

  • D45 Polycythemia vera

  • D47.3 Essential (hemorrhagic) thrombocythemia

  • C93...

CLINICAL PEARLS

  • MPNs result from mutations that activate hematopoiesis, resulting in proliferation of cells of myeloid, erythroid, and/or megakaryocyte lineages.

  • CML is characterized by granulocytosis,...

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