19154798
| Question | Answer |
| The process of formation and development of blood cells is called | hematopoiesis |
| During second trimester of fetal development, primary site of blood cell production is | Liver |
| Which organ is responsible for the maturation of T lymphocytes and regulating CD4 and CD8 expression | thymus |
| The best source of bone marrow for a 20 year old would be | iliac crest |
| Physiologic programmed cell death is called | apoptosis |
| Which organ sequesters platelets? | Spleen |
| What morphological change occurs during normal blood cell maturation? | Condensation of nuclear chromatin |
| What cell is a product of the CLP? | T lymphocyte |
| What cells are products of GMP? | granulocytes and monocytes |
| What growth factor is produced in kidneys and used to treat anemia? | EPO |
| What cytokine is involved in early differentiation of HSCs? | FLT3 ligand |
| What is one response to inadequate red cell production in severe anemia? | extramedullary hematopoiesis in liver and spleen |
| HSC's can adequately produce all lineages of blood cells over a lifetime because they | Can self renew through asymmetric division |
| What cells are produced by MEPs? | Megakaryocytes and erythrocytes |
| What is an erythrocyte progenitor? | HSC MEP CFU-E |
| What is the most mature normoblast? | Orthochromic normoblast |
| What erythroid precursor is blue pink with condensed chromatin and no nucleoli? | Polychromatic normoblast |
| What does EPO affect? | number of normoblast divisions, time between divisions, production of antiapoptotic molecules by progenitors |
| Hypoxia stimulates RBC production by | stimulating EPO production in kidneys |
| In bone marrow RBC precursors are located | surrounding macrophages in erythroid islands |
| What determines timing of egress of RBC from bone marrow | Normoblasts lose CAM receptors for stromal cells as they mature |
| What is most responsible for limiting normal RBC lifespan | Loss of nucleus |
| Fragmentation hemolysis is due to | trauma to RBCs while in circulation |
| Extravascular hemolysis occurs when | splenic macrophages ingest senescent cells |
| A pronormoblast belongs to the | erythron but not the red cell mass |
| A cell with N:C of 4:1 is mostly | composed of nucleus |
| What RBC process doesnt require energy? | oxygen transport |
| What pathway generates ATP anaerobically? | Embden Meyerhof pathway |
| What is the role of 2,3 BPG? | Enhances O2 release from hemoglobin |
| What byproduct detoxifies peroxides in RBCs? | NADPH and reduced glutathione |
| What helps maintain RBC shape? | Cytoskeletal proteins |
| Glycolipids of the RBC membrane | carry RBC antigens |
| RBC membranes block large molecules but allow cations and small molecules to pass, this is called | semipermeability |
| RBC membrane phospholipids are arranged | in two layers that are asymmetric |
| RBC membrane cholesterol is replenished from | plasma |
| What portion of the Embden-Meyerhof pathway reduces iron to the physiologic +2 from +3? | Methemoglobin reductase pathway |
| What is an example of an integral protein? | Glycophorin A |
| Abnormal membrane protein linkages may be seen as | changes in cell shape |
| A hemoglobin molecule is made of | one heme and four globin chains |
| What chains are Hemoglobin A made of | alpha and beta |
| The rate limiting step in heme synthesis is suppression of | Protoporphyrin IX reductase |
| What form of hemoglobin is lowest affinity for oxygen? | tense |
| In more acidic blood, which way is the hemoglobin oxygen dissociation curve shifted? | Shifted to the right, reduced oxygen affinity |
| Which hemoglobin is predominant in newborns | Hemoglobin F |
| What is the hemoglobin types from most to least common in healthy adults? | A, A2, F |
| What is the structure of oxidized hemoglobin? | Iron in ferric 3+ state and unable to bind oxygen, also known as methemoglobin |
| Globin subunits in hemoglobin form into | two dimers of alpha-beta chains |
| Alpha and beta genes are | on separate chromosomes but alpha genes are on the same one |
| 2,3 BPG acts by | binding the globin chains to release oxygen from heme |
| Iron is transported in plasma by | transferrin |
| Most available iron in the body is stored as | ferritin |
| Transferrin can be measured by | Serum total iron binding capacity |
| What test best reflects iron deficiency? | soluble transferrin receptor (proportional to the amount of transferrin receptor upregulated by cell iron demand) |
| What membrane protein in enterocytes transports iron from the intestinal lumen? | DMT1 |
| How is iron incorporated into heme? | DMT1 moves ferric iron into enterocytes, ferroportin moves iron into plasma, transferrin binds ferric iron, transferrin receptor moves the transferrin iron complex into cell, iron is moved to the mitochondria |
| Iron is removed from cells by | ferroportin |
