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56 Cards in this Set
- Front
- Back
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Polycythemia vera
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- increase in red cell mass and total blood volume
- generalized hyperplasia of the bone marrow w/ subsequent increases in the erythroid, leukocytic, and megakaryocytic series |
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Polycythemia vera prognosis and complications
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- chronic
- 10-20 year life expectancy |
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Pathophysiology of Polycythemia vera
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- overexpansion of red cell mass, hypervolemia, and splenomegalic red cell pooling
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Signs and Symptoms of Polycythemia vera
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- plethora (hypervolemia)
- splenomegaly - neurologic - arterial/venous (stroke) |
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Lab data of polycythemia vera
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- increased RBC, Hct, Hgb
- normal RBC indices - decreased erythropoietin - increase RDW - increase visocosity - no hemosiderin - increase LAP |
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Other reasons for increased RBC
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- smoking
- tumors |
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Diagnosis of polycythemia vera
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- increased Hct above 50% in the absence of dehydration
- oxygen saturation normal |
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Erythrocytes
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extremely increased
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Leukocytes
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increased
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Platelets
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moderately increased
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Leukocyte alkaline phosphate score
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extremely increased
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Total blood volume
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increased
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Erythrocyte sedimentation rate
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decreased
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Serum iron
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decreased
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erythropoietin
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decreased
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Treatment
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- phlebotomy to decreased iron and red cell production
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Secondary polycythemia
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- loss of body fluid
- nothing wrong with hematopoiesis - increases Hct |
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Idiopathic Myelofibrosis
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- clonal disorder of the multipotential progenitor cell compartment
- blood/marrow barrier disrupted, stem cells escape to blood - exramedullary hematopoiesis and marrow fibrosis |
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Myelofibrosis
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- the clinical manisfestation
- occurs secondarily and is not a component of the abnormal clonal proliferation - fibrosis ensues from proliferation of fibroblasts and increased collagen production in reaction to the abnormal clone of hematopoietic cells * result of a product by megakaryocytes |
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Signs and symptoms of myelofibrosis
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- anemia, splenomegaly, and marrow fibrosis
- teardrop cells, nucleated RBCs - decrease RBC - sclerosis of the bone - dysmegakaryocytopoiesis leading to increase of defective plts |
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Treatment of myelofibrosis
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- transfusion of packed red blood cells
- splenectomy - interferon alpha |
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Essential Thrombocythemia
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- thrombocytosis
- marked increase in platelets - >1000x10^9/L - least common MPD |
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Signs and symptoms of ET
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- thrmoboembolism
- bleeding problems - neurologic |
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Lab data of Essential Thrombocythemia
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- increased platelet count
- hypochromia, microcytic |
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Treatment of ET
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- hemapheresis: remove a component
- antiplatelet drug |
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Myeloproliferative disorders are characterized by all of the following except:
A.) clonal disorders B.) they may evolve into acute leukemia C.) initial increase of immature cells D.) increased production of mature cells |
C.) initial increase of immature cells
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In chronic myelogenous leukemia, the total leukocyte (WBC) count is:
A.) extremely increased B.) slightly increased C.) extremely variable D.) usually normal |
A.) extremely increased
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Idiopathic myelofibrosis differes from other types of chronic MPD in which of the following ways?
A.) Ph1 chromosome is present B.) Marrow fibrosis is greatly increased C.) LAP score is increased D.) Platelet count is increased |
B.) Marrow fibrosis is greatly increased
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Which of the following is a remarkable characteristic of polycythemia vera compared with other types of chronic MPD?
A.) extremely increased erythrocyte mass B.) extremely increased leukocyte count C.) extremely increased platelet count D.) teardrop-shaped erythrocytes |
A.) extremely increased erythrocyte mass
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Which of the following is a predominant feature of essential thrombocythemia compared with other types of chronic MPD?
