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Myeloproliferative Neoplasms

Reviewed 06/2019
 


BASICS

DESCRIPTION

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

  • MPNs are characterized by proliferat...

DIAGNOSIS

HISTORY

  • Many MPN cases are found incidentally on routine blood work.

  • CML: 20–50% of patients are asymptomatic at time of diagnosis.

    • Fatigue, malaise, weight loss, excessive perspiration, abdomi...

TREATMENT

GENERAL MEASURES

Low-dose aspirin for ET and PV 

MEDICATION

First Line

  • CML: TKIs are mainstay of treatment.

    • Chronic phase: imatinib mesylate (Gleevec): 400 mg/day is the safest choice.

  • Other optio...

ONGOING CARE

PROGNOSIS

  • CML: 10-year survival rate ~80% from time of diagnosis; 85% will die in blast crisis; 12- to 18-month median survival for accelerated phase; 3- to 6-month median survival for bla...

REFERENCES

1
Fava C, Rege-Cambrin G, Saglio G. The choice of first-line chronic myelogenous leukemia treatment. Ann Hematol.  2015;94(Suppl 2):S123–S131. [View Abstract on OvidMedline]
2
Geyer HL, M...

ADDITIONAL READING

Patel AB, Vellore NA, Deininger MW. New strategies in myeloproliferative neoplasms: the evolving genetic and therapeutic landscape. Clin Cancer Res.  2016;22(5):1037–1047. [Vie...

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 are a group of clonal disorders that share a common cell of origin in the pluripotent hematopoietic stem cell.

  • MPNs are characterized by proliferation of cells of myeloid lineage—g...

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