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Hematology 2
| Question | Answer |
|---|---|
| the stoppage of blood flow | hemostasis |
| inhibits platelet activation; natural anticoagulant | prostacyclin |
| converts plasminogen to plasmin; comes from damaged tissue; fibrinolytic | tissue plasminogen activators |
| secreted by endothelium, required for platelet adhesion | Von Willebrand factor |
| enhances the activity of antithrombin-III | Heparan Sulfate |
| receptor for thrombin; enhances Protein C | Thrombomodulin |
| adhere to injured vessels; promote coagulation on their phospholipid surface; clot retraction | Platelets |
| platelet contraction | Thrombosthenin / Actomyosin |
| platelet shape | Uromodulin / Tubulin |
| controlled by vessel smooth muscle; enhanced by TxA2 and serotonin | vasoconstriction |
| to form initial platelet plug; enhanced by TxA2, serotonin and ADP | platelet aggregation |
| Basic sequence of events in primary and secondary hemostasis | 1. vasoconstriction 2. platelet adhesion 3. platelet aggregation 4. fibrin-platelet plug formation 5. fibrin stabilization |
| Fibrinogen | Factor 1 |
| Prothrombin | Factor 2 |
| Tissue factor | Factor 3 |
| Calcium | Factor 4 |
| Proaccelerin, Labile, Accelerator globulin | Factor 5 |
| Proconvertin, Stable, Autoprothrombin 1 | Factor 7 |
| Antihemophilic factor, Platelet cofactor 1, Antihemophilic A | Factor 8:C |
| Plasma thromboplastin component, Christmas, platelet cofactor 2, Antihemophilia B | Factor 9 |
| Stuart, Prower, Autoprothrombin 3 | Factor 10 |
| Antihemophilic C, Plasma thromboplastin antecedent | Factor 11 |
| Hageman factor, Glass, Contact | Factor 12 |
| Lako-Lorand, Fribrinase, FIbrinoligase, Serum Transglutamase | Factor 13 |
| Fletcher factor | Prekallikrein |
| Intrinsic pathway | 12, 11, 9, 8, HMWK, PK, Ca, Phospholipid |
| Extrinsic pathway | 7, tissue thromboplastin, Ca, Phospholipid |
| Common pathway | 10, 5, 2, 1, Ca, Phospholipid |
| platelets attach to the damaged blood vessel through.. | vWF |
| ___ found on platelet surface acts a receptor for vWF | Gp1B |
| disease with no vWF, deficient of Factor 8:C | Von Willebrand Disease |
| lack of Gp1B, has giant plts | Bernard-Soulier Syndrome |
| components of platelet dense granules | CAPAS (Ca, ATP, Pyrophosphate, ADP, Serotonin) Mg |
| platelets attach to each other through the receptor ___ and __ | Gp2B3A, Fibrin |
| resting platelet shape | biconvex |
| activated platelet shape | irregular shape |
| it can directly activate 9-9a | Kallikrein |
| begins clot dissolution; cleaves factor 12a to 12f | plasmin |
| activates plasminogen | Factor 11 activation |
| pathway initiated with the release of tissue factor | Extrinsic pathway |
| low levels of thrombin acts as an__ | activator |
| high levels of thrombin act as an__ and it activates Protein C | inhibitor |
| inactive substrate which are converted to serine proteases | zymogens |
| an active enzyme | serine proteases |
| what factor do Protein C and S inactivate? | Factor 5 and 8 |
| contact group | 12, 11, prekallikrein, HMWK |
| Prothrombin group | 9, 10, 7, 2, protein C and S |
| Fibrinogen group | 1, 5, 8, 13 |
| coagulation factor group that is reduced by Warfarin and Coumarin | prothrombin group |
| coagulation factor group that is well preserved in stored plasma | prothrombin group |
| coagulation factors that are storage labile | 5 and 8 |
| coagulation factors that are heat labile | 1, 5, 8 |
| a body's defense against occlusion of blood vessel | Fibrinolysis |
| fibrinolysis is dependent on the enzyme__ | plasmin |
| what are the four principal fragments | X, Y, E, D |
| an FDP that is an indicator of in vivo fibrinolysis, it indicates the presence of fibrin split products | D-Dimer |
| a fragment that is a powerful inhibitor of thrombin | Fragment E |
| principal inhibitor of plasmin | A2-antiplasmin |
| principal inhibitor of coagulation | Antithrombin 3 |
| premature activation of the clotting process results to __ tests | falsely shortened |
| labile factors at room temperature | 5, 8 |
| prematurely activated at ref temp | 7 and 11 |
| most commonly used needle size for hemostasis sample collection | 20-gauge` |
| an anticoagulant that binds Calcium | trisodium citrate |
| trisodium citrate preserves what factors | 5,8 |
| underfilled tubes will cause __ coag test result | prolonged |
| preferred anticoagulant | 3.2% Na Citrate |
| in this anticoagulant, Factor 5 is unstable | EDTA |
| this anticoagulant acts with antithrombin 3 and inhibits all stages of coagulation | Heparin |