A.) Variable number of platelets B.) Moderately increased number of platelets C.) Extremely increased number of platelets D.) increased marrow fibrosis |
C.) extremely increased number of platelets
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In MPD, the test results of disorders of hemostasis and coagulation that are most likely to be abnormal are:
A.) Decreased platelet count, increased activated partial thromboplastin time, and increased factor V level B.) Increased activated partial thromboplastin time, decreased factor V level, and increased concentration of antithrombin III in many C.) Decreased activated partial thromboplastin time, decreased factor V level, and increased concentration of D-dimers D.) Decreased concentration of D-dimers, decreased concentration of antithrombin III, and increased concentration of plasmin-alpha-2 plasmin inhibitor complex |
B.) Increased activated partial thromboplastin time, decreased factor V level, and increased concentration of antithrombin III in many
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Interferon alfa has been shown to:
A.) stimulate trilineage cell proliferation B.) suppress proliferation of hematopoietic progenitor cells C.) subdue erythropoiesis only D.) suppress megakaryocytopoiesis only |
B.) suppress proliferation of hematopoietic progenitor cells
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A leukemia of long duration that affects the neutrophilic granulocytes is referred to as:
A.) acute lymphoblastic leukemia B.) acute myelogenous leukemia C.) acute monocytic leukemia D.) chronic meylogenous leukemia |
D.) chronic myelogenous leukemia
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The alkaline phosphatase cytochemical staining reaction is used to differentiate between:
A.) chronic lymphoblastic leukemia and acute myelogenous leukemia B.) acute lymphoblastic leukemia and acute myelogenous leukemia C.) chronic myelogenous leukemia and severe bacterial infections D.) leukemoid reactions and severe bacterial infections |
C.) chronic myelogenous leukemia and severe bacterial infections
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Patients with the initial phase of CML are prone to:
A.) weight gain, edema, and fatigue B.) edema, anemia, and splenic infarction C.) low-grade fevers, night sweats, and splenic infarction D.) prominent lymphadenopathy and night sweats |
C.) low-grade fevers, night sweats, and splenic infarction
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The total leukocyte count in CML usually is_____x10^9/L
A.) normal B.) <25 C.) <50 D.) >50 |
D.) >50
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The Philadelphia chromosome is typically associated with:
A.) acute myelogenous leukemia B.) leukemoid reaction C.) acute lymphoblastic leukemia D.) chronic myelogenous leukemia |
D.) chronic myelogenous leukemia
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Patients with polycythemia vera suffer from:
A.) leukemic infiltration B.) bone marrow fibrosis C.) hypervolemia D.) anemia |
C.) hypervolemia
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In polycythemia vera, cytogenic results do not predict/provide:
A.) duration of the disease B.) consequences of myelosuppressive therapy C.) clues to hematological phenotype D.) evolution of the disease |
D.) evolution of the disease
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Hyperviscosity can produce:
A.) anemia B.) dizziness C.) hemorrhages D.) psychotic depression |
B.) dizziness
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The major criteria established by the National Polycythemia Vera Study Group for diagnosis includes all of the following except:
A.) increased red blood cell mass B.) arterial O2 saturation (greater than 92%) C.) leukocytosis D.) splenomegaly |
C.) leukocytosis
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Increased blood viscosity in patients with polycythemia vera can cause a dangerous condition of:
A.) hot flushes B.) shortness of breath C.) high RDW D.) vascular occlusion |
D.) vascular occlusion
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The level of erythropoietin in the urine is_____in patients with polycythemia vera compared with other kinds of polycythemia
A.) increased B.) the same C.) variable D.) decreased |
D.) decreased
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Patients with polycythemia vera demonstrate a(n)______of hemosiderin in the bone marrow.
A.) absence B.) normal amount C.) slightly increased amount D.) extremely increased amount |
A.) absence
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Treated patients with polycythemia vera have a ______life expectancy after diagnosis.
A.) 1 to 6 month B.) 6 to 12 month C.) 1 to 5 year D.) more than 10 years |
D.) more than 10 years
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The primary treatment for polycythemia vera is:
A.) therapeutic phlebotomy B.) myelosuppressive agents C.) radioactive phosphorus D.) low-dose busulfan |
A.) therapeutic phlebotomy
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Idopathic myelofibrosis is also called:
A.) essential thrombocythemia B.) chronic myelogenous leukemia C.) polycythemia vera D.) agnogenic myeloid metaplasia |
D.) agnogenic myeloid metaplasia
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The incidence of idiopathic myelofibrosis is known to increase after exposure to:
A.) sunshine B.) benzene C.) antibiotics D.) interferon |
B.) benzene
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The predominant clinical manifestation of idiopathic myelofibrosis is:
A.) anemia B.) splenomegaly C.) medullary fibrosis D.) all of the above |
D.) all of the above
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The most constant feature of idiopathic myelofibrosis is:
A.) dyserythropoiesis B.) dysleukopoiesis C.) dysmegakaryocytopoiesis D.) trilineage maturational disruption |
D.) trilineage maturational disruption
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A leukoerythroblastic picture includes all of the following except:
A.) teardrop-shaped erythrocytes B.) nucleated erythrocytes C.) immature lymphocytes D.) immature myeloid cells |
C.) immature lymphocytes
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The median survival time for patients with idiopathic myelofibrosis is approximatelyl_____years.
A.) 1 B.) 3 C.) 5 D.) 10 |
C.) 5
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The least common form of chronic MPD is:
A.) polycythemia vera B.) CML C.) idiopathic myelofibrosis D.) essential thrombocythemia |
D.) essential thrombocythemia
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A major criterion for the diagnosis of essential thrombocythemia is:
A.) absence of Ph1 chromosome B.) increased red blood cell mass C.) mild neutrophilia in peripheral blood D.) persistent increase of platelets in peripheral blood |
D.) persistent increase of platelets in peripheral blood
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The most common disorder in patients with essential thrombocythemia is:
A.) splenomegaly B.) thrombotic or bleeding problems C.) abnormal karyotype D.) anemia |
B.) thrombotic or bleeding problems
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The bone marrow architecture in essential thrombocythemia is similar to the architecture seen in:
A.) erythroid hyperplasia B.) leukocyte hyperplasia C.) chronic myelogenous leukemia D.) polycythemia |
D.) polycythemia
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