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Hematology 2 - CEU MLS > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

clonal hematopoietic stem cell disorders that result in excessive production and overaccumulation of erythrocytes, granulocytes, and platelets in some combination in bone marrow, peripheral blood, and body tissues

A

MYELOPROLIFERATIVE NEOPLASMS (MPN)

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2
Q

“myelo” is associated with:

A

bone marrow and hematopoietic cells
myeloid cells series (RBC, granulocytes, plt.)

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3
Q

In MPN, these cells have an excessive production and overaccumulation

A

erythrocytes
granulocytes
platelets

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4
Q

4 major conditions associated with MPNs

A

Chronic myeloid leukemia (CML)
Polycythemia vera (PV)
Essential thrombocytopenia (ET)
Primary myelofibrosis (PMF)

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5
Q

MPNs with JAK2 mutation

A

Polycythemia vera (PV)
Essential thrombocytopenia (ET)
Primary myelofibrosis (PMF)

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6
Q

Chronic
* Predominant cell type – ?
* Onset: ?
* WBC count – ?
* Production – ?

A
  • Predominant cell type – mature
  • Onset: insidious/gradual (with harmful effects)
  • WBC count – increased
  • Production – lower (months to years)
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7
Q

Myelogenous means?

A

arise from myeloid series cells

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8
Q

Consistently associated with BCR-ABL fusion gene located in Philadelphia chromosome

A

CHRONIC MYELOGENOUS LEUKEMIA

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9
Q

aka Philadelphia chromosome

A

changed chromosome 22

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10
Q

gene and the gene location associated with CML

A

BCR-ABL fusion gene
located in Philadelphia chromosome

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11
Q

reciprocal translocation of DNA between chromosomes 9 and 22

A

Philadelphia chromosome (changed chromosome 22)

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12
Q

Condition with overproduction of tyrosine kinase

A

CHRONIC MYELOGENOUS LEUKEMIA

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13
Q

promotes AML cells survival and proliferation

A

tyrosine kinase

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14
Q

Condition with large numbers of myeloid precursors in BM, peripheral blood, and extramedullary tissues

A

CHRONIC MYELOGENOUS LEUKEMIA

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15
Q

Condition wherein the peripheral blood exhibits leukocytosis with increased myeloid series, particularly the later maturation stages, often with increased eosinophils & basophils

A

CHRONIC MYELOGENOUS LEUKEMIA

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16
Q

Leukocyte Alkaline Phosphatase (LAP) score of CML

A

Dramatically ↓

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17
Q

enzyme found in membranes of secondary granules of neutrophils

A

Leukocyte Alkaline Phosphatase

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18
Q

Stains ALP present in most neutrophil

A

Leukocyte Alkaline Phosphatase Stain (LAPS)

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19
Q

Differentiate CML (↓) from leukemoid reaction or polycythemia vera

A

Leukocyte Alkaline Phosphatase Stain (LAPS)

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20
Q

What is differentiated in Leukocyte Alkaline Phosphatase Stain (LAPS)?

A

CML
Leukemoid reaction or polycythemia vera

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21
Q

How is the degree of reactivity measured in LAPS?

A

scoring each 100 neutrophils according to the amount of precipitated dye present

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22
Q

Scoring of LAPS

A

0 (weak) – 4 (strong)

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23
Q

Anticoagulant used for LAPS specimen?

What must NOT used? What is the result?

A

HEPARIN

EDTA: falsely ↓ LAPS

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24
Q

Conditions with INCREASED LAPS

A

Leukemoid Reaction
Polycythemia Vera
3rd Trimester of Pregnancy

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25
Q

Condition with DECREASED LAPS

A

CML

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26
Q

Condition that is BCR-ABL1 positive

A

CML

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27
Q

BM in this condition exhibits intense hypercellularity with myeloid precursor predominance

A

CML

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28
Q

Megakaryocytes in CML

A

normal to ↑

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29
Q

Can progress from chronic stable phase through an accelerated phase into transformation to acute leukemia (rapid progress and onset of symptoms)

A

CML

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30
Q

TREATMENTS FOR CML

A

BM Transplantation - preferred
Tyrosine kinase inhibitor

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31
Q

targets abnormal BCR-ABL protein to block its function causing the CML cells to die

A

Tyrosine kinase inhibitor

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32
Q

Peripheral blood of this condition has immature basophils and neutrophils, micromegakaryocyte, spectrum of granulocytes, including multiple myelocytes, bands, and immature basophil

A

CML

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33
Q

He first described Polycythemia Vera as Polycythemia Rubra Vera

A

William Osler (1910)

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34
Q

Clinical description of Polycythemia vera according to William Osler (1910)

A

engorged veins
plethora (face and palm swelling)
↑ RBC count
leukocytosis
thrombocythemia

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35
Q

He added PV to the classification of MPNs

A

Dameshek (1951)

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36
Q

Main differential diagnosis of Polycythemia Vera according to Dameshek (1951)

A

Reactive erythrocytosis due to hypoxia

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37
Q

Mechanism of hypoxia causing reactive erythropoiesis

A

Erythropoiesis is stimulated = ↑ RBC production = makes blood thicker than normal → blood clot and other complications

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38
Q

Clonal stem cell disorder characterized by hyperproliferation of erythroid, myeloid, and megakaryocytic lineages

A

POLYCYTHEMIA VERA

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39
Q

Lineages with hyperproliferation in Polycythemia vera

A

erythroid
myeloid
megakaryocytic

40
Q

WHO Diagnostic Criteria for Polycythemia vera must meet?

