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169 Cards in this Set
- Front
- Back
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where is 1/3 of platelet pool stored
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spleen
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vWF receptor
fibrinogen receptor |
vWF - GpIb
fibrinogen - GpIIb/IIIa |
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differentiate what's contained in dense granules and a-granules of platelets
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dense granules (ADP and calcium)
a-granules (vWF and fibrinogen) |
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differentiate where monocytes and macrophages are located
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monocyte - blood
macrophage - tissue |
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what activates macrophages
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IFN-gamma
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associated with large, kidney-shaped nucleus with "frosted-glass" cytoplasm
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monocyte
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associated with bilobate nucleus with large eosinphilic granules of uniform size
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eosinphils
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defends against helminthic infections via major basic protein
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eosinophils
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causes of eosinophilia
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NAACP:
neoplastic, asthma, allergic process, collagen vascular disease, parasites (invasive helminths) |
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mediates allergic reaction
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basophils
mast cells |
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can bind the Fc portion of IgE while mediating allergic reaction
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mast cell
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this drug prevents mast cell degranulation (used to treat asthma)
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cromyolyn sodium
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B cell markers
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CD19 and CD20
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associated with being a plasma cell neoplasm
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multiple myeloma
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where do B and T cells mature respectively - both are made in the bone marrow
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B - bone marrow
T -thymus |
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T cell markers
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CD3, CD4, CD8
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express MHC II and FcR on surface, main inducers of primary antibody response
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dendritic cells
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what type of Ig are anti-A/B vs. Anti-Rh
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Anti-A/B - IgM
Anti-Rh - IgG (can cross the placenta) |
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what causes hemolytic disease of the newborn
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Rh- mother exposed to Rh+ fetus, future pregnancies the mother will have anti-Rh IgG that crosses placenta and causes hemolysis
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thromboplastin = tissue factor = ?
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Factor VII
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deficiency of factor VIII
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hemophilia A
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deficiency of factor IX
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hemophilia B
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decreased synthesis of factors II, VII, IX, X, protein C and S
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vitamin K deficiency
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inhibits thrombin (II) and factors IX, X, XI, and XII
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antithrombin
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effects of bradykinin
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vasodilation
increases permeability pain |
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what activates vitamin K
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epoxide reductase
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what does warfarin inhibit
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epoxide reductase (activates vitamin K)
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what drug activates antithrombin
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heparin
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mutation that produces a factor V resistant to activated protein C's inhibition
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factor V leidin
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cleaves plasminogen into plasmin
*plasmin ultimately cleaves fibrin mesh |
tissue plasminogen activator
*thrombolytic |
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two cofactors for protein C
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protein S
thrombomodulin |
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what does protein C do
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cleaves and inactivates factors V and VIII
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what binds to exposed collagen upon endothelial damage and starts the platelet plus formation
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vWF
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what do platelets release once they bind to vWF via GpIb receptor
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release ADP and Ca - both are necessary for coagulation cascade)
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two function of ADP once released from platelets
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1. helps platelets adhere to endothelium
2. induces GpIIb/IIIa receptor expression at platelet surface |
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function of fibrinogen
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binds GpIIb/IIIa receptors and links platelets
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thromboxane A2, PGI2 and NO in coagulation
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TXA2 (pro-aggregation factor) - decreases blood flow and increases platelet aggregation
PGI2 and NO (anti-aggregation factor) - increases blood flow and decreases platelet aggregation |
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inhibits cyclooxygenase and therefore thromboxane A2 synthesis
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aspirin
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inhibit ADP-induced expression of GPIIb/IIIa
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ticlopidine
clopidogrel |
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inhibits GpIIb/IIIa directly
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abciximab
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acanthocyte (spiny RBC)
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liver disease
abetalipoproteinemia |
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associated with basophilic stippling
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TAIL:
thalassemias anemia of chronic disease iron deficiency lead poisoning |
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associated with bite cells
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G6PD deficiency
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associated with schistocytes (helmet cells)
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DIC
TTP/HUS traumatic hemolysis |
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associated with teardrop cells
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bone marrow infiltration
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associated with target cells
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HbC
asplenia liver disease thalassemia |
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associated with Heinz bodies
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a-thalassemia
G6PD deficiency |
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mechanism of Heinz body
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oxidation of iron from ferrous to ferric form leads to denatures hemoglobin precipitation and damage to RBC membrane - eventually forms bite cells in G6PD deficiency
