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Myeloproliferative Disorders Flashcards

1
Q

Hypercellular marrow with abnormal megakaryocytes early on

A

Primary Myelofibrosis

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2
Q

Appearance of a “spent” phase with fibrosis and cytopenias

A

myeloproliferative disorders

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3
Q

Progress to a “spent” phase‐later in the disease course • Extreme marrow fibrosis • Extramedullary hematopoiesis (liver , spleen )‐ organomegaly

A

Polycythemia vera

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4
Q

Tyrosine kinase induces signaling via (1) pathways

A

RAS and JAK/STAT

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5
Q

Bone marrow cells mature via growth factors that bind receptors on progenitor cells which activate (1) which activate pathways for cell growth and survival

A
  1. tyrosine kinases
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6
Q

What are the clinical signs of polycythemia vera?

A

Plethoric, cyanosis, headache, dizziness, HTN, pruritis at night, hyperuricemia

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7
Q

Symptomatic hyperuricemia‐ increased cell turnover

A

Polycythemia vera and primary myelofibrosis

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8
Q

What is the most characteristic symptom of polycythemia vera? What causes it?

A

Pruritus at night due to basophils releasing histamine

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9
Q

Inappropriate release of fibrogenic factors from neoplastic megakaryocytes

A

Primary Myelofibrosis Platelet derived growth factor and TGF‐beta

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10
Q

CML has markedly hypercellular bone marrow containing hyperplasia of what cell types?

A

myelocytes metamyelocytes bands eosinophils basophils megakaryocytes

but not erythrocytes

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11
Q

How is CML diagnosed?

A

High WBC, peripheral smear and bone marrow findings BCR/ABL fusion detected –chromosome analysis or by PCR

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12
Q

Death within months if untreated due to bleeding or thrombosis

A

Polycythemia vera

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13
Q

70% of blast crises are (1) leukemia

A

acute myeloid

refers to CML

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14
Q

How do you distinguish polycythemia vera from secondary polycythemia?

A

P. vera high oxygen saturation and low erythropoetin Secondary polycythemia low oxygen saturation and high erythropoetin

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15
Q

participates in JAK.STAT pathway downstream from hematopoietic growth factors

A

JAK2

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16
Q

Mutated (1) lead to growth factor independent proliferation and survival of (2)

A
  1. tyrosine kinases 2. marrow progenitors refers to myeloproliferative disorders
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17
Q

Major bleeding and thrombotic events occur

A

Polycythemia vera

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18
Q

Absence of fibrosis

A

essential thrombocytosis

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19
Q

mutations in essential thrombocytosis

A

JAK2 activating mutations MPL mutation thrombopoietin activated protein kinase Calreticulin mutation‐functions in the endoplasmic reticulum

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20
Q

Features of Primary Myelofibrosis

A

Pancytopenia • Extramedullary hematopoiesis

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21
Q

presence of a chimeric BCR‐ABL fusion gene coding an active BCR‐ABL tyrosine kinase resulting in?

A

growth factor independent proliferation and survival of marrow progenitors refers to CML (Chronic myelogenous leukemia)

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22
Q

Iron deficiency from bleeds has a beneficial effect

A

Polycythemia vera

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23
Q

CML peripheral blood findings

A

Leukocytosis (high WBC count) Entire spectrum of marrow cells present Basophilia Blast count < 10%

