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Questions and Answers
What is one of the major criteria for diagnosing polycythemia vera?
Which of the following is considered a minor criterion for polycythemia vera?
What is required to diagnose post-polycythemia vera myelofibrosis?
In the risk stratification model for polycythemia vera, what age is associated with the highest risk score?
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Which factor contributes 2 points in the MIPSS-PV risk stratification model?
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What is clonal hematopoiesis?
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Which mutation is commonly associated with chronic neutrophilic leukemia?
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What characterizes age-related clonal hematopoiesis (ARCH)?
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What condition involves a sustained and unexplained cytopenia without detectable driver mutations?
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What is a common clinical presentation of chronic myeloid leukemia?
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Which factor often influences the prognosis of chronic neutrophilic leukemia?
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What features characterize polycythemia vera?
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What is associated with chronic eosinophilic leukemia?
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What signifies clonal hematopoiesis of indeterminate potential (CHIP)?
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What is a common finding in the blood smear of someone with chronic neutrophilic leukemia?
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Study Notes
Myeloid Precursor Lesions
- Clonal hematopoiesis (CH) involves clonal growth of hematopoietic cells originating from affected stem or progenitor cells, irrespective of the underlying cause.
- Age-related clonal hematopoiesis (ARCH) is the presence of clonal hematopoiesis detectable in peripheral blood (PB) or bone marrow (BM) associated with aging, without diagnosis of hematological disorders.
- Clonal hematopoiesis of indeterminate potential (CHIP) is characterized by the detection of somatic mutations in myeloid malignancy genes at a variant allele frequency (VAF) ≥ 2%, without any diagnosed hematologic disorder.
- Clonal cytopenia of undetermined significance (CCUS) features persistent unexplained cytopenias that do not meet criteria for defined myeloid neoplasms.
- Idiopathic cytopenia of unknown significance (ICUS) refers to sustained unexplained cytopenia without identifiable driver mutations associated with blood cancers.
Myeloproliferative Neoplasms
- Chronic Myeloid Leukemia (CML) is marked by splenomegaly, leukocytosis, and a myelocyte bulge with ≥ 20% myeloid blasts or > 5% lymphoid blasts present in the blood.
- Chronic Neutrophilic Leukemia (CNL) may exhibit a myeloid-to-erythroid (M:E) ratio of over 20:1 without fibrosis or dysplasia; commonly involves CSF3RT6181 mutation alongside ASXL1, TET2, or DNMT3A mutations.
- Survival rate for Chronic Neutrophilic Leukemia averages around 2 years, with patients dying from infections, bleeding, or organ failure related to the disease.
- Positive prognostic indicators include the presence of STAT5B p.N642H as the sole mutation, while its combination with other mutations lowers survival (median survival of 14 months).
- Chronic Eosinophilic Leukemia is diagnosed with eosinophils in PB exceeding 1.5 × 10^9/L on at least two occasions and can involve abnormal T-cell populations reflecting lymphocytic variant Hypereosinophilia Syndrome (HES).
Polycythemia Vera (PV)
- Characterized by erythrocytosis, often alongside leukocytosis and thrombocytosis, typically associated with activating JAK2 mutations.
- Diagnostic criteria include meeting either all three major criteria or the first two major plus one minor criterion:
- Major: Elevation of hemoglobin (≥ 16.5 g/dL in men, ≥ 16.0 g/dL in women) or hematocrit (≥ 49% in men, ≥ 48% in women); trilineage hypercellularity in bone marrow with marked erythroid and myeloid proliferation; presence of JAK2 p.V617F or exon 12 mutation.
- Minor: Subnormal serum erythropoietin levels.
- Criteria for diagnosing post-polycythemia vera myelofibrosis include prior PV diagnosis and grade 2–3 bone marrow fibrosis.
- Additional diagnostic indicators may include anemia, leukoerythroblastosis, splenomegaly, and constitutional symptoms such as significant weight loss or fever.
PV Risk Factors
- Risk stratification includes age, venous thrombosis, and leukocytosis to predict survival outcomes, with the Mayo Clinic identifying significant associations:
- Age ≥ 67 years: 5 points, age 57–66 years: 2 points, presence of venous thrombosis: 1 point, leukocytosis (≥ 15 × 10^9/L): 1 point.
- Survival outcomes based on stratification:
- High-risk (≥ 3 points): median survival around 9.6 years.
- Intermediate risk (1-2 points): median survival approximately 18 years.
- Low risk (0 points): median survival extending to 28 years.
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Description
Explore the nuances of myeloid precursor lesions and clonal hematopoiesis in this quiz based on Chapter 2. Delve into topics such as age-related clonal hematopoiesis and the implications of clonal outgrowth of hematopoietic cells. Test your knowledge on these essential concepts in hematology.
