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NCCN > MPN > Flashcards

MPN Flashcards

1
Q

Treatment for symptomatic PMF

A
  • Ruxolitinib
  • Peginterferon alfa-2a
  • Hydroxyurea, if cytoreduction would be symptomatically beneficial
  • Pacritinib (if platelets < 50 x 109/L)
  • Momelotinib (category 2B)
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2
Q

If asymptomatic, monitor for signs and symptoms of disease progression every _____

A

3-6 months

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3
Q

Evaluation for allogeneic HCT is recommended for

A
  • Patients with low platelet counts
  • Complex cytogenetics
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4
Q

Treatment for Higher Risk PMF, Plt >50, with symptomatic splenomegaly and/or constitutional symptoms

ALWAYS ASSESS IF TRANSPLANT CANDIDATE

A
  • Ruxolitinib (category 1)
  • Fedratinib (category 1)

Others:
* Momelotinib
* Pacritinib (category 2B)

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5
Q

Treatment for Higher Risk PMF, Plt <50

ALWAYS ASSESS IF TRANSPLANT CANDIDATE

A
  • Pacritinib (category 1)

Other recommended regimen:
Momelotinib (category 2B)

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6
Q

MANAGEMENT OF MF-ASSOCIATED ANEMIA

Symptomatic splenomegaly and/or constitutional symptoms currently controlled on a JAK inhibitor

A

Preferred regimen
* Clinical trial

Other recommended regimens
* Ruxolitinib combination
Add luspatercept-aamt
Add erythropoiesis-stimulating agents (ESAs) (if serum EPO < 500 mU/mL)
Add danazol (category 2B)

Useful in certain circumstances
* Change to momelotinib
* Change to pacritinib

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7
Q

MANAGEMENT OF MF-ASSOCIATED ANEMIA

Symptomatic splenomegaly and/or constitutional symptoms currently not controlled

A

Preferred regimens
* Clinical trial
* Momelotinib

Other recommended regimens:
* Pacritinib
* Ruxolitinib combination
Add luspatercept-aamt
Add erythropoiesis-stimulating agents (ESAs) (if serum EPO < 500 mU/mL)
Add danazol (category 2B)

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8
Q

MANAGEMENT OF MF-ASSOCIATED ANEMIA

Anemia and no symptomatic splenomegaly and/or constitutional symptoms

A

Preferred regimen
* Clinical trial

Other recommended regimens
* Luspatercept-aamt
* ESAs (if serum EPO <500 mU/mL)
* Danazol
* Momelotinib (category 2B)
* Pacritinib (category 2B)

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9
Q

Useful for del(5q)

A

Lenalidomide + prednisone

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10
Q

Medication that requires prostate cancer screening and monitoring of LFTs as well as the use of concomitant medications such as statins are recommended over concerns for increased risk of rhabdomyolysis

A

Danazol

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11
Q

DYNAMIC INTERNATIONAL PROGNOSTIC SCORING SYSTEM (DIPSS)

A
  • Age
  • White blood cell count
  • Hemoglobin, g/dL ≥10 <10
  • Peripheral blood blast, %
  • Constitutional symptoms

Low 0
Intermediate-1 (INT-1) 1 or 2
Intermediate-2 (INT-2) 3 or 4
High 5 or 6

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12
Q

DIPSS-PLUS

A
  • DIPSS low-risk 0
  • DIPSS intermediate-risk 1 (INT-1) 1
  • DIPSS intermediate-risk 2 (INT-2) 2
  • DIPSS high-risk 3
  • Platelets < 100 x 109/L 1
  • Transfusion need 1
  • Unfavorable karyotype 1

Low 0
Intermediate-1 (INT-1) 1
Intermediate-2 (INT-2) 2 or 3
High 4 to 6

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13
Q

Unfavorable karyotype (in DIPSS Plus)

A
  • Complex karyotype
  • Sole o ttwo abnormalities that include
  • trisomy 8
  • 7/7q-
  • i(17q)
  • -5/5q-
  • 12p-
  • inv(3),
  • 11q23 rearrangement
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14
Q

