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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