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Myeloproliferative Disorders Flashcards

1
Q

Myeloproliferative disorders

These are clonal neoplastic disorders of _____________ characterized by excessive proliferation of one or more of the ______ cell lineages ( granulocytic, erythroid ,megakaryocytic , and ____ cells)

A

pluripotent haematopoietic stem cell (HSC)

myeloid

masst

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2
Q

Myeloproliferative disorders characteristics are:

  1. Originates from ________
  2. Usually _______ predominates in a given disorder.
  3. Enlargements of _____ and ____ occur commonly.
  4. Usually in (children or adults?) with a peak in the ___ to ____ decade
A

a single stem cell

one cell line

spleen and liver

adults; 5th to 7th

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3
Q

INTRODUCTION to myeloproliferative disorders

Maturation of blood cells is relatively (normal or abnormal?) , so that (mature or immature ?) cells increase in number in the absence of ___________

A

Normal

mature

physiologic stimulus.

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4
Q

INTRODUCTION to myeloproliferative disorders

There is a close relationship between different disorders with ________, overlapping ___________, and progression to __________ and ___________.

A

interconversions

manifestations

myelofibrosis and acute leukaemia

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5
Q

INTRODUCTION to myeloproliferative disorders

Extramedullary hematopoiesis: blood cell production can also occur outside the bone marrow leading to ________________

A

enlargement of the liver and spleen.

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6
Q

CLASSIFICATION is Based on nature of predominant proliferating cell line:

____________ (erythroid)

___________(megakaryocytic)

________________(granulocytes)

__________________ (fibrous tissue)

A

Primary polycythemia

Essential thrombocythemia

Chronic myeloid leukemia

Primary myelofibrosis

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7
Q

CLASSIFICATION is Based on nature of predominant proliferating cell line:

_____________ leukaemia

_____________ leukaemia

Mastocytosis

_________________ (unclassifiable)

A

Chronic neutrophilic

Chronic eosinophilic

Myeloproliferative neoplasm

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8
Q

PATHOGENESIS of Myeloproloferative diseases

The presence of mutated, constitutively activated _______ protein: ______.

Which results in the ______- (dependent or independent?) proliferation and survival of marrow progenitors

Proliferation of HSCs leads to a _______ marrow.

Proliferating cells undergo ( normal or abnormal?) differentiation, which results in increased blood counts of one or more mature blood elements.

A

tyrosine kinases ; JAK2

growth factor

independent; hypercellular

normal

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9
Q

Tyrosine kinases activation normally occurs by the binding of __________ to ________ on the marrow progenitor cells.

A

hematopoietic growth factors

surface receptors

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10
Q

POLYCYTHAEMIA

Polycythemia (Erythrocytosis) is defined as an increase in the ____________, usually with a corresponding increase in _________ level.

A

total red cell mass (RCM)

haemoglobin

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11
Q

POLYCYTHAEMIA

The increase in RCM can be ______ or _______.

A

absolute or relative

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12
Q

POLYCYTHAEMIA

can be classified into:

________ polycythemia (red cell mass is __________)

__________ or __________ polycythemia (red cell mass is _______________).

A

Absolute

raised

Relative or pseudo

normal but the plasma volume is reduced

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13
Q

POLYCYTHAEMIA

Absolute polycythemia : which is subdivided into ____ and _______ polycythemia.

A

primary and secondary

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14
Q

POLYCYTHAEMIA

In men, Hb > ____ g/dL or HCT >____

In women, Hb >____ g/dL or HCT >___

A

18.5; 0.52

16.5; 0.48

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15
Q

Relative polycythemia

Characterized by ______ plasma volume with a _____ red cell mass.