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associated with Howell-Jolly bodies
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functional hyposplenia or asplenia
*basophilic nuclear remnants |
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thalassmia associated with Asian and African populations
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a-thalassemia
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Hb Barts (y-4)
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deletion of 4 a-globulin genes, incompatible with life
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deletion of 3 a-globulin genes
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HbH disease (B-4)
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differentiate the defect found in a-thalassemia vs. B-thalassemia (biochemically)
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a-thalassemia - a-globulin gene mutations (deletions)
B-thalassemia - point mutations in splicing sites and promoter sequences |
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diagnosis confirmed by increased HbA2 (>3.5%)
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B-thalassemia minor (heterozygote)
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associated with severe anemia requiring blood transfusions and marrow expansion (crew cut on skull x-ray) leading to skeletal deformities
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B-thalassemia major (homozygote)
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associated with decreased ferrochelatase and ALA dehydratase along with inhibits rRNA degradation leading to aggregation of ribosomes
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lead poisoning
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associated with a X-linked defect in aminolevulinic acid synthase gene
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siderblastic anemia
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what causes the ringed siderblasts in siderblastic anemia
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iron-laden mitochondria
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associated with increased iron and ferritin, with normal TIBC
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sideroblastic anemia
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treatment of sideroblastic anemia
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pyridoxine therapy (B6)
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causes of siderblastic anemia (defect in heme synthesis)
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ALA synthase deficiency
lead alcohol B6 deficiency |
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causes of microcytic, hypochromic anemia (MCV < 80)
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iron deficiency
a-thalassemia B-thalassemia lead poisoning siderblastic anemia |
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characteristics of lead poisoning
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LEAD:
1. lead lines on gingivae and long bones 2. encephalopathy and erythrocyte basophilic stippling 3. abdominal colic and anemia 4. drops - wrist and foot drop |
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causes megaloblastic macrocytoic anemia (MCV > 100)
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folate deficiency
B12 deficiency |
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associated with increased homocysteine but normal methylmalonic acid
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folate deficiency
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associated with both increased homocysteine and methylmalonic acid
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B12 deficiency
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causes nonmegaloblastic macrocytic anemias
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1. liver disease
2. alcoholism (can occur in absence of folate/B12 deficiency) 3. reticulocytosis (bigger than mature RBCs) 4. congenital deficiencies of purine or pyrimidine synthesis |
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two general categories for nomocytic, normochromic anemia
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nonhemolytic vs. hemolytic
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associated with decreased heptoglobin, increased LDH, hemoglobin in urine
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intravascular hemolysis
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associated with increased LDH, increased unconjugated bilirubin, causes include: hereditary spherocytosis, G6PD deficiency, sickle cell anemia
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extravascular hemolysis
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nonhemolytic causes of normocytic, normochromic anemia
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anemia of chronic disease
aplastic anemia kidney disease |
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this disorder is associated with inflammation which increases hepcidin which decreases release of iron from macrophages
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anemia of chronic disease
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associated with decreased iron and TIBC, but increased ferritin
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anemia of chronic disease
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pancytopenia characterized by severe anemia, neutropenia, and thrombocytopenia
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aplastic anemia
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defect in proteins interacting with RBC membrane skeleton and plasme membrane, causes round RBC with no central pallor
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hereditary spherocytosis
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associated with increased MCHC and RDW
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hereditary spherocytosis
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lab test used for hereditary spherocytosis
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positive osmotic fragility test
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x-linked deficiency that causes decreased glutathione and increases RBC susceptiblity to oxidant stress
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G6PD deficiency
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associated with blood smear showing RBC with Heinz bodies and bite cells
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G6PD deficiency
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this autosomal recessive defect in RBCs is associated with hemolytic anemia in a newborn
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pyruvate kinase deficiency
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two diseases associated with "crew cut" on skull x-ray due to marrow expansion
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sickle cell disease
B-thalassemia major |
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what precipitates sickling in sickle cell anemia
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low oxygen content of dehydration
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what is associated with resistance to malaria
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heterozygous sickle cell trait
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complications associated with sickle cell disease
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1. aplastic crisis (parvovirus infection)
2. autosplenectomy - increased risk of infection with encapsulated organisms 3. salmonella osteomyelitis 4. vaso-occlusive (painful crisis) 5. renal papillary necrosis 6. splenic sequuestration crisis |
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treatment of sickle cell crisis
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hydroxyurea (increases HbF)
bone marrow transplantation |
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diseases associated with intrinsic hemolytic normocytic anemia
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hereditary spherocytosis
G6PD deficiency pyruvate kinase deficiency sickle cell anemia HbC defect PNH |
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diseases associated with extrinsic hemolytic normocytic anemia
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autoimmune hemolytic anemia
microangiopathic anemia macroangiopathic anemia malaria and Babesia infections |
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warm vs. cold agglutinin autoimmune hemolytic anemia
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warm - IgG, chronic anemia seen in SLE, CLL, or drugs