| After endocytosis, transferrin receptor is | recycled into the plasma membrane to bind more transferrin |
| What regulates release of iron from enterocytes? | Hepcidin |
| How do you calculate percent transferrin saturation? | Total serum iron/total serum iron binding capacity |
| What are the axes of a Thomas plot? | Hemoglobin content of reticulocytes vs soluble transferrin receptor/log ferritin |
| Neutrophils and monocytes are direct descendants of a common progenitor known as | GMP |
| The stage in neutrophilic development where the nucleus is indented in a kidney shape and cytoplasm has lavendar secondary granules is called | Metamyelocyte |
| Type II myeloblasts are categorized by | Less than 20 primary granules per cell |
| What is the function of neutrophils? | Nonspecific destruction of foreign organisms |
| What immune cells regulate the immune response, mediate allergic inflammation, and destroy helminths? | Basophils and eosinophils |
| What class of immunoglobulin reacts with basophils and mast cells? | IgE |
| What cell type can differentiate into macrophages, osteoclasts, or dendritic cells? | Monocytes |
| Macrophages role in adaptive immunity is | degrading antigen and presenting it to lymphocytes |
| What is a B cell's final stage after activation? | Plasma cell |
| What megakaryocyte progenitor undergoes endomitosis? | LD-CFU-Meg |
| What growth factor induces megakaryocyte growth and differentiation? | TPO |
| What platelet organelle sequesters ionic calcium? | DTS |
| What platelet membrane receptor binds fibrinogen and supports platelet aggregation? | GP IIb/IIIa |
| What platelet membrane phospholipid flips to the plasma side and serves as the assembly point for coagulation factors? | Phosphatidylserine |
| What eicosanoid metabolite produced by endos suppresses platelet activity? | prostacyclin |
| What molecule is stored in platelet dense granules? | serotonin |
| What plasma protein is needed for platelet adhesion? | VWF |
| Reticulated platelets can be enumerated in peripheral blood to detect | increased platelet production |
| platelet adhesion refers to | platelets binding to surfaces such as subendothelial collagen |
| If potassium cyanide and potassium ferricyanide are used to determine hemoglobin concentration the final product is | cyanmethemoglobin |
| What interferes with cyanmethemoglobin method of hemoglobin determination? | increased lipids, lyse resistant RBCs, increased WBC count |
| What does reticulocyte count assess? | Erythropoietic activity of bone marrow |
| What happens to ESR if there is inflammation? | Increases |
| On automated CBC machines, what is hematocrit calculated from? | MCV |
| If MCHC is elevated and the peripheral shows agglutination, what will RBC count and MCV show? | RBC count will decrease and MCV will increase |
| Low voltage DC is used by automated machines to measure | total cell volume |
| Orthogonal light scatter is used to measure | internal complexity of the cell |
| What are common clinical symptoms of anemia? | fatigue and shortness of breath |
| What should be considered in a patient's history when investigating anemia? | diet and medications, occupation, hobbies, travel, bleeding episodes in family members |
| What is reduced in long standing anemia? | oxygen affinity of hemoglobin |
| Anemia where HSC's are diminished but RBC precursors are normal is considered anemia by mechanism of | insufficient erythropoiesis |
| What test value indicates shortened RBC lifespan and hemolytic anemia? | increased red cell distribution width |
| Poikilocytosis is detected by | peripheral smear examination |
| In men and postmenopausal women with adequate diet, iron deficiency anemia most often results from | chronic GI bleeding |
| When would levels of free erythrocyte protoporphyrin be increased? | Any condition where iron cannot be incorporated into protoporphyrin IX |
| Hepcidin levels in anemia of chronic inflammation | increase during inflammation and reduce iron absorption from enterocytes |
| sideroblastic anemias are caused by | failure to incorporate iron into protoporphyrin IX |
| Clinical consequences of pancytopenia are | fatigue, infection, and bleeding |
| Acquired aplastic anemia that is idiopathic | has no known cause |
| Acquired idiosyncratic aplastic anemia is caused by | destruction of stem cells by autoimmune response |
| What would you expect to find in a peripheral smear for severe aplastic anemia? | macrocytosis, thrombocytopenia, neutropenia |
| Anemia caused by chronic kidney disease is caused by | inadequate production of EPO |
| Unlike inherited aplastic anemia, Diamond Blackfan anemia | only affects erythropoiesis |
| Define hemolytic disorder | Disorder with increased destruction of RBCs after they enter the bloodstream |
| Both fragmentation and macrophage mediated hemolysis will result in | elevated urobilinogen in urine (from bilirubin metabolism) |