A

reduced

normal

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16
Q

Relative polycythemia

May result from ________: following ———-,———- , excessive use of _______

A

dehydration

vomiting, diarrhea,

diurectics

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17
Q

Relative polycythemia

It can occur in ______ syndrome(______ spurious )

______ age
_____weight
Hypertensive
_____

A

Gaisbock

Middle

Over

anxiety

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18
Q

POLYCYTHAEMIA

Secondary
Caused by physiologically appropriate increase in __________ in :

(Low or high?) altitudes
Pulmonary disease and alveolar hypoventilation (______)
Cardiovascular disease , especially ___________
Increased _____ of haemoglobin
Familial (congenital) polycythemia
Heavy cigarette smoking

A

erythropoietin

High; sleep apnea

cyanotic heart disease

affinity

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19
Q

Causes of relative polycythemia

______________.

_________ syndrome

A

Haemoconcentration

Gaisbock

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20
Q

POLYCYTHEMIA VERA(PCV)

Clonal, acquire disorder of the pluripotent haematopoietic stem cells causing excessive proliferation of the __________________________________ lineage

A

erythroids, myeloids, and megakaryocyte

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21
Q

POLYCYTHEMIA VERA(PCV)

However, the major manifestation is seen in _______.
M

A

erythropoiesis

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22
Q

POLYCYTHEMIA VERA(PCV)

Many cases of PCV also has an increase number of granulocytes or platelets

T/F

A

T

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23
Q

POLYCYTHEMIA VERA

Two phases of PV can be recognized:

A _________ or _________ phase

A _____ or ___________ phase

A

proliferative or polycythemic

“spent”, or post-polycythemic

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24
Q

POLYCYTHEMIA VERA

A proliferative or polycythemic phase: a ________ phase characterized by ________ leading to ____________

A “spent”, or post-polycythemic phase: this is characterized by _______ and ______

A

initial; proliferation of erythoid cells

increased red cell mass

cytopaenia and myelofibrosis

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25
Q

_______ is the most common myeloproliferative disorder.

A

PCV

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26
Q

Epidemiology of PCV

The incidence is approximately ___ cases per _____ per year.

Median age at diagnosis is ____ years

A

3; 100000

60

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27
Q

PCV occurs at all ages

PCV is very common below 30 years

A

T

F(but it is very rare below 30 years)

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28
Q

Pathophysiology of PCV

An ____eased number of cases have been reported in survivors of the ___________

Cytogenetic abnormalities are found in only _____% of patients at diagnosis.

A

incr ; Hiroshima atomic bomb.

10-20

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29
Q

Pathophysiology of PCV

____________ mutation associated

Growth factor- independent proliferation and progenitor survival

A

JAK2 V617F

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30
Q

A genetic predisposition has been reported in some families of patients that have PCV

T/F

A

T

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31
Q

Pathophysiology of PCV

Increased _______in PV results in an increased _______ and increased blood ______ which can lead to vascular _______ and/or _______.

A

red cell production

red cell mass ; viscosity; thrombosis; bleeding

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32
Q

Pathophysiology of PCV

The ______ is directly proportional to the number of thrombotic events.

A

hematocrit

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33
Q

Pathophysiology of PCV

Studies have demonstrated a reduction in cerebral blood flow in patients with hematocrits above ____%.

___________ can also contribute to bleeding and thrombosis.

A

53

Thrombocytosis

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34
Q

Clinical features of PCV

Patients are frequently (symptomatic or asymptomatic?) at diagnosis and are discovered ______ when an elevated hematocrit is noted on a FBC.

Most patients will develop symptoms as the __________ and _______ ————

A

asymptomatic; incidentally

hematocrit and/or platelet count increase.

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35
Q

PCV Clinical features

Major symptoms can be listed as follows:

•Symptoms of _______ associated with an elevated hematocrit include headache, _______, _______, weakness, ______ and _______

A

hyperviscosity

blurred vision; dizziness

priapism and paresthesias.

36
Q

PCV Clinical features

_________ typically following a (cold or hot?) shower may be the main complaint. The etiology is unclear but may be associated with cutaneous _________

A

Pruritus

Hot

mast cell degranulation.

37
Q

PCV Clinical Features

Venous or arterial _______, involving ________ vessels is common.

Complications include _______, TIA, CVA, myocardial infarction, and ________

A

thrombosis

small and large

cyanosis
deep vein thrombosis.

38
Q

PCV Clinical Features

________ sites of thromboses are seen more frequently in PCV: splenic, hepatic, portal, and mesenteric veins

Ten percent of patients with _________ syndrome (_______________) have coexisting PV.

