BLOCK 6 - EXAM 2 POST QUIZ CONTENT| TOP SCORES
MADE SIMPLE | TRUSTED TEST SOLUTIONS!
Myeloproliferative Neoplasms Answer: Clonal hematopoietic stem cell disorders
caused by mutated tyrosine kinases or other aberrations in signalling pathways
- Mutations do not impair differentiation → increased production of one or more mature
blood elements
Clonal hematopoietic stem cell disorders caused by mutated tyrosine kinases or other
aberrations in signalling pathways
- Mutations do not impair differentiation → increased production of one or more mature
blood elements Answer: Myeloproliferative Neoplasms
What neoplasm is caused by mutations that do not impair WBC differentiation →
increased production of one or more mature blood elements Answer: Myeloproliferative
Neoplasms
Types of Myeloproliferative Neoplasms Answer: - Chronic myeloid leukemia
- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
- Chronic myeloid leukemia
- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
What type of neoplasms? Answer: Myeloproliferative Neoplasms
Chronic myeloid leukemia mutation Answer: BCR-ABL fusion gene [t(9;22)]
BCR-ABL fusion gene mutation [t(9;22)] Answer: Chronic myeloid leukemia
Polycythemia vera mutation Answer: JAK 2 mutation
What neoplasms are caused by JAK 2 mutations? Answer: - Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
1
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,- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
are caused by what mutations? Answer: JAK 2 mutation
Essential thrombocythemia mutation Answer: JAK 2 mutation
Primary myelofibrosis mutation Answer: JAK 2 mutation
JAK 2 mutations Answer: - Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
Distinguished from other myeloproliferative neoplasms by presence of chimeric
BCRABL gene Answer: Chronic Myeloid Leukemia
>90% of cases have a reciprocal (9;22)(q34;q11) translocation (the so-called
Philadelphia chromosome) Answer: Chronic Myeloid Leukemia
Chronic Myeloid Leukemia treatment Answer: BCR-ABL tyrosine kinase inhibitors
(imatinib)
BCR-ABL tyrosine kinase inhibitors (imatinib) Answer: Treatment for Chronic Myeloid
Leukemia
Chronic Myeloid Leukemia clinical manifestations Answer: Insidious onset
- Mild to moderate anemia and hypermetabolism due to increased cell turnover →
fatigability, weakness, weight loss, and anorexia
- First symptom may be abdominal discomfort or pain from splenomegaly
Chronic Myeloid Leukemia morphology Answer: - Leukocytosis, often >100,000
cells/mm3: Neutrophils, band forms, metamyelocytes, myelocytes, eosinophils,
basophils
- Splenomegaly
What leukemia causes leukocytosis, often >100,000 cells/mm3: Neutrophils, band
forms, metamyelocytes, myelocytes, eosinophils, basophils
- Also causes Splenomegaly Answer: Chronic Myeloid Leukemia
BCR-ABL tyrosine kinase inhibitors are used to treat? Answer: Chronic Myeloid
Leukemia
2
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,Polycythemia Vera Answer: Increased marrow production of erythrocytes,
granulocytes, and platelets (panmyelosis)
- ↑Erythrocytes (polycythemia) responsible for most of clinical symptoms
- Mutations in JAK2
Increased marrow production of erythrocytes, granulocytes, and platelets (panmyelosis)
- ↑Erythrocytes (polycythemia) responsible for most of clinical symptoms
- Mutations in JAK2 Answer: Polycythemia Vera
Polycythemia Vera pathogenesis Answer: Mutations in JAK2 → constitutive activation
of JAK2 tyrosine kinase → ↑cell proliferation
Mutations in JAK2 → constitutive activation of JAK2 tyrosine kinase → ↑cell proliferation
Answer: - Polycythemia Vera
- Essential thrombocythemia
- Primary myelofibrosis
Polycythemia Vera clinical manifestations Answer: - Plethora and cyanosis due to
stagnation and deoxygenation of blood in peripheral vessel
- Itchiness, especially after a warm bath or shower (aquagenic pruritus)
- Severe burning pain and red-blue discoloration of hands (erythromelalgia)
- Episodic blood clots in blood vessels of extremities
- Plethora and cyanosis due to stagnation and deoxygenation of blood in peripheral
vessel
- Itchiness, especially after a warm bath or shower (aquagenic pruritus)
- Severe burning pain and red-blue discoloration of hands (erythromelalgia)