cold - IgM, acute anemia triggered by cold, mycoplasma pneumoniae or infectious mononucleosis |
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anti-Ig antibody added to patient's RBCs agglutinate if RBCs are coated with the Ig
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direct coombs' test
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normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface Ig
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indirect coombs' test
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RBCs are damaged when passing through obstructed or narrowed vessel lumen; seen in DIC, TTP/HUS, SLE, and malignant hypertension
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microangiopathic anemia
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prosthetic heart valves and aortic stenosis may cause hemolytic anemia secondary to mechanical destruction
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macroangiopathic anemia
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iron storage protein of body
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ferritin
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transports iron in blood
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transferrin
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what is the serum iron, transferrin (TIBC), ferritin, and % saturation in iron deficiency
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*decreased serum iron
increased TIBC decreased ferritin decreased % saturation |
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what is the serum iron, transferrin (TIBC), ferritin, and % saturation in chronic disease
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decreased serum iron
decreased TIBC *increased ferritin normal % saturation -TIBC is decreased because body stores iron in cells to prevent pathogens from acquiring circulating iron |
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what is the serum iron, transferrin (TIBC), ferritin, and % saturation in hemochromatosis
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*increased serum iron
decreased TIBC increased ferritin increased % saturation |
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what is the serum iron, transferrin (TIBC), ferritin, and % saturation in pregnancy/oral contraceptives
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normal serum iron
*increased TIBC normal ferritin decreased % saturation |
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what is the serum iron, transferrin (TIBC), ferritin, and % saturation in lead poisoning
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increased serum iron
decreased TIBC normal ferritin increased % saturation |
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serum iron and TIBC are usually inverse of each other, which two disorders is this not the case
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anemia of chronic dieases - decreased TIBC to keep iron away from pathogens
pregnancy/oral contraceptive use - directly increases TIBC regardless of what serum iron levels are |
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associated with protoporphyrin accumulation in blood due to ferrochelatase and ALA dehydratase enzymes being affected
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lead poisoning
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associated with porphobilinogen deaminase defect leading to painful abdomen, red win-colored urine, polyneuropathy, and psychological disturbances
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acute intermittent porphyria
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treatment of acute intermittent porphyria
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glucose and heme, these inhibit ALA synthase
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associated with uroporphyrinogen decarboxylase defect leading to blistering cutaneous photosensitivity and tea-colored urine
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porphyria cutanea tarda
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most common porphyria
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porphyria cutanea tarda (uroporphyrinogen decarboxylase defect)
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what do the heme concentrations do to ALA synthase activity
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inverse of each other:
decreased heme causes increased ALA synthase activity |
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tests function of all factors except VII and XIII
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PTT (intrinsic pathway)
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associated with hemarthroses, easy bruising, and increased PTT
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hemophilia A or B
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PT and PTT of vitamin K deficiency
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both are prolonged
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symptoms of platelet abnormalities
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mucous membrane bleeding
epistaxis petechiae/purpura increased bleeding time |
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increased bleeding time due to defect in platelet-to-collagen adhesion
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decreased GpIb - Bernard-Soulier disease
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increased bleeding time due to defect in platelet-to-platelet aggregation
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decreased GpIIb/IIIa - Glanzmann's thrombasthenia
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decreased platelet survival due to anti-GpIIb/IIIa antibodies causing peripheral platelet destruction and megakaryocytosis
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idiopathic thrombocytopenic purpura (ITP)
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decreased platelet survival due to deficiency of ADAMTS 13 (vWF metalloprotease - decreased degredation of vWF multimers)
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thrombotic thrombocytopenic purpura (TTP)
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associated with increased large vWF multimers, increased platelet aggregations and thrombosis
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TTP
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symptoms associated with TTP
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neurologic symptoms
renal symptoms fever thrombocytopenia microangiopathic hemolytic anemia |
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most common inherited bleeding disorder, autosomal dominant
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von Willibrand's disease
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treatment of vWD
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desmopressin which release vWF stores in endothelium
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platelet count, bleeding time, PT and PTT seen in vWD
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normal platelet count
increased bleeding time normal PT normal or slightly increased PTT (depends on severity due to carrying of factor VIII) |
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platelet count, bleeding time, PT and PTT seen in DIC
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decreased platelet count
increased bleeding time increased PT and PTT |
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associated with schistocytes, increased fibrin split products (D-dimers), and decreased factors V and VIII
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DIC
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most common cause of inherited hypercoagulability
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factor V Leiden
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decreased ability to inactivate factors V and VIII
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protein C or S deficiency
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associated with increased risk of thrombotic skin necrosis with hemorrhage following administration of warfarin
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protein C or S deficiency
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hereditary thrombosis syndromes leading to hypercoagulability
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Factor V Leiden
prothrombin gene mutation ATIII deficiency Protein C or S deficiency |
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prothrombin gene mutation
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mutation in 3' untranslated region
associated with venous clots |
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differentiate leukemia vs. lymphoma