MUTATION-ENHANCED IPSS (MIPSS-70) FOR PATIENTS WITH PMF AGE ≤70 YEARS

A
  • Hemoglobin < 10 g/dL 1
  • Leukocytes >25 x 109/L 2
  • Platelets < 100 x 109/L 2
  • Circulating blasts ≥2% 1
  • Bone marrow fibrosis grade ≥ 2 1
  • Constitutional symptoms 1
  • CALR type-1 unmutated genotype 1
  • High-molecular-risk (HMR) mutations 1
  • ≥2 HMR mutations 2
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15
Q

High-molecular-risk (HMR) mutations

A
  • ASXL1
  • EZH2
  • SRSF2
  • IDH1/2

SEAI

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16
Q

MUTATION AND KARYOTYPE-ENHANCED IPSS
(MIPSS-70+ VERSION 2.0) FOR PATIENTS WITH PMF

A
  • Severe anemia (Hemoglobin < 8 g/dL in women and < 9 g/dL in men) 2
  • Moderate anemia (Hemoglobin 8–9.9 g/dL in women and 9–10.9 g/dL in men) 1
  • Circulating blasts ≥2% 1
  • Constitutional symptoms 2
  • Absence of CALR type 1 mutation 2
  • HMR mutations
  • ≥2 HMR mutations 3
  • Unfavorable karyotype 3
  • Very-high-risk (VHR) karyotype 4

Very low 0
Low 1–2
Intermediate 3–4
High 5–8
Very high ≥9

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17
Q

Unfavorable karyotype (in MIPSS-70+ Version 2.0)

A
  • Any abnormal karyotype other than normal karyotype
  • Sole abnormalities of:
  • 20q-
  • 13q-
  • +9
  • chromosome 1 translocation/duplication,
  • -Y or sex chromosome abnormality other than –Y
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18
Q

VHR karyotype:

A
  • Single/multiple abnormalities of −7
  • i(17q)
  • inv(3)/3q21
  • 12p−/12p11.2
  • 11q−/11q23
  • Other autosomal trisomies not including + 8/+9 (eg, +21, +19)
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19
Q

MYELOFIBROSIS SECONDARY TO PV AND ET-PROGNOSTIC MODEL (MYSEC-PM)

A
  • Age at diagnosis 0.15 per patient’s year of age
  • Hemoglobin < 11 g/dL 2
  • Circulating blasts ≥3% 2
  • Absence of CALR type 1 mutation 2
  • Platelets < 150 x 109/L 1 Constitutional symptoms 1

Low <11
Intermediate-1 (INT-1) ≥11 and <14
Intermediate-2 (INT-2) ≥14 and <16
High ≥16

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20
Q

POLYCYTHEMIA VERA

Low-risk:

High-risk:

A

Low-risk
* Age < 60 years and no prior history of thrombosis

High-risk
* Age ≥ 60 years and/or prior history of thrombosis

21
Q

TREATMENT FOR LOW-RISK POLYCYTHEMIA VERA

A
  • Manage cardiovascular risk factors
  • Aspirin (81–100 mg/day)
  • Phlebotomy (to maintain hematocrit < 45%)

Aspirin twice daily may be considered for patients with refractory symptoms

22
Q

TREATMENT FOR HIGH-RISK POLYCYTHEMIA VERA

In addition to Aspirin, Phlebotomy

A

Preferred regimens:
* Hydroxyurea
* Ropeginterferon alfa-2b-njft

Other recommended regimen:
* Peginterferon alfa-2a
Useful in certain circumstances:
* Ruxolitinib

23
Q

FDA approved for the treatment of patients with PV who have had an inadequate response to or are intolerant of hydroxyurea.

Have activity after inadequate response or loss of response to other agents besides hydroxyurea

A

Ruxolitinib

24
Q

MIPSS-PV

A
  • Thrombosis history 1
  • Leukocyte count ≥15x109/L 1
  • Age >67 2
  • Adverse mutations (SRSF2) 3

Low 0–1
Intermediate 2–3
High ≥4

25
Q

ESSENTIAL THROMBOCYTHEMIA

Very-low-risk

Low-risk

Intermediate-risk

High-risk

A

Very-low-risk
* Age ≤60 years, no JAK2 mutation, no prior history
of thrombosis

Low-risk
* Age ≤60 years, with JAK2 mutation, no prior
history of thrombosis

Intermediate-risk
* Age ˃60 years, no JAK2 mutation, no prior history
of thrombosis