| What test is a good indicator of accelerated erythropoiesis? | reticulocyte count |
| What is consistent with megaloblastic anemia? | Increased MCV |
| If folate levels are decreased but B12 levels are normal, what would you expect methylmalonic acid to be? | Normal because it depends on B12 |
| If folate levels are decreased but B12 levels are normal, what do you expect homocysteine levels to be? | Increased since folate deficiency causes accumulation of homocysteine |
| If folate levels are normal and B12 levels are decreased, what do you expect methylmalonic acid levels to be? | elevated |
| Megaloblastic anemia can also be categorized as | macrocytic |
| Besides macrocytosis, megaloblastic anemia can be characterized by | hypersegmentation of neutrophils |
| What would you expect to find with elevated titers of intrinsic factor blocking antibodies? | hypersegmented neutrophils, macrocytic RBC, low B12 levels, high levels of methylmalonic acid |
| What is the role of intrinsic factor? | facilitates absorption of vitamin B12 and is secreted by parietal cells in stomach |
| What is the dominant metabolically active form of B12 | Holotranscobalamin |
| Folate and vitamin B12 work together to produce | DNA |
| Why does megaloblastic anemia cause macrocytosis? | Lack of genetic material results in a reduction in the number of cell divisions despite normal cytoplasm development |
| Pernicious anemia most commonly affects | Persons older than 60 |
| What would you expect to find in classical fragmentation hemolysis? | schistocytes, elevated total and indirect serum bilirubin, increased urinary urobilinogen, severely decreased haptoglobin |
| What is a distinct symptom of hemolytic anemia? | Jaundice |
| What would you expect to find for macrophage mediated hemolytic anemia vs fragmentation anemia? | Spherocytosis |
| Where is the majority of urobilinogen excreted? | in the intestines |
| For hereditary spherocytosis, what do you expect to see in the CBC | Increased MCHC |
| Hereditary spherocytosis results in cells | with increased osmotic fragility, negative DAT, polychromasia |
| Hereditary elliptocytosis is caused by | defects in horizontal membrane protein interactions |
| What do you expect to see in hereditary pyropoikilocytosis | poikilocytosis, increased RDW, thermal sensitivity, microspherocytosis |
| Acanthocytes are associated with | abetalipoproteinemia (fat soluble vitamin absorption deficiency), McLeod's disease, severe liver disease |
| G6PD deficiency is most commonly manifested as | Acute hemolytic anemia caused by drug exposure or infection |
| The most common cause of hereditary non-spherocytic hemolytic anemia is | pyruvate kinase deficiency |
| What feature is found in all microangiopathic hemolytic anemias? | intravascular RBC fragmentation |
| Typical findings in thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are | schistocytes and thrombocytopenia |
| Idiopathic TTP is caused by | Autoimmune antibodies binding ADAMTS-13 (which normally regulated VWF) and causing VWF platelet thrombi |
| What abnormal test results distinguish DIC from TTP or HUS? | PT and PTT (DIC depletes coagulation factors) |
| What does haptoglobin do? | Binds and removes free hemoglobin in circulation |
| Which plasmodium can cause malaria relapses? | P. vivax |
| How does malaria cause anemia? | Lysis of infected RBCs, immune response mediated destruction of RBCs, inhibition of erythropoiesis |
| What RBC morphology is seen after a severe burn injury? | microspherocytes and schistocytes (like in hereditary pyropoikilocytosis) |
| TTP unlike HUS displays | a large increase in serum lactate dehydrogenase |
| Immune hemolytic anemia is caused by | alloantibody or autoantibody against RBC antigen |
| Immune hemolysis with IgM antibodies is mediated by | compleMent |
| What RBC is typically found in IgG mediated hemolysis? | spherocytes |
| Positive DAT in autoimmune hemolytic anemia is evidence that | IgG antibody is sensitizing the patient's red blood cells |
| What are mechanisms of drug induced hemolytic anemia? | antibody production to drug after membrane adsorption, antibody to drug complexed to an RBC membrane protein, induction of RBC autoantibody production |
| Drug adsorption induced AIHA occurs | extravascularly |
| DHTR (delayed hemolytic transfusion reaction) is | due to an anamnestic response after repeat exposure to a blood group antigen |
| Chronic secondary cold agglutinin disease is most often associated with | B cell malignancy |
| A qualitative abnormality in hemoglobin incudes | any modification in the amino acids in a globin chain |
| When do patients with sickle cell disease usually exhibit symptoms | 6 months old, when Hb F starts to decrease |
| Megalobastic anemia in SCD can be prevented by prophylactic administration of | folic acid |
| Whats the most definitive test for Hb S? | Hb electrophoresis at acidic pH |
