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29 Cards in this Set
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An acquired Myeloproliferative Disorder
Exact etiology unknown Mutations in exon 12 or 14 of the JAK2 gene occur in almost 100% of patients with PV Characterized by overproduction of Red Blood Cells (Erythrocytosis) - Risk of a clot White Bloods Cells and Platelets may also be increased |
Polycythemia vera
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Polycythemia vera is associated with an increase risk of?
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Associated with increase risk of leukemia
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What are two important things to do with polycythemia vera?
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Control symptoms and RBC count
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What are the four types of polycythemia vera?
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Primary, Secondary, Relative (Spurious), Congenital PV Disorders
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Erythroid production is independent of erythropoietin (EPO)
Serum EPO level is low |
Primary
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Caused by oxygen deprivation resulting in increase in EPO stimulating RBC production
Smoking, Living at high altitudes, COPD, EPO secreting tumor |
Secondary
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High concentration of RBCs due to contracted plasma volume
Diuretic use, Dehydration, Vomiting, Burns |
Relative (Spurious)
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What is the most common form of polycythemia vera?
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Primary
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What can you use to help prevent complications with secondary polycythemia vera?
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O2
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This is found in the EPO receptor gene and can lead to perminant EPO production.
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Congenital polycythemia vera disorders.
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What are the clinical manifestations with polycythemia vera?
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Asymptomatic
Headache Dizziness, Lightheadedness Tinnitus Blurred Vision Epistaxis, Bleeding Gums Fatigue, Shortness of Breath Numbness, Tingling Arms, Legs, Hands, Feet Thrombolitic Event MI, PE, DVT, Stroke Pruritus Aquagenic Pruritus Facial Plethora Splenomegaly Hepatomegaly |
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This is a clinical manifestation of polycythemia vera, and it is noticed in the peripheral extremities?
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Numbness, tingling
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This is a clinical manifestation of polycythemia vera, and it can sometimes be the first presentation?
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Thrombotic events - MI, PE, DVT, Stroke
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This is a clinical manifestation of polycythemia vera, it can be much worse when coming in contact with water?
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Pruritus - Aquagenic Pruritus
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This is a clinical manifestation of polycythemia vera, it can appear red due to increased RBC accumulation?
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Facial plethora
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What are the diagnostics used with polycythemia vera?
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Complete Blood Count
Normal RBC morphology Normal Oxygen saturation Erythropoietin Assay Granulocyte DNA for JAK2 Bone Marrow Biopsy |
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Part of the diagnostics of polycythemia vera:
Elevated Hematocrit Males >52%, Females >48% Elevated Hemoglobin Normal to high WBC and platelets |
Complete Blood Count
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Part of the diagnostics of polycythemia vera:
Low in Primary - Due to mutation High in Secondary - Due to decreased 02 |
Erythropoietin Assay
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Part of the diagnostics of polycythemia vera:
Hypercellular with Panhyperplasia |
Bone Marrow Biopsy - Not needed for diagnosis
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This is a TX for polycythemia vera:
Used to reduce the Hematocrit <45% in men, <40% in females Approximately 500mL of blood is removed weekly until appropriate hematocrit level is achieved Decreasing the hematocrit has been shown to reduce the incidence of thrombotic complications May cause secondary iron deficiency |
Phlebotomy
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Patients are recommended to take what every day to prevent thrombosis in polycythemia vera?
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Baby ASA
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Treatment for polycythemia vera:
Hydroxyurea Reduces RBCs, WBCs & Platelets Interferon Alpha Weekly subcutaneous dose Reduces RBCs, Splenomegaly & Pruritus Anagrelide Platelet reduction |
Cytoreductive therapy
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This may lead to an increased risk of leukemia if done on a regular basis in the TX of polycythemia vera?
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Cytoreductive therapy
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This is a part of the polycythemia vera TX and is a part of cytoreductive therapy. It may cause patient to develop influenza like syndrome.
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Interferon Alpha
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Psychosis may develop as a side effect to this polycythemia vera treatment?
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Cytoreductive therapy
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How do you TX the pruritus associated with polycythemia vera?
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Add bicarbonate or starch to cool bath water
Antihistamines Serotonin Reuptake Inhibitors Fluoxetine (Prozac) , Paroxetine (Paxil) Topical Capsaicin Phototherapy |
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Majority of morbidity and mortality from PV is related to
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Arterial Thrombosis
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PV may progress to
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myelofibrosis or chronic myeloid leukemia
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In 5% of cases, PV progresses to ? which is typically refractory to therapy
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acute myeloid leukemia
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