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Hematology II > Lecture 4 > Flashcards

Lecture 4 Flashcards

1
Q

What are myeloproliferative neoplasm

A

Are clonal hematopoetic disorders caused by genetic mutation in the hematopoetic stem cells

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2
Q

what are the characteristics of myeloproliferative neoplasms

A
  1. There is expansion, excessive production and over accumulation of erythrocytes, granules and plateletes.
  2. it is a genetic disorder
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3
Q

What are the main phases of myeloproliferative neoplasms (MPNs)?

A

MPNs are predominantly chronic, but they can have accelerated sub-acute or acute phases

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4
Q

How do clinical and morphologic findings impact the delineation between sub-acute and chronic phases in MPNs?

A

In certain patients, it is challenging to clearly differentiate between sub-acute and chronic phases based on clinical and morphologic features.

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5
Q

What are the four predominant disorders included in the WHO classification of MPNs?

A
  1. Chronic myelogenous leukemia (CML): A myeloproliferative disorder characterized by the Philadelphia chromosome (BCR-ABL1 fusion gene).
  2. Polycythemia vera (PV): Also known as Polycythemia rubra vera, PV involves an overproduction of red blood cells.
  3. Essential thrombocytopenia (ET): A disorder characterized by elevated platelet counts.
  4. Primary myelofibrosis (PMF): Also known as agnogenic myelofibrosis with myeloid metaplasia and chronic idiopathic myelofibrosis, PMF leads to bone marrow fibrosis and abnormal blood cell production.
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6
Q

What alternative names are given for Polycythemia vera and Primary myelofibrosis in medical terminology?

A
  1. Polycythemia vera is also known as Polycythemia rubra vera.
  2. Primary myelofibrosis is also referred to as agnogenic myelofibrosis with myeloid metaplasia and chronic idiopathic myelofibrosis.
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7
Q

How do clinical and morphologic findings contribute to the diagnosis of different phases of MPNs

A

Clinical and morphologic findings are essential but can sometimes make it difficult to clearly delineate between sub-acute and chronic phases in certain patients due to their similarities.

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8
Q

What distinguishes Chronic myelogenous leukemia (CML) from other MPNs?

A

CML is characterized by the presence of the Philadelphia chromosome (BCR-ABL1 fusion gene), which is not seen in other MPNs.

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9
Q

Which MPN is associated with bone marrow fibrosis and abnormal blood cell production?

A

Primary myelofibrosis (PMF) is associated with bone marrow fibrosis and abnormal blood cell production

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10
Q

differentiate CML and polycythemia vera

A

CML is overproduction of granulocytes while PV is overproduction of erythrocytes

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11
Q

What is the primary feature of Primary myelofibrosis (PMF) in terms of cell production?

A

PMF involves both overproduction of hematopoietic cells and stimulation of fibroblast production, leading to ineffective hematopoiesis and peripheral blood cytopenias

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12
Q

How is Essential thrombocytopenia (ET) characterized in MPNs

A

ET is characterized by increased megakaryocytopoiesis, resulting in peripheral blood thrombocytosis.

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13
Q

What are Myeloproliferative neoplasms (MPNs)?

A

MPNs are stable chronic disorders that can transform into more aggressive cellular growth phases, such as acute myeloid leukemia or acute lymphoblastic leukemia.

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14
Q

How do MPNs manifest in different phases

A

MPNs may initially present as stable chronic disorders but can transform to sub-acute and eventually to an aggressive cellular growth phase. They can also manifest a depleted cellular phase like bone marrow hypoplasia or exhibit symptoms of aggressive cellular expansion.

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15
Q

What is the significance of bone marrow hypoplasia in MPNs

A

Bone marrow hypoplasia is a manifestation of MPNs where there is a depleted cellular phase, affecting blood cell production.

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16
Q

How do familial MPNs differ from sporadic cases?

A

Familial MPNs occur in families where two or more members are affected, suggesting a genetic predisposition. Sporadic cases occur without a family history.

17
Q

What are the potential outcomes when MPNs transform into aggressive cellular growth phases?

A

MPNs can progress to acute myeloid leukemia or acute lymphoblastic leukemia, leading to more severe clinical manifestations.

18
Q

What clinical features characterize MPNs during the subacute and aggressive cellular expansion phases?

A

During the subacute phase, MPNs may exhibit clinical symptoms and morphologic patterns. In the aggressive phase, cellular expansion becomes more pronounced, impacting blood cell production.

19
Q

what are other less common MPN conditions that have been classified

A
  1. Chronic neutrophilic leukemia
  2. Chronic eosinophil leukemia
  3. mastocytosis
20
Q

What is Chronic Myelogenous Leukemia (CML)?

A

: CML is a myeloproliferative neoplasm (MPN) arising from a single genetic translocation in a pluripotential hematopoietic stem cell, resulting in a clonal overproduction of the myeloid cell line.

21
Q

How does CML affect the neutrophilic line

A

CML leads to a preponderance of immature cells in the neutrophilic line due to the overproduction of myeloid cells

22
Q

Describe the initial phase of CML

A

CML begins with a chronic clinical phase that is stable

23
Q

What happens during the accelerated phase of CML?

A

In 3 to 4 years, CML progresses to an accelerated phase characterized by increased cell proliferation

24
Q

What is the ultimate outcome if CML is left untreated

A

If left untreated, CML often terminates as an acute leukemia (blastic phase).

25
Q

What type of leukemia does CML transform into during the blastic phase?

A

CML can transform into acute leukemia, specifically acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL).

26
Q

What is the primary cellular abnormality in CML?

A

The clonal overproduction of myeloid cells due to a genetic translocation in pluripotential hematopoietic stem cells characterizes CML.

27
Q

What are the clinical features of Chronic Myelogenous Leukemia

A

The clinical features of CML, all secondary to massive pathologic accumulation of myeloid progenitor cells in bone marrow (BM), peripheral blood (PB), and extramedullary tissues, include:
Frequent infections
Anemia
Bleeding
Splenomegaly

28
Q

: How does CML affect the neutrophilic line?

A

CML results in a preponderance of immature cells in the neutrophilic line due to the clonal overproduction of myeloid progenitor cells.

29
Q

What is the significance of splenomegaly in CML?

A

Splenomegaly (enlarged spleen) is a common feature in CML due to the accumulation of abnormal cells in the spleen

30
Q

How does anemia manifest in patients with CML

A

Anemia occurs because of decreased red blood cell production and the presence of immature cells in the blood.

31
Q
A

Hematology II (19 decks)

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