A

Unusual

Budd-Chiari

hepatic/IVC thrombosis

39
Q

PCV Clinical Features

Abnormalities in __________ can lead to epistaxis, bruising, gastrointestinal and gingival bleeding.

Hyper metabolism caused by __________ can lead to ________ and _______

A

platelet function

increased blood cell turnover

hyperuricemia and gout.

40
Q

PCV Clinical Features

Gastrointestinal complaints are (common or rare?) including __________ and ________.

Physical findings include _____,________, and _________

A

Common

epigastric discomfort and peptic ulcer disease

splenomegaly, hepatomegaly and facial plethora.

41
Q

Laboratory findings of PCV

Full blood count:
1. The haemoglobin , haematocrit and red cell count are increased (In men, Hb >_____ g/dL or HCT >0.52 and in women, Hb >_____ g/dL or HCT >0.48).

  1. A ______ leukocytosis, raised ______ and raised _______ count is present in about half of the patients.
A

18.5

16.5

neutrophil; platelets; basophil

42
Q

Laboratory findings of PCV

JAK2 mutation analysis: JAK2 mutation is present in the _______ and peripheral blood _______ in 95% of patients.

____________________ (NAP) score is usually
increased.

A

bone marrow; granulocytes

Neutrophil alkaline phosphatase

43
Q

Laboratory findings of PCV

Bone marrow examination: The bone marrow is _______ with prominent _______ , best assessed by a ______ biopsy.

Serum erythropoietin assay: Serum erythropoietin usually low in __________ but high in _______________

A

hypercellular; megakaryocytes; trephine

polycythemia vera

secondary polycythemia.

44
Q

Laboratory findings of PCV

Arterial oxygen saturation

T/F

A

T

45
Q

Criteria for diagnosis of polycythaemia (rubra) vera

Major: ________ or __________

Minor: _____,______, or ________

A

Elevated red cell mass

JAK2-V617F mutation

Hypercellular bone marrow
Low erythropoietin (EPO)
EEC growth in vitro

46
Q

PCV treatment

Treatment focuses on decreasing the _________ to reduce ________ and its attendant complications.

A

hematocrit

plasma viscosity

47
Q

PCV treatment

The goal of therapy is a hematocrit <___% and a platelet count <______.

A

45

400,000

48
Q

PCV treatment

_________
___________ agents

A small daily dose of ______ is recommended for its anti-platelet effect.

___________ to prevent urate nephropathy

A

Phlebotomy

Myelosuppressive

aspirin

Allopurinol

49
Q

Prognosis of PCV

Without therapy, median survival of patients with PV is only __________, but is more than ______ with treatment.

Major causes of death are _______ and _______

Up to 20% of patients develop __________ or __________ which are fatal.

A

6-18 months; 10 years

thrombosis and hemorrhage.

acute myelogeneous leukemia or myelodysplastic syndrome

50
Q

Prognosis of PCV

PCV patients can develop a “spent phase” late in their courses that resembles chronic idiopathic _______ with _____ and marrow _____ and ____ – referred to as post polycythemic myelofibrosis or _________

Post polycythemic myelofibrosis frequently transforms to _________

A

myelofibrosis; cytopenia

hypoplasia and fibrosis; secondary myelofibrosis.

acute leukemia

51
Q

ESSENTIAL THROMBOCYTHEMIA

Clonal myeloproliferative disorder that involves primarily the _____ cell line.

It leads to the dysregulated production of large numbers of _________ in the marrow resulting in marked rise in platelet counts.

________ mutation are found in approximately 50% of patients with ET.

A

megakaryocyte

mature megakaryocytes

JAK2

52
Q

ET Epidemiology

The incidence of the disease is ______/100,000 individuals annually.

Usually occurs at the age of _______ years.
A second peak occurs at approximately ____ years of age, typically in (men or women?) .

A

2-3

50-60

30

Women

53
Q

ET Clinical features

Many patients are (symptomatic or asymptomatic?) and discovered when a routine blood count is performed.

