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Questions and Answers
What characterizes Myeloproliferative Neoplasms (MPN)?
What characterizes Myeloproliferative Neoplasms (MPN)?
- They are primarily inherited disorders.
- They result from viral infections.
- They involve abnormalities in pluripotent stem cells. (correct)
- They only affect the lymphatic system.
Which of the following is a major diagnostic entity of Myeloproliferative Neoplasms?
Which of the following is a major diagnostic entity of Myeloproliferative Neoplasms?
- Multiple myeloma
- Chronic myeloid leukemia (correct)
- Severe aplastic anemia
- Acute lymphoblastic leukemia
What type of cell proliferation is indicated in Chronic Myeloid Leukemia (CML)?
What type of cell proliferation is indicated in Chronic Myeloid Leukemia (CML)?
- Massive overproduction of normal-appearing granulocytes (correct)
- Overproduction of platelets without differentiation
- Increased erythropoietin production
- Massive overproduction of lymphocytes
What is a common feature of the mutated stem cells in Myeloproliferative Neoplasms?
What is a common feature of the mutated stem cells in Myeloproliferative Neoplasms?
Which of the following is NOT a type of Myeloproliferative Neoplasm?
Which of the following is NOT a type of Myeloproliferative Neoplasm?
What role do tyrosine kinases play in Myeloproliferative Neoplasms?
What role do tyrosine kinases play in Myeloproliferative Neoplasms?
Which pathophysiological change is commonly associated with Myeloproliferative Neoplasms?
Which pathophysiological change is commonly associated with Myeloproliferative Neoplasms?
What can be an observed result of erythropoietin activity in the context of Myeloproliferative Neoplasms?
What can be an observed result of erythropoietin activity in the context of Myeloproliferative Neoplasms?
What is a primary feature of Polycythemia Vera?
What is a primary feature of Polycythemia Vera?
What mutation is commonly associated with Polycythemia Vera?
What mutation is commonly associated with Polycythemia Vera?
Which of the following symptoms is least likely associated with Essential Thrombocythemia?
Which of the following symptoms is least likely associated with Essential Thrombocythemia?
In which disorder is extramedullary hematopoiesis most commonly observed?
In which disorder is extramedullary hematopoiesis most commonly observed?
How is the platelet count in Essential Thrombocythemia typically characterized?
How is the platelet count in Essential Thrombocythemia typically characterized?
Which laboratory finding is most common in Polycythemia Vera?
Which laboratory finding is most common in Polycythemia Vera?
What clinical manifestation is often seen in patients with Primary Myelofibrosis?
What clinical manifestation is often seen in patients with Primary Myelofibrosis?
Which of the following is typically NOT a clinical symptom of Polycythemia Vera?
Which of the following is typically NOT a clinical symptom of Polycythemia Vera?
What are the key features of fibrosis in peripheral blood?
What are the key features of fibrosis in peripheral blood?
Which of the following accurately describes Myelodysplastic Syndromes (MDS)?
Which of the following accurately describes Myelodysplastic Syndromes (MDS)?
What morphological feature is commonly seen in the bone marrow of patients with advanced fibrosis?
What morphological feature is commonly seen in the bone marrow of patients with advanced fibrosis?
Which mutations are commonly implicated in the pathogenesis of Myelodysplastic Syndromes?
Which mutations are commonly implicated in the pathogenesis of Myelodysplastic Syndromes?
What abnormal findings might be observed in the complete blood count (CBC) of a patient with Myelodysplastic Syndromes?
What abnormal findings might be observed in the complete blood count (CBC) of a patient with Myelodysplastic Syndromes?
What genetic mutation is commonly associated with Chronic Myeloid Leukemia (CML)?
What genetic mutation is commonly associated with Chronic Myeloid Leukemia (CML)?
Which symptom is least likely to be an initial clinical feature of Chronic Myeloid Leukemia (CML)?
Which symptom is least likely to be an initial clinical feature of Chronic Myeloid Leukemia (CML)?
During the chronic phase of CML, what is the typical expected leukocyte count?
During the chronic phase of CML, what is the typical expected leukocyte count?