A

3 major criteria and 1 minor criterion

41
Q

MAJOR CRITERIA OF POLYCYTHEMIA VERA accdg. to WHO

A

Hemoglobin, Hematocrit, RBC mass (has assigned values)

Bone marrow showing hypercellularity for age with trilineage growth (panmyelosis) including
prominent erythroid, granulocytic, megakaryocytic
proliferation with pleomorphic, mature
megakaryocytes (difference is size)

Presence of JAK2V617F/JAK2 exon 12 mutation

42
Q

PV Major criteria for hemoglobin (men & women)

A

Hemoglobin (men): >16.5 g/dL
Hemoglobin (women): >16.0 g/dL

43
Q

PV Major criteria for hematocrit (men & women)

A

Hematocrit (men): >49%
Hematocrit (women): >48%

44
Q

PV Major criteria for RBC mass

A

↑ RBC mass mean normal

45
Q

protein important for controlling the hematopoietic stem cells production of blood cells

A

Janus kinase (JAK)

46
Q

switch of valine (V) to phenylalanine (F) at position 617 (changes the shape of JAK2 protein)

A

JAK2V617F

47
Q

PV Minor criterion

A

Subnormal serum EPO level

48
Q

Additional diagnostic features of PV

A

1) Arterial oxygen saturation of 92% (normal) or
greater and splenomegaly

2) Thrombocytosis: > 400x10^9 platelets/L

3) Leukocytosis: >12x10^9cells/L w/o fever/ infection

4) ↑ LAP, serum vitamin B12, or unbound vitamin B12 binding capacity

49
Q

Arterial oxygen saturation of PV

A

92% (normal) or greater

50
Q

Platelet count of PV

A

> 400x10^9 platelets/L (thrombocytosis)

51
Q

WBC count of PV

A

> 12x10^9cells/L (leukocytosis)
*w/o fever / infection

52
Q

LAP, serum vitamin B12, or unbound vitamin B12 binding capacity of PV

A

↑

53
Q

Clinical S/S of PV

A

Hallmark: Plethora
hyperviscosity
hypertension (50% of pt)
– Headache
– Weakness
– Pruritis - ↑ blood histamine
– Weight loss
– Fatigue
1/2 of PV pt. – thrombocytosis
1/3 of PV pt. – thrombotic/hemorrhagic episodes

54
Q

Hyperviscosity in PV is due to?

A

↑ RBC mass

55
Q

Hypertension in PV is due to?

A

HCT >60%

56
Q

Presenting symptoms associated with hyperviscosity and hyperproliferation

A

Headache
Weakness
Pruritis
Weight loss
Fatigue

57
Q

Hallmark of PV

A

Plethora

58
Q

Associated with ↑ blood histamine and is often not associated with a visible rash in PV patients

A

Pruritis

59
Q

Thrombocytosis are found in ____ of patients

A

1/2

60
Q

Thrombotic/hemorrhagic episodes are found in ____ of patients

A

1/3

61
Q

Increased laboratory tests in PV

A

RBC count
Packed cell volume (HCT)
Hemoglobin

62
Q

Normal laboratory test in PV

A

RBC indices

63
Q

Decreased laboratory test in PV

A

Serum erythropoietin (RBC proliferation is independent of endogenous EPO)

64
Q

All of these values are considered as?

20.6 g/dL Hemoglobin

80% Microhematocrit

28,000 × 10^9/L Total leukocyte (WBC) count

1,400 × 10^9/L Platelet count

A

Peak polycythemic values

65
Q

Peak polycythemic values in hemoglobin

A

20.6 g/dL

66
Q

Peak polycythemic values in microhematocrit

A

80%

67
Q

Peak polycythemic values in total leukocyte (WBC) count

A

28,000 × 10^9/L

68
Q

Peak polycythemic values in platelet count

A

1,400 × 10^9/L

69
Q

TREATMENTS FOR PV

A

Therapeutic phlebotomy
Myelosuppressive therapy
Targeted molecular therapy

70
Q

A treatment for PV wherein there is a controlled removal of large blood volume to reduce blood volume, red cell mass and iron stores