- Episodic blood clots in blood vessels of extremities
- Splenomegaly in ≈70% of patients
Clinical manifestations of? Answer: Polycythemia Vera
Driving force behind the erythrocytosis in Polycythemia Vera Answer: JAK2 mutation
(not EPO)
- EPO levels will be low
EPO levels in polycythemia Vera Answer: Low
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, - JAK2 mutation (not EPO) is the driving force behind the erythrocytosis in P. vera
Spent phase of Polycythemia Vera Answer: Myelofibrosis and extensive extramedullary
hematopoiesis
Myelofibrosis and extensive extramedullary hematopoiesis are characteristics of what
phase of Polycythemia Vera Answer: Spent phase of Polycythemia Vera
A small percentage of Polycythemia Vera can transform into? Answer: Acute Myeloid
Leukemia
Polycythemia Vera morphological findings Answer: - Hypercellular bone marrow
- Spent phase: Extensive marrow fibrosis
- Hypercellular bone marrow
- Spent phase: Extensive marrow fibrosis
Morphological findings of? Answer: Polycythemia Vera
Essential Thrombocythemia Answer: Blood disorder characterized by the
overproduction of platelets by megakaryocytes in the absence of another cause
- JAK2 mutation
- Separated from polycythemia vera by absence of polycythemia
Megakaryocytic hyperplasia is seen in what myeloproliferative neoplasm? Answer:
Essential Thrombocythemia
Blood disorder characterized by the overproduction of platelets by megakaryocytes in
the absence of another cause
- JAK2 mutation
- Separated from polycythemia vera by absence of polycythemia Answer: Essential
Thrombocythemia
What myeloproliferative neoplasm is separated from polycythemia vera by absence of
polycythemia Answer: Essential Thrombocythemia
Essential Thrombocythemia clinical manifestations Answer: Vascular occlusion
(thrombosis) or hemorrhage
Vascular occlusion (thrombosis) or hemorrhage is a common finding of what
myeloproliferative neoplasm? Answer: Essential Thrombocythemia
Essential Thrombocythemia morphological findings Answer: -Bone marrow
4
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MADE SIMPLE | TRUSTED TEST SOLUTIONS!
Myeloproliferative Neoplasms Answer: Clonal hematopoietic stem cell disorders
caused by mutated tyrosine kinases or other aberrations in signalling pathways
- Mutations do not impair differentiation → increased production of one or more mature
blood elements
Clonal hematopoietic stem cell disorders caused by mutated tyrosine kinases or other
aberrations in signalling pathways
- Mutations do not impair differentiation → increased production of one or more mature
blood elements Answer: Myeloproliferative Neoplasms
What neoplasm is caused by mutations that do not impair WBC differentiation →
increased production of one or more mature blood elements Answer: Myeloproliferative
Neoplasms
Types of Myeloproliferative Neoplasms Answer: - Chronic myeloid leukemia
- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
- Chronic myeloid leukemia
- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
What type of neoplasms? Answer: Myeloproliferative Neoplasms
Chronic myeloid leukemia mutation Answer: BCR-ABL fusion gene [t(9;22)]
BCR-ABL fusion gene mutation [t(9;22)] Answer: Chronic myeloid leukemia
Polycythemia vera mutation Answer: JAK 2 mutation
What neoplasms are caused by JAK 2 mutations? Answer: - Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
1
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,- Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
are caused by what mutations? Answer: JAK 2 mutation
Essential thrombocythemia mutation Answer: JAK 2 mutation
Primary myelofibrosis mutation Answer: JAK 2 mutation
JAK 2 mutations Answer: - Polycythemia vera
- Essential thrombocythemia
- Primary myelofibrosis
Distinguished from other myeloproliferative neoplasms by presence of chimeric
BCRABL gene Answer: Chronic Myeloid Leukemia
>90% of cases have a reciprocal (9;22)(q34;q11) translocation (the so-called
Philadelphia chromosome) Answer: Chronic Myeloid Leukemia
Chronic Myeloid Leukemia treatment Answer: BCR-ABL tyrosine kinase inhibitors
(imatinib)
BCR-ABL tyrosine kinase inhibitors (imatinib) Answer: Treatment for Chronic Myeloid