______ manifestation and a predisposition to _______ events are the main clinical findings

A

asymptomatic

Bleeding

thrombotic

54
Q

ET Clinical features

_________ is classic of ET and is believed to be due to microvascular thrombosis of the digital vessel of fingers and toes.

Spontaneous ______ due to placental thrombosis.

A

Erythromelalgia

abortion

55
Q

ET Clinical features

Bleeding occurs most commonly from mucosal surfaces especially the __________

There may be mild ________ and less often _______________.

A

gastrointestinal tract.

splenomegaly

hepatomegaly

56
Q

ET clinical features

Transformation of ET to ________ or a _________ occurs in fewer than ___% of patients.

There can also be delayed disease transformation into a fibrotic state called ______________ or _________

A

acute myeloid leukemia; myelodysplastic syndrome

5

post thrombocythemia myeloid metaplasia or myelofibrosis

57
Q

ET Lab features

Peripheral blood film shows marked ________.

The platelets often display _______ (range from tiny forms to Giant platelets)

A

thrombocytosis

anisocytosis

58
Q

ET Lab features

The bone marrow biopsy is usually ______. The most striking abnormality is marked proliferation of ___________________, usually occurring in loose clusters

A

hypercellular

large and giant megakaryocytes

59
Q

ET Lab features

The white blood cell count and differential are usually (normal or abnormal?) .

The red blood cells are ____cytic and _____chromic. _______________________.

A

Normal

normo

normo

Leukoerythroblastosis

60
Q

Diagnostic Criteria for ET

  1. Persistent elevation of the _____ count ≥ _____ x 109/L.
  2. Megakaryocyte proliferation with _______ and ———- morphology: _______ or _______ granulocyte or erythroid proliferation.
A

Platelet; 450

large and mature

little or no

61
Q

Diagnostic Criteria for ET

  1. Not meeting ______ criteria for CML, PV, PMF, MDS, or other myeloid neoplasm.
  2. Demonstration of ________ or other clonal marker or in the absence of a clonal marker, no evidence of reactive thrombocytosis.
A

WHO

JAK2-V617F

62
Q

ET Treatment and Prognosis

ET is (an indolent or aggressive?) disorder, characterised by long symptom free intervals, interrupted by occasional life- threatening _________ or _______ episodes.

Most patients enjoy a normal life expectancy.

A

indolent

thromboembolic or hemorrhagic

63
Q

ET Treatment and Prognosis

Risk stratification in ET is primarily based upon factors that influence the occurrence of _______ complications.

Patients are considered to be at high risk if they are >____ years of age and/or have a __________

A

thrombotic

60

prior history of thrombosis.

64
Q

PRIMARY MYELOFIBROSIS (PMF)

Clonal disorder of pluripotent stem cell characterised by the proliferation of mainly _________ and _______ elements in the bone marrow associated with reactive _______________________

A

megakaryocytic and granulocytic

deposition of bone marrow connective tissue.

65
Q

PRIMARY MYELOFIBROSIS (PMF)

As the bone marrow becomes ____ and normal hematopoiesis can no longer occur, ______________ (EMH) occurs in the _________ - which worsens throughout the disease course.
•

A

fibrotic

extramedullary hematopoiesis

liver and spleen

66
Q

PRIMARY MYELOFIBROSIS (PMF)

The EMH seems to be (effective or ineffective?) and the patients become _________.

A leukoerythroblastic blood smear with ________ shaped red cells ( _______ ) is a characteristic finding in the fibrotic stage.

A

ineffective; pancytopenic

tear drop; dacrocytes

67
Q

PRIMARY MYELOFIBROSIS (PMF)

Bone marrow fibrosis results from stimulation of _______ proliferation and ________ synthesis by _____________ and transforming growth factor

A

fibroblastic; collagen

platelet derived growth factor

68
Q

PMF Epidemiology

Is a (common or rare?) disease

The incidence MF is ___ per 100,000 populations per year.

. Occurs predominantly in in persons over the age of ____ year.

A

Rare; 1.5

60

69
Q

PMF Epidemiology

Exposure to ______ and ______ has been documented in some cases.

