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Clinical Medicine 2 > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What represents stem cell derived clonal myeloproliferation with a propensity to evolve into acute myeloid leukemia (AML), also called the blast-phase myeloproliferative neoplasms?

A

myeloproliferative disorders

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2
Q

Myeloproliferative disorders are (…) in nature, deriving from a genetically transformed (…) that results in clonal myeloproliferation

A
  • clonal
  • hematopoietic, bone marrow stem cells
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3
Q

Mutations of the (…) gene are responsible for the polycythemia vera and a high proportion of cases of essential thrombocythemia and primary myelofibrosis

A

Janus kinase 2 (JAK2)

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4
Q

What is a member of the tyrosine kinase family of enzymes ad is involved in the signal transduction for erythropoietin, thromopoietin, and granulocyte colony-stimulating factor (G-CSF) among other entitites?

A

janus kinase 2 (JAK2)

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5
Q

What gene is mutated in a significant proportion of essential thrombocythemia and primary myelofibrosis patients?

A

the thrombopoietin receptor gene (MPL), or the calreticulin (CALR)

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6
Q

What is it called when an overproduction of RBCs occurs?

A

polycythemia

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7
Q

What is a result of dehydration as the fluid loss results in relative increase in RBC counts and hemoglobin and hematocrit values and resolves with fluid intake?

A

relative polycythemia

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8
Q

What can absolute polycythemia be characterized into?

A
  • primary
  • secondary
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9
Q
  • Which absolute polycythemia is described as an abnormal regulation of the multipotent hematopoietic stem cells?
  • What is another name for this?
A
  • primary absolute polycythemia
  • polycythemia vera
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10
Q

Which absolute polycythemia is described as an increase in erythropoietin as a normal response to chronic hypoxia and an inappropriate response to erythropoietin-secreting tumors?

A

secondary absolute polycythemia

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11
Q

What is the most common absolute polycythemia?

A

secondary polycythemia

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12
Q

A patient comes in with elevated hemoglobin and hematocrit, red cell mass and plasma volume measurements show elevated red cell mass. O2 saturation is found to be > 93% and JAK2 V617F is positive. What is the diagnosis?

A

polycythemia vera

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13
Q

What is a chronic neoplastic, non-malignant condition in which there is an overproduction of RBCs (freqeuntly with an increased WBC count and platelet count)?

A
  • polycythemia vera
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14
Q

Polycythemia vera is associated with elevated levels of what?
What organ may be enlarged?

A
  • RBCs, WBCs, platelets
  • spleen
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15
Q

Polycythemia vera results in an acquired mutation in what?

A

Janus kinase 2 (JAK2)

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16
Q

What is the function of the mutated JAK2 in polycythemia vera?

A

acts like EPO; activates the EPO receptors causing them to be constitutively active, regardless of EPO level

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17
Q

What can polycythemia vera be converted into?

A

acute myeloid leukemia

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18
Q

Regarding polycythemia vera:
- the (…) becomes enlarged, frequently with abdominal pain and discomfort
- As the disease progresses, (…) and (…) increases
- (…) is intensified by heat or exposure to water; this is known as (…)

A
  • spleen
  • blood cellularity and viscosity
  • intense, painful itching; aquagenic pruritis
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19
Q

Signs and symptoms of polycythemia vera are dependent on what?

A

RBC elevation

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20
Q

What are some signs and symptoms of polycythemia vera?

A
  • ruddy cyanosis
  • HTN
  • splenomegaly
  • HA
  • sweating
  • weight loss
  • paresthesias
  • dizziness
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21
Q

Patients with polycythemia can develop (…) from hepatosplenomegaly

A

portal HTN

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22
Q
  • Patients with polycythemia vera are at a high risk of (…)
  • This is due to (…) and (…)
  • This risk is aggrevated by (…)
A
  • thrombosis
  • elevated RBC mass and increased blood viscosity
  • elevated platelet count
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23
Q

What occurs in 2/3 of patients with polycythemia vera without treatment due to elevated RBC mass and increased blood viscosity?