Leukemia
Chronic Myeloid Leukemia clinical manifestations Answer: Insidious onset
- Mild to moderate anemia and hypermetabolism due to increased cell turnover →
fatigability, weakness, weight loss, and anorexia
- First symptom may be abdominal discomfort or pain from splenomegaly
Chronic Myeloid Leukemia morphology Answer: - Leukocytosis, often >100,000
cells/mm3: Neutrophils, band forms, metamyelocytes, myelocytes, eosinophils,
basophils
- Splenomegaly
What leukemia causes leukocytosis, often >100,000 cells/mm3: Neutrophils, band
forms, metamyelocytes, myelocytes, eosinophils, basophils
- Also causes Splenomegaly Answer: Chronic Myeloid Leukemia
BCR-ABL tyrosine kinase inhibitors are used to treat? Answer: Chronic Myeloid
Leukemia
2
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,Polycythemia Vera Answer: Increased marrow production of erythrocytes,
granulocytes, and platelets (panmyelosis)
- ↑Erythrocytes (polycythemia) responsible for most of clinical symptoms
- Mutations in JAK2
Increased marrow production of erythrocytes, granulocytes, and platelets (panmyelosis)
- ↑Erythrocytes (polycythemia) responsible for most of clinical symptoms
- Mutations in JAK2 Answer: Polycythemia Vera
Polycythemia Vera pathogenesis Answer: Mutations in JAK2 → constitutive activation
of JAK2 tyrosine kinase → ↑cell proliferation
Mutations in JAK2 → constitutive activation of JAK2 tyrosine kinase → ↑cell proliferation
Answer: - Polycythemia Vera
- Essential thrombocythemia
- Primary myelofibrosis
Polycythemia Vera clinical manifestations Answer: - Plethora and cyanosis due to
stagnation and deoxygenation of blood in peripheral vessel
- Itchiness, especially after a warm bath or shower (aquagenic pruritus)
- Severe burning pain and red-blue discoloration of hands (erythromelalgia)
- Episodic blood clots in blood vessels of extremities
- Plethora and cyanosis due to stagnation and deoxygenation of blood in peripheral
vessel
- Itchiness, especially after a warm bath or shower (aquagenic pruritus)
- Severe burning pain and red-blue discoloration of hands (erythromelalgia)
- Episodic blood clots in blood vessels of extremities
- Splenomegaly in ≈70% of patients
Clinical manifestations of? Answer: Polycythemia Vera
Driving force behind the erythrocytosis in Polycythemia Vera Answer: JAK2 mutation
(not EPO)
- EPO levels will be low
EPO levels in polycythemia Vera Answer: Low
3
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, - JAK2 mutation (not EPO) is the driving force behind the erythrocytosis in P. vera
Spent phase of Polycythemia Vera Answer: Myelofibrosis and extensive extramedullary
hematopoiesis
Myelofibrosis and extensive extramedullary hematopoiesis are characteristics of what
phase of Polycythemia Vera Answer: Spent phase of Polycythemia Vera
A small percentage of Polycythemia Vera can transform into? Answer: Acute Myeloid
Leukemia
Polycythemia Vera morphological findings Answer: - Hypercellular bone marrow
- Spent phase: Extensive marrow fibrosis
- Hypercellular bone marrow
- Spent phase: Extensive marrow fibrosis
Morphological findings of? Answer: Polycythemia Vera
Essential Thrombocythemia Answer: Blood disorder characterized by the
overproduction of platelets by megakaryocytes in the absence of another cause
- JAK2 mutation
- Separated from polycythemia vera by absence of polycythemia
Megakaryocytic hyperplasia is seen in what myeloproliferative neoplasm? Answer:
Essential Thrombocythemia
Blood disorder characterized by the overproduction of platelets by megakaryocytes in
the absence of another cause
- JAK2 mutation
- Separated from polycythemia vera by absence of polycythemia Answer: Essential
Thrombocythemia
What myeloproliferative neoplasm is separated from polycythemia vera by absence of
polycythemia Answer: Essential Thrombocythemia
Essential Thrombocythemia clinical manifestations Answer: Vascular occlusion
(thrombosis) or hemorrhage
Vascular occlusion (thrombosis) or hemorrhage is a common finding of what
myeloproliferative neoplasm? Answer: Essential Thrombocythemia
Essential Thrombocythemia morphological findings Answer: -Bone marrow
4
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