A

Therapeutic phlebotomy

71
Q

A treatment for PV for suppressing RBC overproduction

A

Myelosuppressive therapy

72
Q

A treatment for PV wherein molecules targeted is JAK2 mutation

A

Targeted molecular therapy

73
Q

aka ESSENTIAL THROMBOCYTOSIS

A

Essential thrombocytemia
Primary thrombocytosis
Idiopathic thrombocytosis
Hemorrhagic thrombocythaemia

74
Q

Condition with significant increase in circulating platelets, in excess of 1,000 × 10^9/L

A

ESSENTIAL THROMBOCYTOSIS/
ESSENTIAL THROMBOCYTHEMIA

75
Q

Circulating platelets in essential thrombocytosis is in excess value of

A

1,000 × 10^9/L

76
Q

WHO criteria required for essential thrombocytosis diagnosis

A

sustained thrombocytosis with a platelet count of > or equal to 400 x 10^9/L

77
Q

↑ platelet counts may be encountered as a reactive phenomenon, secondary to a variety of systemic conditions, or essential thrombocythemia, a BM primary disorder

A

essential thrombocytosis

78
Q

MAJOR CRITERIA OF ESSENTIAL THROMBOCYTOSIS/ ESSENTIAL THROMBOCYTHEMIA

A
  1. Megakaryocyte proliferation with large and mature morphology, little to no granulocyte or erythroid proliferation
  2. Rarely, minor (grade 1) increase in reticulin fibers
  3. Must not meet any criteria for BCR-ABL1-positive CML, PV, PMF, MDS or other myeloid neoplasms
  4. JAK2 V617F, CALR, or MPL mutations
79
Q

reticulin fibers level in essential thrombocytosis

A

Rarely, minor (grade 1) increase

80
Q

Mutations in essential thrombocytosis

A

JAK2 V617F, CALR, or MPL mutations

81
Q

MINOR CRITERION OF ESSENTIAL THROMBOCYTOSIS/
ESSENTIAL THROMBOCYTHEMIA

A

Presence of a clonal marker
OR
Absence of evidence of reactive thrombocytosis

82
Q

Describe platelets of patient with essential thrombocytosis

A

Appear normal
Giant bizarre platelets
Platelet aggregates
Micromegakaryocytes
Megakaryocyte fragments

83
Q

Describe erythrocytes of patient with essential thrombocytosis

A

Normocytic and normochromic,

unless iron deficiency is present secondary to excessive bleeding

84
Q

Splenic infarction in patient with essential thrombocytosis has a PBS inclusions and appearance such as?

A
  • Howell-Jolly bodies
  • Nucleated RBCs
  • Poikilocytosis
85
Q

TREATMENT FOR ET

A

plateletpheresis – quickly reduce platelet ct.
reduce platelet count and control thrombosis

86
Q

similar to blood donation but with apheresis machine which returns blood in cycle

A

plateletpheresis

87
Q

results when primary myelofibrosis progeny cells stimulate BM fibroblasts to secrete excessive collagen

A

PRIMARY MYELOFIBROSIS

88
Q

nucleated RBC (normoblasts) and myelocytes are released into the circulation (leukoerythroblastosis) when there is extramedullary hematopoiesis (ie, non-marrow organs) have taken over blood cell production because of fibrosed marrow

A

PRIMARY MYELOFIBROSIS

89
Q

disrupts normal BM architecture and replaces hematopoietic tissue resulting in pancytopenia

A

collagen overproduction

90
Q

what is in excess in primary myelofibrosis

A

collagen

91
Q

collagen overproduction disrupts normal BM architecture and replaces hematopoietic tissue leading to?

A

pancytopenia
(decreased production in all cell types)

*followed by secondary hematopoietic tissue production of cells

92
Q

process where nucleated RBC (normoblasts) and myelocytes are released into the circulation

A

leukoerythroblastosis

93
Q

MAJOR CRITERIA OF PRIMARY MYELOFIBROSIS

A
  1. Megakaryocytic proliferation with abnormal morphology, usually accompanied by reticulin and/or collagen fibrosis
  2. Not meeting the criteria for other MPNs
  3. Evidence of JAK2V617F or other related mutations
94
Q

MINOR CRITERIA OF PRIMARY MYELOFIBROSIS

A
  1. Leukoerythroblastosis (presence of nucleated red cells and immature white cells)
  2. Anemia
  3. Increased serum lactic dehydrogenase (LDH) levels
  4. Splenomegaly
95
Q

presence of nucleated red cells and immature white cells

A

Leukoerythroblastosis

96
Q

Describe Prefibrotic stage of PMF

A
  • Markedly hypercellular BM
  • Dysmegakaryopoiesis
  • Megakaryocytes are morphologically abnormal with bizarre hyperchromatic nuclei (arrow) –> key to PMF recognition
97
Q

key to PMF recognition

A

Megakaryocytes are morphologically abnormal with bizarre hyperchromatic nuclei

Hematology 2 - CEU MLS (4 decks)

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