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24
Q

Osteosclerosis replaces the marrow spaces

A

Primary Myelofibrosis

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25
Mutations originate in (1) level or (2) level
1. multipotential myeloid progenitor 2. pluri‐potential stem cell refers to myeloproliferative disorders
26
Transforms to AML‐up to 20% of cases
Primary Myelofibrosis
27
Increased HCT, blood viscosity,abnormal platelet function
Polycythemia vera
28
How is CML treated?
Hematopoietic stem cell transplant for young stable patients Therapy with BCR/ABL tyrosine kinase inhibitors
29
Obliterative bone marrow fibrosis in primary myelofibrosis leads to?
pancytopenia and extra medullary hematopoesis
30
6mos or so‐enters “blast crisis” acute leukemia; 50% enter blast crisis without the accelerated period
CML
31
In myeloproliferative disorders, (1) is not impaired and increased numbers of (2) are therefore produced
1. differentiation 2. mature blood elements
32
Identical to “spent “phase of other myeloproliferative disorders
Primary Myelofibrosis
33
In essential thrombocytosis progenitors undergo (1) independent‐proliferation
thrombopoietin
34
Pancytopenia
Primary Myelofibrosis
35
BCR gene –chromosome ?
22
36
Slow progression (3 yr. survival even if untreated) Followed by “accelerated phase”‐increasing anemia and thrombocytopenia
CML
37
major clinical manifestations of essential thrombocytosis
Thrombosis and hemorrhage from defective platelet function • DVT • Portal and hepatic vein thrombosis • MI; throbbing/burning sensation of hands and feet
38
Leukoerythroblastosis (myelocytes, metamyelocytes and early erythroid forms in peripheral blood)
Primary Myelofibrosis AND infiltrative marrow processes such as metastatic tumor
39
How does CML present?
Hypermetabolism resulting in fatigue, weakness, weight loss, anorexia Dragging sensation or pain from splenic infarcts
40
Hyper cellular marrow of all cell lines but mostly red cell series; basophilia
Polycythemia vera
41
Reciprocal translocation Philadelphia chromosome (9;22)(q34;q11)
CML (Chronic myelogenous leukemia)
42
Marked megakaryocyte increase with bizarre abnormal forms
essential thrombocytosis
43
Infections; Bleeding and thrombotic episodes
Primary Myelofibrosis
44
Mild hepatosplenomegaly‐extramedullary hematopoiesis‐50%
essential thrombocytosis
45
throbbing/burning sensation of hands and feet
Erythromelalgia; due to platelet aggregates occurs in essential thrombocytosis
46
Treatment for polycythemia vera
Simple regular phlebotomy and low dose aspirin
47
(1) to (2) substitution at residue 617
1. Valine 2. phenylalanine refers to Polycythemia vera
48
Large platelets in peripheral blood
essential thrombocytosis
49
thrombopoietin activated protein kinase
MPL - mutated in essential thrombocytosis and primary myelofibrosis
50
Primary Myelofibrosis mutations
Activating JAK2 mutations‐50% • MPL mutations
51
Median survival‐up to 15 yrs with long asymptomatic periods
essential thrombocytosis
52
Variable transformation to (1) in myeloproliferative disorders
acute leukemias
53
Progressive replacement of marrow with (1)
1. collagen Primary Myelofibrosis
54
Primary Myelofibrosis treatment
JAK2 inhibitors can decrease the splenomegaly somewhat Hematopoietic stem cell transplant for younger pts
55
essential thrombocytosis elevated (1) without (2)
1. platelet counts 2. polycythemia or marrow fibrosis
56
panmyelosis of proliferation red cells, white cells, and platelets
Polycythemia vera
57
functions to promote cell proliferation and manufacturing in the ER
Calreticulin - mutated in essential thrombocytosis
58
S/S of Primary Myelofibrosis
Dragging sensation –massively enlarged spleen Fatigue, weight loss, night sweats Hyperuricemia‐increased cell turnover
59
Strong association with activating point mutations in JAK/2 TYROSINE KINASE
Polycythemia vera, essential thrombocytosis, primary myelofibrosis
60
obliterative marrow fibrosis
Primary Myelofibrosis
61
ABL gene‐chromosome ?
9
62
1% transform to AML
Polycythemia vera
63
Dacrocytes (Tear‐drop Rbc’s)
Primary Myelofibrosis
64
Progressive marrow fibrosis and hypo cellularity and atypical megakaryocytes
Primary Myelofibrosis
65
Mild organomegaly; Minimal extramedullary hematopoiesis
Polycythemia vera
66
Other CML findings
splenomegaly and hepatomegaly, splenic infarcts
67
mild organomegaly is seen in which conditions?
polycthemia vera and essential thrombocytosis
68
dragging sensation or pain from splenic infarcts is seen in which myeloproliferative disorders?
CML and primary myelofibrosis
69
High WBC count
CML
70
Very high hematocrit
polycythemia vera
71
elevated platelet counts
essential thrombocytosis

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