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Lecture 20

Chronic Myeloproliferative Disorders: CML, MF, PV, ET

(T or F) Myeloproliferative disorders exhibit increased production of one or more peripheral blood lineage with dysplasia. False. All myeloproliferative disorders exhibit increased production of one or more peripheral blood lineages without dysplasia.
(T or F) All myeloproliferative disorders exhibit expansion of stem cells without block in maturation. True.
What is the molecular defect associated with all myeloproliferative disorders? Persistent activation of tyrosine kinases
What are the symptoms of Chronic Myelogenous Leukemia (CML)? (1) Fatigue (2) Weight loss (3) Abdominal pain or fullness (4) Easy bruising or bleeding
What are the physical signs of Chronic Myelogenous Leukemia (CML)? (1) Splenomegaly (2) Hepatomegaly (3) Fever, hypermetabolic state (4) Purpura
What are the characteristic leukocyte alkaline phosphatase levels in Chronic Myelogenous Leukemia (CML)? Decreased
What are the characteristic leukocyte uric acid levels in Chronic Myelogenous Leukemia (CML)? Increased from increased cell turnover
How is a reactive leukocytosis from infection distinguished from a leukocytosis in Chronic Myelogenous Leukemia (CML)? High leukocyte alkaline phosphatase (LAP) indicates a reactive process where as CML typically has decreased LAP levels.
What is the pathogenesis of Chronic Myelogenous Leukemia (CML)? Translocation of chromosome 9 and 22 moves bcr gene next to abl tryosine kinase gene, creating a fusion protein. Bcr-abl fusion protein results in a constitutive cytoplasmic tyrosine kianse activity, promoting cell proliferation and survival.
What are the phases of Chronic Myelogenous Leukemia (CML)? (1) Chronic phase (2) Accelerated phase (3) Blast phase
What type of acute leukemia does majority of Chronic Myelogenous Leukemia (CML) progress to? 85% of CML progresses to AML.
What is a possible curative option for Chronic Myelogenous Leukemia (CML)? Allogeneic bone marrow transplant
What is the mechanism of Gleevec? Gleevac is a Imatinib mesylate which is an inhibitor of bcr-abl tryosine kinase.
What is the initial treatment of Chronic Myelogenous Leukemia (CML)? Gleevec
What pharmacologic agents can be used to induce remission of Chronic Myelogenous Leukemia (CML)? (1) Gleevec (2) Interferon
What are the treatment options for Chronic Myelogenous Leukemia (CML)? (1) Bone marrow transplant (2) Gleevec (3) Interferon (4) Chemotherapy
What is the median age of diagnosis of Polycythemia vera (PV)? PV is a disease of older adults, with median age at diagnosis of 60 years.
What are the typical symptoms of Polycythemia vera (PV)? (1) Headaches (2) Dyspnea (3) Sweats (4) Dizziness (5) Pruritis
What are the typical physical signs of Polycythemia vera (PV)? (1) Splenomegaly (2) Hepatomegaly (3) Plethora (4) Hypertension
What are majority of the symtpoms in Polycythemia vera (PV) a result of? Symptoms experienced are often the result of increased viscosity due to the increased hematocrit.
What are the bone marrow biopsy findings of Polycythemia vera (PV)? (1) Hypercellular with erythroid hyperplasia (2) Decreased iron stores (3) Increased megakaryocytes
What are the diagnostic criteria for Polycythemia vera (PV)? Presence of 4 of the following: (1)Elevated red cell mass (2)Normal arterial oxygen saturation (3)Thrmbocytosis and leukocytosis (4)Marrow hypercelluarity with absent iron stores (5)Low erythropoietin level (6)Abnormal marrow proliferative capacity
What are causes of secondary erythrocytosis? (1) Hemoconcentration (2) Hypoxia (3) CO intoxification (4) Inappropriate erythropoietin production
Pathophysiology of Polycythemia vera (PV) JAK2 is a tyrosine kinase that is downstream of the Epo receptor. Mutant JAK2 V617F has constitutively active tyrosine kinase activity mimicking Epo signaling resulting in activation of transcription factors, stem cell expansion, megakaryopoiesis.
