Myeloproliferative neoplasm medical therapy: Difference between revisions

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The mainstay of therapy for myeloproliferative neoplasm is [[chemotherapy]], [[aspirin]], and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19</ref>
The mainstay of therapy for myeloproliferative neoplasm is [[chemotherapy]], [[aspirin]], and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19</ref>
==Medical Therapy==
==Medical Therapy==
Medical therapy for myeloproliferative neoplasm include:<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/myeloproliferative/hp/chronic-treatment-pdq</ref>
Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm.
*[[Chemotherapy]]
 
*[[Aspirin]]
===Polycythemia vera===
*Palliative care.
 
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
|valign=top|
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Therapy}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Mechanism of Action}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Dosing}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Adverse Effects}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
Aspirin
| style="padding: 5px 5px; background: #F5F5F5;" |
Irreversibly inhibits cyclooxygenase-1 and -2 (COX-1 and COX-2)
| style="padding: 5px 5px; background: #F5F5F5;" |
81mg PO daily
| style="padding: 5px 5px; background: #F5F5F5;" |
Mucosal bleeding
Gastrointestinal bleeding
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Hydroxyurea
| style="padding: 5px 5px; background: #F5F5F5;" |
Inhibits ribonucleotide reductase
| style="padding: 5px 5px; background: #F5F5F5;" |
20mg/kg PO daily
| style="padding: 5px 5px; background: #F5F5F5;" |
Anemia, thrombocytopenia, ulcerations, secondary cancers
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Primary myelofibrosis
| style="padding: 5px 5px; background: #F5F5F5;" |
Megakaryocyte
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*Presence of megakaryocyte proliferation and atypia with reticulin fibrosis
*Not meeting criteria for other myeloproliferative neoplasms
*Presence of ''JAK2'', ''CALR'', or ''MPL'' mutation
''Minor criteria'':
*Anemia
*White blood cell count >11,000 per microliter.
*Palpable splenomegaly
*Elevated LDH
*Leukoerythroblastic smear
Diagnosis requires meeting all major criteria and at least 1 minor criterion.
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Chronic myeloid leukemia
| style="padding: 5px 5px; background: #F5F5F5;" |
Common myeloid progenitor
| style="padding: 5px 5px; background: #F5F5F5;" |
*Presence of BCR-ABL translocation (chromosomes 9 and 22)
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Chronic neutrophilic leukemia
| style="padding: 5px 5px; background: #F5F5F5;" |
Neutrophil
| style="padding: 5px 5px; background: #F5F5F5;" |
*Peripheral blood white blood cell count > 25,000 per microliter with rare myeloblasts and no dysgranulopoiesis
*Bone marrow hypercellularity with increased granulocytes and normal maturation and <5% myeloblasts
*Not meeting criteria for other myeloproliferative neoplasms
*Absence of genetic rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', or ''PCM1-JAK2''
*Presence of ''CSF3R'' ''T618I'' or other characteristic mutation
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Chronic eosinophilic leukemia
| style="padding: 5px 5px; background: #F5F5F5;" |
Eosinophil
| style="padding: 5px 5px; background: #F5F5F5;" |
No formal W.H.O. criteria
*Typically associated with >1,500 eosinophils per microliter in peripheral blood
*Typically associated with rearrangements of ''PDGFRA'', ''PDGFRB'', ''FGFR1'', ''JAK2''
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Myeloproliferative neoplasm, unclassifiable
| style="padding: 5px 5px; background: #F5F5F5;" |
Variable
| style="padding: 5px 5px; background: #F5F5F5;" |
Not meeting criteria for other subcategories
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
Mastocytosis
| style="padding: 5px 5px; background: #F5F5F5;" |
Mast cell
| style="padding: 5px 5px; background: #F5F5F5;" |
''Major criteria'':
*Dense multifocal aggregates of >15 mast cells in bone marrow or other organ
''Minor criteria'':
*Presence of ''c-kit'' ''D816V''mutation
*Expression of CD2, CD25, or both on mast cells
*Serum tryptase level >20ng/ml when patient is at baseline health
*Atypical morphology or spindles in >25% of mast cells in bone marrow or other organ
Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria.
|}
 
 
   
   
==References==
==References==

Revision as of 18:09, 10 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

The mainstay of therapy for myeloproliferative neoplasm is chemotherapy, aspirin, and palliative care. Treatment is directed at reducing the excessive numbers of blood cells.[1]

Medical Therapy

Medical therapy for myeloproliferative neoplasm is based on the specific subtype of myeloproliferative neoplasm.

Polycythemia vera

Therapy Mechanism of Action Dosing Adverse Effects

Aspirin

Irreversibly inhibits cyclooxygenase-1 and -2 (COX-1 and COX-2)

81mg PO daily

Mucosal bleeding Gastrointestinal bleeding

Hydroxyurea

Inhibits ribonucleotide reductase

20mg/kg PO daily

Anemia, thrombocytopenia, ulcerations, secondary cancers

Primary myelofibrosis

Megakaryocyte

Major criteria:

  • Presence of megakaryocyte proliferation and atypia with reticulin fibrosis
  • Not meeting criteria for other myeloproliferative neoplasms
  • Presence of JAK2, CALR, or MPL mutation

Minor criteria:

  • Anemia
  • White blood cell count >11,000 per microliter.
  • Palpable splenomegaly
  • Elevated LDH
  • Leukoerythroblastic smear

Diagnosis requires meeting all major criteria and at least 1 minor criterion.

Chronic myeloid leukemia

Common myeloid progenitor

  • Presence of BCR-ABL translocation (chromosomes 9 and 22)

Chronic neutrophilic leukemia

Neutrophil

  • Peripheral blood white blood cell count > 25,000 per microliter with rare myeloblasts and no dysgranulopoiesis
  • Bone marrow hypercellularity with increased granulocytes and normal maturation and <5% myeloblasts
  • Not meeting criteria for other myeloproliferative neoplasms
  • Absence of genetic rearrangements of PDGFRA, PDGFRB, FGFR1, or PCM1-JAK2
  • Presence of CSF3R T618I or other characteristic mutation

Chronic eosinophilic leukemia

Eosinophil

No formal W.H.O. criteria

  • Typically associated with >1,500 eosinophils per microliter in peripheral blood
  • Typically associated with rearrangements of PDGFRA, PDGFRB, FGFR1, JAK2

Myeloproliferative neoplasm, unclassifiable

Variable

Not meeting criteria for other subcategories

Mastocytosis

Mast cell

Major criteria:

  • Dense multifocal aggregates of >15 mast cells in bone marrow or other organ

Minor criteria:

  • Presence of c-kit D816Vmutation
  • Expression of CD2, CD25, or both on mast cells
  • Serum tryptase level >20ng/ml when patient is at baseline health
  • Atypical morphology or spindles in >25% of mast cells in bone marrow or other organ

Diagnosis requires meeting the one major plus one minor criterion, or 3 minor criteria.


References

  1. National Cancer Institute. Physician Data Query Database 2015.http://www.cancer.gov/types/leukemia/hp/cml-treatment-pdq#section/_19

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