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leukemia - widespread involvement of bone marrow with tumor cells usually found in peripheral blood
lymphoma - discrete tumor masses arising from lymph nodes |
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leukemoid reaction
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increased WBC count with left shift (80% bands) and increased leukocyte alkaline phosphatase, due to infection
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what determines prognosis of Hodgkin's lymphoma
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increased lymphocytes with decreased Reed-Sternberg cells
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cells markers from RS cells
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CD30 and CD15 B-cell origin
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which type of Hodgkin's lymphoma is associated with lacunar cells and primarily affects young adults
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nodular sclerosing
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what is the lymphocyte-to-RS cell ratio in nodular sclerosing Hodgkin's lymphoma
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increased lymphocytes/RS cells
good prognosis |
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4 B-cell non-Hodgkin's lymphomas
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Burkitt's lymphoma
diffuse large B-cell lymphoma mantle cell lymphoma follicular lymphoma |
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translocation associated with Burkitt's lymphoma
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t(8:14) c-myc gene translocation
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lymphoma with starry-sky appearance (sheets of lymphocytes with interspersed macrophages), associated with EBV
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Burkitt's lymphoma
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most common adult non-hodgkin lymphoma
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diffuse large B-cell lymphoma
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translocation in mantle cell lymphoma
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t(11:14)
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what corresponds with poor prognosis in mantle cell lymphoma
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CD5+
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translocation in follicular lymphoma
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t(14:18) bcl-2 expression
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what does bcl-2 expression in follicular lymphoma do
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inhibits apoptosis
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two types of T cell non-Hodgkin lymphomas
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adult T-cell lymphoma
mycosis fungoides/sezary syndrome |
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Lymphoma associated with HTLV-1 infection, adults present with cutaneous lesions
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adult T- cell lymphoma
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lymphoma associated with cutaneous patches/nodules that is CD4 positive
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mycosis fungoides/sezary syndrome
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most common primary tumor arising within the bone in the elderly
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multiple myeloma
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what is associated with: primary amyloidosis, punched-out lytic bone lesions, M spike on protein electrophoresis (gamma area), Bence Jones protein (Ig light chains in urine), and routleaux formation
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Multiple Myeloma
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other problems associated with MM
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hypercalcemia
renal insufficiency anemia bone lytic lesions |
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distinguish which types of Ig are overproduces in MM vs. Waldenstrom's macroglobulinemia
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MM - IgG or IgA
Waldenstrom's - IgM |
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disease with hyperviscosity symptoms and M spike due to increased IgM production
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Waldenstrom's macroglobinulinemia
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leukemia associated with being TdT+ and CALLA+
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ALL
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marker of pre-T and pre-B cells
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TdT+
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leukemia seen in older individual (>60 years) and is associated with warm antibody autoimmune hemolytic anemia
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CLL (chronic lymphocytic leukemia)
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leukemia associated with staining TRAP (tartrate-resistant acid phosphatase) positive
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hairy cell leukemia
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leukemia associated with Auer rods
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acute myelogenous leukemia
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which form of AML responds to all-trans retinoic acid (vitamin A)
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M3 subtype
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leukemia associated with t(9:22) bcr-abl Philadelphia chromosome
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CML
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what happens when CLL has a "blast crisis"
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transforms into AML or ALL
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leukemia associated with very low leukocyte alkaline phosphatase
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CML
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which leukemia responds to imatinib
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CML
anti-bcr-abl antibody |
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leukemia associated with non-antibody-producing B cells and therefore these patients are susceptible to infection
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CLL
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peroxidase-positive cytoplasmic inclusions in granulocytes and myeloblasts of AML
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Auer Rods
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translocation seen in M3 type of AML
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t(15:17)
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proliferative disorder of defective S-100 and CD1a expressing cells with Birbeck granules (tennis rackets)
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langerhans cell histiocytosis
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which chronic myeloproliferative disorders are associated with JAK2 mutatioins
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polycythemia vera
essential thrombocytosis myelofibrosis |
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what is JAK2 involved in
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hematopoietic growth factor signaling, mutations are implicated in meyloproliferative disorders other than CML
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associated with decreased RBCs with teardrop shaped myeloid cells (including RBCs)
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myelofibrosis
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plasma volume, RBC mass, O2 saturation, and erythropoietin seen in relative polycythemia
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decreased plasma volume
normal RBC mass, O2 saturation, and EPO |
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plasma volume, RBC mass, O2 saturation, and erythropoietin seen in appropriate absolute polycythemia
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normal plasma volume
increased RBC mass decreased O2 saturation increased EPO |
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plasma volume, RBC mass, O2 saturation, and erythropoietin seen in inappropriate absolute polycythemia
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normal plasma volume
increased RBC mass normal O2 saturation increased EPO |
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plasma volume, RBC mass, O2 saturation, and erythropoietin seen in polycythemia rubra vera
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increased plasma volume
increased RBC mass normal O2 saturation decreased EPO |