Up to 50% of patients have clonal ______ abnormalities at diagnosis.

A

benzene and ionizing radiation

karyotypic

70
Q

PMF Clinical Features

About _____ of patients are asymptomatic at the time of diagnosis n.

The symptoms of this disease are often non-specific. The majority are due to the ________ and ______.

A

one third

anemia and splenomegaly

71
Q

PMF Clinical Features

The anemia can lead to weakness, fatigue and dyspnea.

The enlarged spleen and liver can lead to abdominal discomfort and early _____.

Progressive ______ is common because of early satiety

_________ is present in at least 50% of the patients.

A

satiety

weight loss

Hepatomegaly

72
Q

PMF Clinical Features

Other constitutional symptoms include fever, anorexia, night sweats and bone pain.

As the disease progresses, _______ occurs due to both ____________ and decreased ______________

A

pancytopenia

splenic sequestration

effective hematopoiesis.

73
Q

PMF Clinical Features

Rarely, extramedullary hematopoiesis can occur at other sites such as the skin, lung, bladder, lymph nodes, genitourinary tract, gastrointestinal tract and the central nervous system.

T/F

A

T

74
Q

PMF Clinical Features

Bleeding is typically due to _______ or related to ______ from portal vein thrombosis.

The proliferation of cells leads to chronic overproduction of _____ which can cause attacks of gouty arthritis.

A

thrombocytopenia; varices

uric acid

75
Q

PMF Diagnosis

gold standard” for the diagnosis of PMF is ???

A

There is no “gold standard” for the diagnosis of PMF.

76
Q

PMF Diagnosis
Full blood count; RBC

Haemoglobin usually ____(because of _________ and _______)

_____chromic ____cytic indices

A

low

ineffective erythropoiesis and hypersplenism

Normo; normo

77
Q

PMF Diagnosis

Full blood count; WBC

____________ but it can be markedly _____.

Platelet; usuall ____________,but ____eases as diseases progress

A

normal or reduced

normal or elevated

decr

78
Q

PMF Diagnosis

Blood film:
•________ shaped red cell
•____________
•Bone marrow aspirate: usually _____(____)

•Bone marrow biopsy: shows _____,increase in the number of __________. It is essential for the diagnosis

A

Tear drops

Leucoerythroblastosis

unsucessfull (dry tap)

fibrosis; abnormal megakaryocytes

79
Q

Leucoerythroblastosis (________ red cells and ________ white blood cell)

A

nucleated

immature

80
Q

PMF stages

_________ (_______) stage:

_______ stage:

A

Prefibrotic(cellular)

Fibrotic

81
Q

PMF stages

Prefibrotic(cellular) stage:

_________ marrow due to _______ proliferation
 Minimal or absent reticulin fibrosis Increased and abnormal ________

A

Hypercellular; granolocytes

megakaryocytes

82
Q

PMF stages

Fibrotic stage:

Presence of _______ fibrosis

_____________ features

A

marrow

Leucoerythroblastic

83
Q

PMF Treatment and Prognosis

____________ treatments to reverse the process of idiopathic myelofibrosis.

Treatment is mainly ________

Most care is directed toward ___________

A

There are no available

supportive
symptomatic management

84
Q

PMF Treatment and Prognosis

The anemia usually requires _____________ but may also respond to __________, ( low or high?) dose steroid or erythropoietin.

Patients younger than ______ years old should be considered for allogeneic bone marrow transplantation.

A

red blood cell transfusions

androgens ; Low

55

85
Q

PMF Treatment and Prognosis

The most common causes of death include infection, hemorrhage, thrombosis, cardiovascular disease, cerebrovascular disease and transformation to acute leukemia which occurs in 10-20% of patients in the first 10 years.

A

T

86
Q

PMF Treatment and Prognosis

_______ splenomegaly can be treated with chemotherapy (Hydroxyurea), splenectomy, splenic radiation or alpha Interferon.

A

Painful

87
Q

PMF Treatment and Prognosis

Average life expectancy is the worst among the CMPDs at ______years, but may approach _____ years in young patients with good prognostic factors.

A

5-7

15

House Hemat (29 decks)

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