High-risk
* History of thrombosis at any age or age >60 years
with JAK2 mutation

26
Q

TREATMENT FOR VERY-LOW-RISK OR LOW-RISK OR INTERMEDIATE-RISK ESSENTIAL THROMBOCYTHEMIA

A
  • Manage cardiovascular risk factors
  • Aspirin (81–100 nmg/day) for patientsb with vasomotor/microvascular disturbances
27
Q

TREATMENT FOR HIGH-RISK ESSENTIAL THROMBOCYTHEMIA

In addition to Aspirin

A

Hydroxyurea

Other recommended regimens for cytoreductive therapy:
* Peginterferon alfa-2a
* Anagrelide

28
Q

Can be considered for patients in need of cytoreductive therapy who are younger or pregnant or who defer hydroxyurea

A

Peginterferon alfa-2a

29
Q

Used in emergent situations, eg, severe thrombocytosis-related neurologic complications

A

Plateletpheresis

30
Q

MIPSS-ET

A
  • Male sex 1
  • Leukocyte count ≥11 x 109/L 1
  • Adverse mutations (SRSF2, SF3B1, U2AF1, TP53) 2
  • Age >60 4

Low 0–1
Intermediate 2–5
High ≥6

31
Q

Treatment for Accelerated/ blast phase MPN

MPN-accelerated phase (blasts 10%– 19% in peripheral
blood or bone marrow)

MPN blast phase (blasts ≥20% in peripheral blood or bone marrow)

A

HCT

32
Q

Treatment for Accelerated/ blast phase MPN

Not a candidate for transplant

A
  • Clinical trial
  • HMA ± JAK inhibitors
  • HMA + venetoclaxd
  • Low-intensity chemotherapy
33
Q

CRITERIA

PMF, early/prefibrotic stage (pre-PMF)

A

Major criteria
1. Bone marrow biopsy showing megakaryocytic proliferation and atypia, bone marrow fibrosis grade < 2, increased age-adjusted bone marrow cellularity, granulocytic proliferation, and (often) decreased erythropoiesis
2. JAK2, CALR, or MPL mutation or presence of another clonal marker or absence of reactive bone marrow reticulin fibrosis
3. Diagnostic criteria for BCR::ABL1-positive CML, PV, ET, myelodysplastic syndromes, or other myeloid neoplasms are not met

Minor criteria
* Anemia not attributed to a comorbid condition
* Leukocytosis ≥11 x 109/L
* Palpable splenomegaly
* LDH level above the above reference range

The diagnosis of pre-PMF or overt PMF requires all 3 major criteria and at least 1 minor criterion confirmed in 2 consecutive determinations

34
Q

PMF, overt fibrotic stage

A

Major criteria
1. Bone marrow biopsy showing megakaryocytic proliferation and atypia, accompanied by reticulin and/or collagen fibrosis grades 2 or 3
2. JAK2, CALR, or MPL mutation or presence of another clonal marker or absence of reactive myelofibrosis
3. Diagnostic criteria for ET, PV, BCR::ABL1-positive CML, myelodysplastic syndrome, or other myeloid neoplasms are not met

Minor criteria
* Anemia not attributed to a comorbid condition
* Leukocytosis ≥ 11 x 109/L
* Palpable splenomegaly
* LDH level above the above reference range
* Leukoerythroblastosis

The diagnosis of pre-PMF or overt PMF requires all 3 major criteria and at least 1 minor criterion confirmed in 2 consecutive determinations

35
Q

Morphology of megakaryocytes in PMF:

A
  • Small to giant megakaryocytes with a prevalence of severe maturation defects (cloud-like, hypolobulated, and hyperchromatic nuclei)
  • Presence of abnormal large dense clusters (mostly >6 megakaryocytes lying strictly adjacent)
36
Q

CRITERIA

PV

A

Major criteria
1. Elevated hemoglobin concentration or elevated hematocrit or increased red blood cell mass
2. Bone marrow biopsy showing age-adjusted hypercellularity with trilineage proliferation (panmyelosis), including prominent erythroid, granulocytic, and increase in pleomorphic, mature megakaryocytes without atypia
3. Presence of JAK2 V617F or JAK2 exon 12 mutation

