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Questions and Answers
What is the primary genetic defect associated with polycythemia vera?
What is the primary genetic defect associated with polycythemia vera?
- Mutation in the TP53 gene
- Loss of function in the erythropoietin receptor
- Chromosomal translocation between chromosomes 9 and 22
- Clonal mutation in the JAK2 gene (correct)
Which of the following is NOT a common clinical feature of polycythemia vera?
Which of the following is NOT a common clinical feature of polycythemia vera?
- Hypertension
- Low erythropoietin levels
- Hyperactive bone marrow (correct)
- Massive splenomegaly
What risk is associated with myeloproliferative disorders, particularly chronic myelogenous leukemia?
What risk is associated with myeloproliferative disorders, particularly chronic myelogenous leukemia?
- Transformation to acute myelogenous leukemia (correct)
- Development of autoimmune disorders
- Deficiency in red blood cells
- Increased susceptibility to lymphomas
Which of the following best describes the general pathogenesis of myeloproliferative disorders?
Which of the following best describes the general pathogenesis of myeloproliferative disorders?
What type of blood disorder is characterized by reduced neutrophils, RBCs, and platelets?
What type of blood disorder is characterized by reduced neutrophils, RBCs, and platelets?
What is the most common type of cell found in hairy cell leukemia?
What is the most common type of cell found in hairy cell leukemia?
Which clinical feature is typically associated with hairy cell leukemia?
Which clinical feature is typically associated with hairy cell leukemia?
What is the prognosis for the aggressive form of leukemia mentioned?
What is the prognosis for the aggressive form of leukemia mentioned?
Which laboratory finding is associated with myelodysplastic syndromes?
Which laboratory finding is associated with myelodysplastic syndromes?
Which chromosomal abnormality is notably associated with certain leukemias?
Which chromosomal abnormality is notably associated with certain leukemias?
How does treatment for hairy cell leukemia typically proceed?
How does treatment for hairy cell leukemia typically proceed?
What is a common laboratory finding in early-stage hematologic diseases?
What is a common laboratory finding in early-stage hematologic diseases?
What is the treatment option that can potentially result in a cure for certain leukemias?
What is the treatment option that can potentially result in a cure for certain leukemias?
What is the primary cause of granulocytopenia in the context of leukopenia?
What is the primary cause of granulocytopenia in the context of leukopenia?
Which condition is associated with pancytopenia?
Which condition is associated with pancytopenia?
What is a common symptom of neutropenia?
What is a common symptom of neutropenia?
What age group is most commonly affected by acute lymphoblastic leukemia (ALL)?
What age group is most commonly affected by acute lymphoblastic leukemia (ALL)?
Which of the following is a cause of lymphocytopenia?
Which of the following is a cause of lymphocytopenia?
Which laboratory finding is characteristic of acute myeloblastic leukemia (AML)?
Which laboratory finding is characteristic of acute myeloblastic leukemia (AML)?
Which condition is known to cause eosinophilia?
Which condition is known to cause eosinophilia?
What is a typical clinical feature of chronic lymphocytic leukemia (CLL)?
What is a typical clinical feature of chronic lymphocytic leukemia (CLL)?
What distinguishes acute lymphoblastic leukemia (ALL) from acute myeloblastic leukemia (AML) in terms of prognosis?
What distinguishes acute lymphoblastic leukemia (ALL) from acute myeloblastic leukemia (AML) in terms of prognosis?
In chronic myelogenous leukemia (CML), what is a common age range for diagnosis?
In chronic myelogenous leukemia (CML), what is a common age range for diagnosis?
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Study Notes
Leukocytes
- Leukocytes are white blood cells found in the peripheral blood.
- They are primarily produced in the bone marrow.
- A single pluripotent stem cell gives rise to the myeloid and lymphoid precursor cells.
Leukopenia
- Leukopenia is a decrease in WBCs in the blood.
