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Myeloproliferative Disorders Flashcards

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1
Q

What is a myeloproliferative neoplasm (MPN)?

A

A clonal proliferation of hematopoietic myeloid stem cells in the bone marrow.

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2
Q

What are the types of myeloproliferative neoplasms?

A
  • Chronic myeloid leukemia (CML)
  • Polycythemia vera (PV)
  • Essential thrombocythemia (ET)
  • Myelofibrosis (MF)
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3
Q

Define myeloid malignancies.

A

Clonal diseases derived from a single cell in the marrow that has undergone genetic alteration.

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4
Q

What are the common causes of myeloid malignancy?

A
  • Hereditary causes
  • Acquired from irradiation
  • Chemicals
  • Previous chemotherapy
  • Infections
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5
Q

What cellular changes may occur in myeloid malignancy?

A
  • Proliferation
  • Increased survival
  • Loss of maturation
  • Loss of differentiation
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6
Q

What mechanisms of genetic alteration are involved in myeloid malignancies?

A
  • Stimulation of tyrosine kinase
  • Deletion of tumor suppressor gene
  • Activation of oncogenes
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7
Q

What is polycythemia?

A

An increase in hemoglobin concentration above the upper limit of normal for the patient’s age and sex.

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8
Q

What are the subtypes of polycythemia?

A
  • Relative polycythemia
  • Absolute polycythemia (Primary and Secondary)
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9
Q

What is the definition of essential thrombocytosis (ET)?

A

Sustained increase in the platelet count (> 450,000/ml) due to megakaryocyte proliferation.

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10
Q

What are common clinical presentations for myeloproliferative neoplasms?

A
  • Constitutional symptoms (weight loss, night sweats, fever)
  • Anemia or hyperviscosity
  • Headache
  • Fatigue
  • Shortness of breath
  • Easy bruising or bleeding
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11
Q

What is the major criterion for diagnosing Polycythemia Rubra Vera?

A
  • High hematocrit (> 49% in men)
  • High hemoglobin (> 16.5 g/dL in men)
  • Raised red cell mass (> 25%)
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12
Q

What are the minor criteria for diagnosing Polycythemia Rubra Vera?

A

Subnormal serum erythropoietin test.

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13
Q

What is the typical treatment for Polycythemia Rubra Vera?

A
  • Venesection
  • Hydroxyurea
  • Interferon
  • Aspirin (75 mg/D)
  • Anagrelide
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14
Q

What is the prognosis for Polycythemia Rubra Vera?

A

Median survival is 10-16 years. May develop myelofibrosis or AML.

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15
Q

What are common causes of thrombocytosis?

A
  • Increased bone marrow production (reactive process)
  • Redistribution of platelets
  • Overproduction of platelets by neoplastic progenitor cells
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16
Q

What symptoms are associated with essential thrombocytosis?

A
  • Asymptomatic
  • Constitutional symptoms (weight loss, fever, pruritus)
  • Abdominal pain from splenomegaly or hepatomegaly
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17
Q

True or False: Myeloid malignancies can be caused by genetic alterations.

A

True

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18
Q

Fill in the blank: Myeloproliferative disorders are characterized by __________ of hematopoietic myeloid stem cells.

A

clonal proliferation

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19
Q

What is a common complication in women of childbearing age with a history of thrombosis?

A

Spontaneous abortion due to placental thrombosis

This can lead to various hemorrhagic events.

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20
Q

What is the most common site of hemorrhagic events in patients with thrombosis?

A

Gastrointestinal tract

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21
Q

What is increased in the peripheral blood film examination in thrombosis patients?

A

Platelet count, sometimes erythrocytosis and leukocytosis

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22
Q

What does a bone marrow biopsy show in patients with thrombosis?

A

Marked megakaryocytic hyperplasia and clustering of bizarre megakaryocytes

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23
Q

What are the major criteria for diagnosing essential thrombocythemia?

Study These Flashcards
A

[“Sustained platelet count above 450 × 10^9/L”, “Bone marrow biopsy showing proliferation of megakaryocyte lineage”, “No other myeloid malignancy or related syndromes”, “Presence of an acquired pathogenetic mutation (JAK2, CALR, MPL)”]

24
Q

What are the minor criteria for diagnosing essential thrombocythemia?