What laboratory finding is indicative of advanced stages in Chronic Myeloid Leukemia (CML)?
What laboratory finding is indicative of advanced stages in Chronic Myeloid Leukemia (CML)?
What is the characteristic feature of the bone marrow in Chronic Myeloid Leukemia (CML)?
What is the characteristic feature of the bone marrow in Chronic Myeloid Leukemia (CML)?
Which of the following is NOT a phase of disease progression in Chronic Myeloid Leukemia (CML)?
Which of the following is NOT a phase of disease progression in Chronic Myeloid Leukemia (CML)?
What is a potential consequence of splenomegaly in individuals with Chronic Myeloid Leukemia (CML)?
What is a potential consequence of splenomegaly in individuals with Chronic Myeloid Leukemia (CML)?
During the blast crisis phase of CML, what percentage of blasts is typically observed in the bone marrow?
During the blast crisis phase of CML, what percentage of blasts is typically observed in the bone marrow?
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Study Notes
Myeloproliferative Neoplasms (MPN)
- Acquired malignant clonal disorders of pluripotent stem cells affecting one or more cell lines.
- Characterized by retained differentiation capacity and growth factor independence due to mutated tyrosine kinases.
- Major diagnostic entities include:
- Chronic Myeloid Leukemia (CML)
- Polycythemia Vera (PV)
- Essential Thrombocythemia (ET)
- Primary Myelofibrosis
Chronic Myeloid Leukemia (CML)
- Characterized by massive overproduction of normal-appearing granulocytes.
- Most common in adults aged 25-60, with peak in the 4th and 5th decades.
- 95% of cases exhibit t(9;22) (Philadelphia chromosome), creating BCR-ABL fusion gene with tyrosine kinase activity.
- Initial nonspecific symptoms include fatigue, anemia, and splenomegaly.
- Laboratory findings show elevated leukocyte count (often >100,000 cells/μL) with a predominance of neutrophils.
- Bone marrow is hypercellular with mild reticulin fibrosis; spleen shows extramedullary hematopoiesis.
Progression and Prognosis of CML
- Progresses through three phases:
- Chronic phase: lasts 1-5 years with mild symptoms.
- Accelerated phase: increasing anemia and new thrombocytopenia.
- Blast crisis: >20% blasts in bone marrow, defined as acute leukemia.
- Potential progression to extensive bone marrow fibrosis resembling primary myelofibrosis.
Polycythemia Vera (PV)
- Clonal disorder marked by excessive red cell mass due to activating JAK2 mutations.
- Symptoms are related to increased blood volume and viscosity, leading to thrombotic events.
- Presents insidiously in late middle age with signs including cyanosis, fatigue, and splenomegaly.
- Laboratory findings reveal high red cell counts and hematocrit (>55%).
Essential Thrombocythemia (ET)
- Clonal disorder characterized by sustained high platelet counts (>450,000/µL).
- Associated with risk of hemorrhage and thrombosis; commonly seen in adults over 60.
- Bone marrow shows hyperplasia of megakaryocytes with little granulopoiesis or erythropoiesis.
- Clinical symptoms include gastrointestinal bleeding and erythromelalgia (pain in palms and soles).
Primary Myelofibrosis
- Chronic myeloproliferative disorder with bone marrow fibrosis leading to cytopenia.
- Triggered by fibrogenic factors released from neoplastic megakaryocytes.
- Peripheral blood presents leukoerythroblastosis and bizarre-shaped erythrocytes.
- Bone marrow becomes hypocellular and diffusely fibrotic in advanced stages.
Myelodysplastic Syndromes (MDS)
- Preleukemic disorder characterized by ineffective hematopoiesis, leading to cytopenia in the peripheral blood.
- High risk of transformation to Acute Myeloid Leukemia (AML).
- Commonly affects individuals aged 50 to 70 and may be idiopathic or secondary to therapy.
- Pathogenesis involves mutations in transcription and RNA splicing factors, with notable abnormalities in chromosomes.
- Morphology includes abnormal hematopoietic precursors like megaloblastoid erythroid cells and ring sideroblasts.
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