- Two types are:
- Granulocytopenia (neutropenia; agranulocytosis)
- Lymphocytopenia
- Causes of granulocytopenia include:
- Drug/alcohol effects
- Pancytopenia: decreased neutrophils, RBCs, and platelets. This can be caused by:
- Acute leukemia (total WBCs may be increased or decreased)
- Aplastic anemia
- Myelodysplasia/myelodysplastic syndromes
- Megaloblastic anemia
- Autoimmune destruction
- Symptoms of neutropenia include:
- Infection
- Fever
- Agranulocytic angina (ulcerating, necrotizing lesions of the gingiva and floor of mouth)
- Causes of lymphocytopenia include:
- Malnutrition
- Congenital immune deficiencies (DiGeorge syndrome)
- HIV-AIDS
- Radiation
- Corticosteroids
- Some severe infections, especially disseminated TB; serious COVID-19 (poor prognosis)
Leukocytosis
- Leukocytosis is an increase in WBCs in the blood.
- Two major types:
- Neutrophilia/Granulocytosis
- Lymphocytosis
- Causes of neutrophilia include:
- Pyogenic infections
- Sterile inflammation
- Chronic myelogenous leukemia and polycythemia vera
- Causes of lymphocytosis include:
- Viral infections (EBV, CMV, viral hepatitis)
- Chronic lymphocytic leukemia
- Eosinophilia is caused by:
- Asthma, allergies, and parasites
Acute Leukemias
- Acute lymphoblastic leukemia (ALL)
- Symptoms: fatigue, bleeding, bruising, fever, infection
- Peak age: <15 years
- Laboratory findings: anemia, thrombocytopenia, lymphoblasts present
- Subtypes: B cell type (most common), T cell type
- Cure: Remission 90%, 2/3+ cured
- Acute myeloblastic leukemia (AML)
- Symptoms: fatigue, bleeding, bruising, fever, infection
- Any age, median: 50 years old
- Laboratory findings: anemia, thrombocytopenia, myeloblasts present
- Cure: Chemotherapy cure less likely, bone marrow transplant cures
Chronic Leukemias
- Chronic lymphocytic leukemia (CLL)
- Age: Adults, usually >50 years, median: 60 years
- Most common adult leukemia
- Clinical: Initially asymptomatic, mild lymphadenopathy, later fatigue, weight loss, hematologic disease
- Labs: Lymphocytosis of small uniform lymphocytes, smudge cells
- Treatment: Therapy directed at lineage-specific CD (e.g., anti-CD20), palliative
- Chronic myelogenous leukemia (CML)
- Age: Any age peak: 25-60 years old
- Clinical: Fatigue, splenomegaly, fever, night sweats
- Labs: Neutrophilia with immature forms, eosinophils increased
- Specific labs: Philadelphia chromosome, t(9;22), (bcr;abl)
- Treatment: Tyrosine kinase inhibitors, bone marrow transplant may result in cure
Hairy Cell Leukemia
- Rare B-cell leukemia of middle-aged men
- Characterized by circulating "hairy" type cells, splenomegaly, pancytopenia
- Indolent (slowly progressive)
- Responds to therapy
Myelodysplastic Syndromes (MDS)
- Pancytopenia due to genetic abnormality of stem cells
- Anemia does not respond to treatment
- Risk for AML
- WBCs and platelets are morphologically abnormal and dysfunctional: risk for bleeding, infections
- Poor prognosis, median survival: 9-29 months
Myeloproliferative Disorders (MPDs)
- Exhibit increased hematopoiesis (bone marrow hypercellularity) and splenomegaly
- Risk for transformation to AML
- Arise due to clonal genetic abnormality of a myeloid progenitor cell
- Types:
- Chronic myelogenous leukemia (most common)
- Polycythemia vera (see below)
- Primary myelofibrosis and essential thrombocythemia
Polycythemia Vera (PV)
- Clonal mutation in JAK2 gene that causes increased production of red blood cells (RBCs)
- Independent of erythropoietin
- Absolute polycythemia with low erythropoietin
- Symptoms: plethoric complexion, hypertension, headache, increased risk of thrombosis/bleeding
- Splenomegaly, usually massive
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