Study These Flashcards
A

[“Presence of a clonal marker”, “Absence of evidence for reactive thrombocytosis”]

25
What is required for a diagnosis of essential thrombocythemia?
Meeting all four major criteria or the first three major criteria and both minor criteria
26
What is the aim of treatment for essential thrombocythemia?
To control the platelet count to reduce the risk of thrombosis
27
What are the treatment options for essential thrombocythemia?
["Hydroxyurea", "Anagrelide + Aspirin", "Alpha-interferon", "Plateletpheresis combined with myelosuppressive therapy for severe cases"]
28
What is the primary feature of myelofibrosis?
Progressive generalized fibrosis of the bone marrow
29
What is a consequence of myelofibrosis on blood cell production?
Decreased production of RBC and platelets
30
What percentage of myelofibrosis patients transform into acute myeloid leukemia (AML)?
10-20%
31
What is the median survival for patients with myelofibrosis?
Around 5 years
32
What are common causes of death in myelofibrosis?
["Heart failure", "Infections", "Leukemic transformation"]
33
What are the clinical signs of myelofibrosis?
["Anemia of insidious onset in older individuals", "Massive splenomegaly", "Hypermetabolic symptoms (weight loss, fever, night sweats)", "Bleeding problems"]
34
What laboratory findings are common in myelofibrosis?
["Anemia", "High WCC and platelets at presentation", "Leucopenia and thrombocytopenia later", "Circulating immature myeloid and erythroid cells", "Tear-drop poikilocytes"]
35
What does a bone marrow biopsy show in myelofibrosis?
Fibrotic hypercellular with increased reticulin fibers
36
What is a treatment option for anemia in myelofibrosis?
Folic acid and B12 supplement
37
What is Ruxolitinib used for in myelofibrosis?
To reduce spleen size and improve symptoms
38
What is the role of splenectomy in myelofibrosis?
Considered for patients with severe symptomatic disease
39
What is the focus of therapy for primary myelofibrosis (PMF)?
Reducing the effects of anemia and splenomegaly
40
What is Chronic Myeloid Leukemia (CML)?
A malignant disorder of the haemopoietic stem cell usually associated with the presence of the Philadelphia chromosome (Ph) ## Footnote The Philadelphia chromosome is a shortened chromosome 22 resulting from a translocation between chromosomes 9 and 22.
41
What are the common clinical manifestations of CML?
* Asymptomatic (discovered accidentally) * Anemic manifestations * Sweating * Fever * Weight loss * Abdominal discomfort due to splenic enlargement * Hepatomegaly * Pallor and splenomegaly ## Footnote Over 75% of patients may have splenomegaly.
42
What percentage of CML patients present asymptomatically?
20% ## Footnote 90% present in chronic phase and 10% are incidental findings during unrelated blood tests.
43
What are the laboratory diagnosis features of CML in the chronic phase?
* CBC shows leucocytosis * Bone marrow aspirates show profound hypercellularity * Philadelphia chromosome present in all myeloid cells ## Footnote Basophilia is considered a bad sign.
44
What is the Sokal score used for?
It is a prognostic score for assessing the risk in CML patients.
45
What defines the accelerated phase of CML?
* Rising leucocyte count during adequate treatment * Platelet count <100x10^9/l * Hemoglobin <8.0g/dl * >10% blasts in peripheral blood * >20% basophils and eosinophils in peripheral blood
46
What characterizes the blastic phase of CML?
>30% blasts and promyelocytes in peripheral blood or marrow ## Footnote Normal marrow erythropoiesis and megakaryopoiesis are reduced.
47
What is the first-line treatment for CML?
Imatinib mesylate (400 mg/day) ## Footnote It inhibits tyrosine kinase activity.
48
What are some adverse effects of Imatinib?
* Nausea * Fluid retention * Diarrhea * Muscle cramp * Skin rashes
49
What is leukapheresis used for in CML?
To control blood counts in chronic phase of CML and used in leukocytosis-related complications.
50
What are the two etiologic categories of Myelodysplastic Syndromes (MDS)?
* De Novo * Therapy related ## Footnote De Novo is associated with benzene exposure, cigarette smoking, viruses, and Fanconi’s anemia; Therapy related is associated with alkylating agent chemotherapy and radiation.
51
What are the clinical symptoms of MDS?
* Ecchymoses * Fatigue * Pallor * Abnormal bleeding * Infection
52
What are the prognostic groups for MDS based on survival?
* Good group: Refractory anemia (RA), Refractory anemia with ringed sideroblasts (RARS), 5q- syndrome * Bad group: Refractory anemia with excess blasts (RAEB), Refractory cytopenia with multilineage dysplasia (RCMD)
53
How are patients categorized based on treatment intensity in MDS?
* High intensity: requiring hospitalization, intensive combination chemotherapy and HSCT * Low intensity: outpatient-type treatments, hematopoietic growth factors, differentiation-inducing agents, and low intensity chemotherapy.
54
What is the primary demographic for Myelodysplastic Syndromes?
Occur primarily in older patients, most commonly >70 years.
55
What is the difference between CML and AML in terms of pathophysiology?
CML involves increased rate of proliferation and normal cellular differentiation, while AML involves malignant transformation due to genetic mutations leading to accumulation of blast cells.
56
What is the significance of the Philadelphia chromosome in CML?
It is a genetic marker that indicates the presence of BCR-ABL gene rearrangement.
57
Fill in the blank: Chronic Myeloid Leukemia is characterized by the presence of the _______.
Philadelphia chromosome

Medicine II (5 decks)

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