A

thrombosis

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24
Q

What is the major diagnostic criteria for PV?

(H&H values, etc)

A
  1. hemoglobin >16.5 g/dL (men), >16.0 g/dL (women) OR hematocrit >49% (men), >48% (women) OR increased red cell mass >25% above mean predicted value
  2. bone marrow biopsy showing hypercellular for age, trilineage myeloproliferation
  3. presence of JAK2 mutation
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25
Q

What is the minor diagnostic criteria for PV?

A

subnormal serum eryhtropoietin level (low EPO)

26
Q

What does the diagnosis of PV require?

A

presence of all 3 major criteria OR the first two major criteria and the one minor criteria

27
Q

What is the treatment for polycythemia vera?

A
  1. phlebotomy (withdrawal of 300-500 ml of blood at a time) to reduce erythrocytosis and blood volume
  2. low dose aspirin
  3. cytoreductive therapy (interferon-a, hydroxyurea)
  4. radioactive phosphorus
28
Q

If patients with polycythemia are high risk of thombrosis, poor compliance to phlebotomy, or progressive myeloproliferation (splenomegaly, leukocytosis, thrombocytosis), what therapy should be done?

A

cytoreductive therapy:
1. hydroxyurea as first line therapy
2. interferon in selected patients

29
Q

What patients is interferon treatment for PV selected for?

A
  • age < 40 yo
  • pregnancy
  • intractable pruritis
  • intolerance to hydroxyurea
30
Q

What is the management of PV in these risk groups:
- low risk ( age < 60, no thrombosis hx)
- low risk with extreme thrombocytosis
- high risk (age > 60 or thrombosis hx)

A
  • low dose ASA + phlebotomy
  • low dose ASA + phlebotomy
  • low dose ASA + phlebotomy + hydroxyurea
31
Q

A person arrives in the clinic reporting 4 days of nausea, vomiting, and diarrhea. The hematocrit in 61%. The clinician suspects:
1. primary polycythemia
2. secondary polycythemia
3. relative polycythemia
4. absolute polycythemia

A
  1. relative polycythemia
    indicates hematocrit is due to reduction in fluid volume/dehydration with subsequent hemoconcentration
32
Q

What results from hemoconcentration of the blood associated with dehydration that may be caused by decreased water intake, diarrhea, excessive vomiting, or increased use of diuretics?

A

relative polycythemia

33
Q

What is one of several disorders collectively known as chronic myeloproliferative disorders; occurs in response to abnormal regulation of the hematopoietic stem cells?

A

polycythemia vera (primary polycythemia)

34
Q

What results from chronic hypoxemia, which stimulates the release of EPO?

A

secondary polycythemia

35
Q

What describes an increase in erythrocytes that is not related to volume states and includes both primary and secondary polycythemia?

A

absolute polycythemia

36
Q
  • What is characterized by an overproduction of platelets?
  • What is th usual age of presentation of this myeloproliferative disorder?
  • What is the platelet count usually above in this disorder?
  • This disorder is done by way of a diagnosis of (…)
A
  • essential thrombocythemia (thrombocytosis)
  • 5th-6th decades of life
  • 600,000 microL
  • exclusion
37
Q

What is the least aggressive of the myeloproliferative disorders?

A

essential thrombocythemia

38
Q

What is the clinical presentation of patients with essential thrombocythemia?

A
  • asymptomatic (50% of pts)
  • main issues: thrombotic (venous and arterial)
    **microvessel: TIA, HA, visual disturbances, erythromelalgia
    **macrovessel: renal vein thrombosis, Budd-Chiari
39
Q

What is the diagnostic criteria for essential thrombocythemia?