Minor criterion
* Subnormal serum erythropoietin level

The diagnosis of PV requires either all 3 major criteria or the first 2 major criteria plus the minor criterion

37
Q

Diagnostic thresholds for PV:

Hemoglobin:

Hematocrit:

Red blood cell mass:

A

Hemoglobin: >16.5 g/dL in men and >16.0 g/dL in women

Hematocrit: >49% in men and** >48%** in women

Red blood cell mass: >25% above mean normal predicted value

38
Q

A bone marrow biopsy may not be required in patients with

A
  • Sustained absolute erythrocytosis (hemoglobin concentrations of >18.5 g/dL in men or >16.5 g/dL in women and hematocrit values of >55.5% in men or >49.5% in women) and
  • Presence of a JAK2 V617F or JAK2 exon 12 mutation
39
Q

CRITERIA

ET

A

Major criteria
1. Platelet count ≥450 × 109/L
2. Bone marrow biopsy showing proliferation mainly of the megakaryocytic lineage, with increased numbers of enlarged, mature megakaryocytes with hyperlobulated staghorn-like nuclei, infrequently dense clusters; no significant increase or left shift in neutrophil granulopoiesis or erythropoiesis; no relevant bone marrow fibrosis
3. Diagnostic criteria for BCR::ABL1-positive CML, PV, PMF, or other myeloid neoplasms are not met
4. JAK2, CALR, or MPL mutation

Minor criteria
* Presence of a clonal marker or absence of evidence of reactive thrombocytosis

The diagnosis of ET requires either all major criteria or the first 3 major criteria plus the minor criteria

40
Q

Reactive causes of thrombocytosis

A
  • Iron deficiency
  • Chronic infection
  • Chronic inflammatory disease
  • Medication
  • Neoplasia
  • History of splenectomy
41
Q

Symptom response requires ____% reduction in the MPN-SAF TSS.

A

≥50%

42
Q

Iron chelation could be considered for patients who have received > ______ transfusions and/or ferritin ______ ng/mL in patients with lower-risk
MF.

A

> 20 transfusions and/or
Ferritin >2500 ng/mL

However, the role of iron chelation remains unclear.

43
Q

Consider this vaccine for patients on, or prior to, treatment with a JAK inhibitor

A

Recombinant (killed) zoster vaccine

44
Q

Used in MPN-associated bone pain due to their efficacy in the treatment of growth-factor–related bone pain

A

Loratadine and nonsteroidal anti-inflammatory drugs (NSAIDs) (naproxen)

45
Q

All females with PV should maintain hematocrit, ideally, below the gestational range:

A
  • < 41% trimester 1
  • < 38% trimester 2
  • < 39% trimester 3
46
Q

Definition of High-Risk Pregnancy in PV or ET

A
  • Previous venous and/or arterial thrombosis with/without pregnancy
  • Previous hemorrhage due to PV or ET
  • Previous pregnancy complications:
  • Unexplained death of a morphologically normal fetus ≥ 10 weeks of gestation. Premature delivery of a morphologically normal fetus at < 34 weeks of gestation due to:
    ◊ Severe preeclampsia or eclampsia defined according to standard criteria
    ◊ Recognized features of placental insufficiency
  • ≥3 unexplained consecutive miscarriages at < 10 weeks of gestation, without anatomic, hormonal, or chromosomal abnormalities
  • Unexplained intrauterine growth restriction
  • Significant antepartum or postpartum hemorrhage
47
Q

Recommendations for the Management of High-Risk Pregnancy

A

Treatment to start (continue) when pregnancy test is positive, and pregnancy is considered high risk:
* Low-dose aspirin daily
* Prophylactic LMWH throughout pregnancy and for 6 weeks postpartum
* Cytoreductive therapy with interferon or peginterferon alfa-2a

48
Q

PV and ET

Complete response is defined as:

A
  • Hematocrit less than 45% without phlebotomy
  • Platelet count ≤400 x 109/L
  • WBC count ≤10 x 109/L
  • No disease-related symptoms

Partial response is defined as hematocrit less than 45% without phlebotomy or response in three or more of other criteria.

NCCN (13 decks)

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