| A blood film of an anemic patient with target cells, nucleated RBCs, and hexagonal crystals inside of RBCs indicates | Hb CC, a substitution (lys>glu) in structure of beta globin chains |
| Thalassemias are caused by | absent or defective synthesis of a polypeptide chain in hemoglobin |
| Hemolytic anemia associated with thalassemias is caused by | imbalance of globin chain synthesis |
| B thalassemia minor will cause an increase in which Hb? | Increased HbA2 |
| B Thalassemia RBCs will usually be | microcytic, hypochromia, elliptocytes, target cells, stippling |
| The predominant Hb in B thalassemia major is | Hb F |
| Hb H is formed by | tetramer of beta chains |
| Hb Bart is formed by | tetramer of gamma chains |
| Sickle trait thalassemia progression resembles | sickle cell anemia |
| A peripheral film that shows increased neutrophils, basophils, eosinophils, and platelets is highly suggestive of | CML |
| A patient previously diagnosed with CML has circulating blasts and pros that total 30% of leukocytes, what phase is the disease | Transformation into AML |
| For PV peripheral blood findings typically include | erythrocytosis, granulocytosis, and thrombocytosis |
| A patient with platelet count of 700, observed abnormalities in platelet size/shape/granularity, a white count of 12, hemoglobin 11, and no Philadelphia chromosome is most likely to be diagnosed with | Essential thrombocytosis |
| What are some common complications associated with ET? | thrombosis, hemorrhage, and seizures |
| What would you expect to find in the peripheral for a patient with Primary myelofibrosis? | dacrocytes, nucleated RBCs, IGs |
| Myelofibrosis in PMF is caused by | increased stimulatory cytokines enhancing fibroblast activity |
| A patient previously diagnosed with CML has circulating blasts and pros that total 20% of leukocytes, what phase is the disease | accelerated |
| Myelodysplastic syndromes are most common in age group of | older than 50 years |
| What finding in peripheral blood and bone marrow examination is a major indication of MDS | Dyspoiesis |
| Peripheral findings in MDS may include | Oval macrocytes, agranular neutrophils, micromegakaryocytes, basophilic cytoplasm, giant platelets, ring sideroblasts, hypochromic microcytes, multinucleated RBCs |
| The mitochondria of a ringed sideroblast encircles | one third of the nucleus |
| What test would be best for differential of a patient with anemia, oval macrocytes, and hypersegmented neutrophils? | B12 and folate level test because it could be an MDS or a macrocytic anemia |
| MDS often converts to | AML |
| CML is classified by WHO as | bpth a myeloproliferative neoplasm and a myelodysplastic syndrome |
| The minimum percentage of blasts necessary for a diagnosis of acute leukemia is | 20% |
| A favorable sign for the prognosis of children diagnosed with ALL is | hyperdiploidy |
| Cerebral infiltration with blasts is more commonly seen in | ALL |
| Tumor lysis syndrome is associated with | Renal failure after initial chemotherapy |
| Pure erythroid leukemia is a disorder involving | pronormoblasts and basophilic normoblasts |
| The most common lymphoma in young adults is | Hodgkins lymphoma |
| The major difference between Hodgkin lymphoma and other B cell lymphomas is | presence of numerous reactive lymphocytes but only a few malignant cells |
| The function of the lymph node germinal center is | generating B cells that produce immunoglobulins with highest affinity for a presented antigen |
| B cells can produce highly specific immunoglobulins by process of | somatic mutation |
| Marked paracortical expansion is most commonly seen in | dermatophilic lymphadenopathy |
| The intimal cell that synthesizes VWF is | endothelial cell |
| Factor VII activation is triggered by the structural protein | Tissue Factor |
| If platelets are not aggregating properly, an assay should be done for | fibrinogen |
| Vitamin K will | carboxylate pt group factors to allow calcium binding |
| fibrinopeptides A and B are produced by | activation proteolysis of fibrinogen by thrombin |
| What factor is fibrinogen? | factor I |
| What is factor II | prothrombin |
| Factor IX complexes with Factor VIII to act on | Factor X |
| Factor VIII circulates bound to | VWF |
| Most coagulation factors are synthesized by | the liver |
| What is a typical form of anatomic bleeding? | soft tissue bleed |
| The typical treatment for vitamin K deficiency is | vitamin K and plasma |
| Systemic bleeding is distinguished by | mucocutaneous hemorrhage such as petechiae, purpura, or ecchymoses |
| Primary hemostasis defect is often manifested as | gum bleeding, epistaxis, hematemesis, menorrhagia |
| Anatomic bleeding is usually associated with defects in | secondary hemostasis, such as prolonged bleed after a minor trauma or surgical procedure |
| Thrombin time is an assay for | fibrinogen availability |
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