A
  • platelet count > 600,000
  • hematocrit < 40% or normal RBC mass
  • stainable iron in marrow, normal serum ferritin, or normal RBC mean corpuscular volume
  • no philadelphia chromosome or bc/abl gene rearrangement
  • collagen fibrosis of marrow absent or less than 1/3 biopsy area w/o both splenomegaly and leukoerythroblastic rxn
  • no cytogenic/morphologic evidence of myelodysplastic syndrome
  • no cause for reactive thrombocytosis
40
Q

What is the management of essential thrombocythemia in this risk groups:
- low risk (age < 60, no thrombosis hx)
- low risk with extreme thrombocytosis
- high risk (age > 60 or thrombosis hx)

A
  • low dose aspirin
  • low dose aspirin
  • low dose aspirin + hydroxyurea
41
Q
  • Neither ET nor PV is currently a (…) disease
  • Current therapy has not been shown to (…) survival
A
  • curable disease
  • prolong survival
42
Q

What is the leukemic transformation rate at 10 and 15 years with essential thrombocythemia?

A
  • 2.3% at 10 years
  • 5.5% at 15 years
43
Q

What is a clonal stem cell disorder that results in hyperplasia of atypical megakaryocytes which stimulate a non-clonal proliferation of fibroblasts, which then cause fibrosis of the bone marrow?

A

primary myelofibrosis

44
Q

What do the megakaryocytes in primary myelofibrosis do?

A

stimulate non-clonal proliferation of fibroblasts, which cause fibrosis of the bone marrow

45
Q
  • Primary myelofibrosis is a (…) disease
  • What is the median age of those diagnosed with PMF?
  • What is PMF characterized by?
A
  • rare disease
  • 57
  • anemia, mild neutrophilia, thrombocytosis, splenomegaly
46
Q

What is a cardinal feature of primary myelofibrosis?

A

splenomegaly

47
Q

What is the clinical presentation of a patient who is diagnosed with primary myelofibrosis?

A
  • signs and symptoms of anemia
  • night sweats
  • weight loss
  • hypersplenism and splenomegaly
48
Q

As PMF progressed, what will patients experience?

A
  • bone marrow failure
  • portal hypertension
  • pulmonary hypertension
49
Q

What is included in the magor diagnostic criteria for primary myelofibrosis?

A
  1. presence of megakaryocyte proliferation and atypia usually accompanied by reticulin or collagen fibrosis; in absence of reticulin fibrosis, megakaryocyte changes accompanied by increased bone marrow cellularity (granulocyte proliferation) and decreased erythropoiesis)
  2. not being WHO criteria for PV, CML, MDS, or other myeloid neoplasm
  3. demonstration of mutated JAK2 or other clonal marker; or no evidence of bone marrow fibrosis d/t underlying inflammatory or neoplastic disease
50
Q

What is included in the minor diagnostic criteria for primary myelofibrosis?

A
  • leukoerythroblastosis
  • increased LDH
  • anemia
  • palpable splenomegaly
51
Q

In order for the diagnosis of primary myelofibrosis, what major/minor criteria must be met?

A

all 3 major + 2 minor

52
Q
  • Conventional therapy for PMF is largely palliative and has not been shown to do what?
  • What are the main indications for treatment in PMF?
A
  • improve survival rate
  • anemia and symptomatic splenomegaly
53
Q

What is the treatment for PMF associated anemia?

A
  • RBC transfusions
  • EPO stimulating agents
  • androgens and glucocorticoids
  • investigational agents
54
Q

What is the drug of choice for symptomatic splenomegaly in PMF?

A

hydroxyurea (500 mg orally 2x a day)

55
Q

What are the indications for a splenectomy in PMF patients?

A
  • mechanical discomfort
  • symptomatic portal HTN (ascites/variceal bleeding)
  • frequent RBC transfusions
56
Q

What is the perioperative mortality rate of a splenectomy in PMF?

A

between 5-10%

57
Q

What is this image showing?

A

PV

58
Q

What is this image showing? What disorder is this associated with?

A
  • ruddy cyanosis
  • PV
59
Q

What is this peripheral smear associated with?

A

ET

60
Q

What disorder is this bone marrow examination associated with?

A

ET

61
Q

What is this image showing? What disorder is this associated with?

A
  • splenomegaly
  • PMF
62
Q

What is this bone marrow examination associated with?

A

PMF

Clinical Medicine 2 (9 decks)

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