Treatment for Polycythemia vera (PV) (1) Phlebotomy (2) Hydroxyurea (3) Anagrelide (4) ASA or NSAID (5) others: 32P, busulfan, IFN-alpha, allopurinol, muatant JAK2 inhibitor
What level must the hematocrit be maintained in phlebotomy treatment of Polycythemia vera (PV)? Hct < 45%
What is the mechanism of Anagrelide? It is a specific inhibitor of megakaryocyte maturaiton to control thrmbocytosis.
What are the possible course of progressive Polycythemia vera (PV)? (1) Spent phase: post-polycythemic myelofibrosis (2) Transformation to AML
What is the incidence of transformation of PV to AML with phlebotomy treatment? 2%
What is the incidence of transformation of PV to AML with hydroxyurea treatment? 10%
What is the incidence of transformation of PV to AML with chlorambucil treatment? 13%
What is the incidence of transformation of PV to AML with 32P treatment? 10%
(T or F) Essential thrmbocytosis is at low risk of tranformation to leukemia or myelofibrosis. True.
What are the clinical features of essential thrombocytosis? Thrombotic complications (headaches, visual disturbances, erythromelalgia) (2) Hemorrhagic complications (superifical cutaneous or mucosal bleeding) (3) splenomegaly
What medications can exacerbate the hemorrhagic complications of essential thrombocytosis? ASA or NSAIDs
What is the typical CBC of essential thrombocytosis? Platelet count > 600,000 with normal hemoblogin and red cell mass
Bone marrow biopsy findings of essential thrmbocytosis Megakryocyte heyperplasia with presence of iron in the marrow
What conditions are associated with reactive thrombocytosis? (1) Infectious/inflammatory states (2) Surgical procedures or tissue damage (3) Malignancy (4) Fe deficiency, hemolytic anemia, acute blood loss (5) Post-splenectomy (6) Rebound post chemotherapy or ITP (7) Renal failure/nephrotic syndrome.
What is the indications for treatment of essential thrombocytosis? (1) symptomatic (2) age>60 (3) history of thrombosis (4) cardiovascular risk factors
What pharmacologic agents are used to treat vasomotor symptoms of essential thrombocytosis? ASA
(T or F) Treatment decreases the risk of miscarriage in the 1st trimester in essential thrombocytosis. False. Treatment doe snot appear to decrease risk of 1st trimester miscarriages.
What pharmacologic agents treat essential thrombocytosis? (1) Hydroxyurea (2) Angrelide (3) Interferon
What pharmacologic agent can be used to treat essential thrombocytosis in pregnant patients? Interferon
What are the symptoms of Chronic Idiopathic Myelofibrosis? (1) Fatigue (2) Weight loss (3) Night sweats
What are the physical signs of Chronic Idiopathic Myelofibrosis? (1) Splenomegly (2) Hepatomegaly
Microscopic findings of Chronic Idiopathic Myelofibrosis on peripheral blood smear Leukoerythroblastic blood smear: (1) Immature meyloid cells (2) Nucleated RBC (3) Teardrop cells
Bone marrow biopsy findings of Chronic Idiopathic Myelofibrosis (1) Extensive marrow fibrosis (2) Dysplastic megakaryocyte hyperplasia (3) Dilation of marrow siusoids with intravascular hematopoiesis (4) Osteosclerosis in advanced disease
Location of extramedullary hematopoiesis in Chronic Idiopathic Myelofibrosis (1) spleen (2) liver
Treatment for thrombocytopenia in Chronic Idiopathic Myelofibrosis Decrease spleen size: (1) chemotherapy (2) Radiation (3) Splenectomy
Treatment for anemia in Chronic Idiopathic Myelofibrosis Trial of erythropoietin, androgens. Transfusion as needed.
Created by: UVAPATH4