| anticoagulant that can be used for platelet retention test | heparin |
| changes in pH can __ clotting times | prolong |
| centrifugation of coag test | 2000g for 10mins |
| Tests for the Intrinsic and Common Pathway | 1. Lee and White 2. Plasma Recalcification Time 3. APTT |
| are lipoprotein which can be fractionated into lipids and proteins | Thromboplastin |
| only the phospholipid component is present | partial thromboplastin |
| activators for APTT | Kaolin, Celite, Ellagic Acid, Micronized Silica |
| APTT is used for monitoring __ therapy | heparin |
| most sensitive thromboplstin | Manchester Reagent |
| Test for Extrinsic and Common Pathway | Prothrombin Time |
| PT Time is the test of choice for monitoring __ therapy | warfarin/coumarin |
| therapeutic range for INR | 2.0-3.0 |
| provides a means of standardizing PT report worldwide | International Normalized Ratio |
| INR of normal patient | 0.9-1.4 |
| utilizes the powerful coagulant properties of Russel's Viper venom | Stypven time |
| capable of bypassing the action of f7 and directly activates 10-10a | stypven time |
| stypven time normal value | 6-10 seconds |
| AKA Duckhert's Test | 5M Urea solubility test |
| used to screen for fibrinogen deficiency | thrombin time |
| thrombin time normal values | 10-14 seconds |
| assess fibrinogen deficiency in patients undergoing heparin therapy | reptilase time |
| reagent for reptilase time: | Bothropsatrox snake venom |
| normal value of Reptilase time | 10-15 seconds |
| thrombin reagent concentration in Claus FI assay | 50 NIH units/mL |
| in Claus FI, PPP to be tested is diluted __ with Owren buffer | 1:10 |
| APTT: Abnormal NP: NC associated with Bleeding | F8 and F9 inhibitor |
| APTT: Abnormal NP: NC associated with Thrombosis | Lupus Anticoagulant |
| PT: N, APTT: A, AP: NC, AS: C | Factor 9 |
| uses Rees-Ecker as diluent, uses light microscopy | Tonkantin method |
| uses 1% ammonium oxalate | Brecher-Cronkite / phase microscopy |
| reference method for manual platelet count | Brecher-Conkrite / phase microscopy |
| 1 platelet = RBC | 25 |
| approximately, __ platelets/OIF | 8-20 |
| factor used in platelet estimation on PBS | 2000 |
| aggregation disorders; NORMAL RISTOCETIN | Glanzmann thrombasthenia, afibrinogenemia |
| adhesion defects; ABNORMAL RISTOCETIN | vWD, Bernard Soulier Syndrome |
| purpura associated with abdominal pain | Henoch's purpura |
| purpura associated with joint pain | Schonlein purpura |
| anemia generalized BM suppression leading to a decrease in all cell types | Aplastic Anemia |
| absent or decreased and abnormal BM megakaryocytes | Thrombocytopenia with Absent Radius |
| (Storage pool defect) alpha granules are missing, giant platelet and appear gray in Wright Stain | Gray platelet syndrome |
| (storage pool defect)lacks dense and alpha, characterized by a triad of THROMBOCYTOPENIA, RECURRENT INFX, ECZEMA. | Wiskott-Aldrich Syndrome |
| (storage pool defect) giant lysosomes, lacks beta | Chediak-Higashi |
| (storage pool defect) lacks beta and dense. | Hermansky - Pudlak |
| Ashkenazi Jews, Rosenthal Syndrome | Factor 11 Hemophilia C |
| Treatment for Hemophilia A deficiency | Cryoprecipitate, F8 concentrate |
| Treatment for Hemophilia B deficiency | FFP, F9 concentrate |
| most frequently encountered hereditary coagulopathy | vWD |
| recommended for vWD initial workup | CBC, APTT, PT |
| Owren's disease | Factor V deficiency |
| low levels / decreased fibrinogen (<100mg/dL) | hypofibrinogenemia |
| in DIC, D-Dimer is | positive |
| a test for secondary FDP | protamine sulfate gelation test |
| principle for protamine sulfate gelation test | Paracoagulation |
| specific than protamine sulfate test. principle: 50% ethanol | Ethanol gelation test |
| latex D-Dimer assay positive after __ hours of DIC onset | 4 |
| measures secondary fibrinolysis | Latex D-Dimer assay |
| results in reduction in the oxygen-carrying capacity of the blood and subsequent hypoxia | Anemia |
| mechanisms of Anemia | Blood loss Impaired RBC production Accelerated hemolysis |
| hypochromia grading: area of central pallor is one-half of cell diameter | 1+ |
| hypochromia grading: area of central pallor is two-thirds of cell diameter | 2+ |
| hypochromia grading: area of central pallor is three quarters of cell diameter | 3+ |
| hypochromia grading: thin rim of hemoglobin | 4+ |
| rouleaux grading: aggregates of 3-4 RBCs | 1+/slight |
| rouleaux grading: aggregates of 5-10 RBCs | 2+/moderate |
| rouleaux grading: numerous aggregates with only few free RBCs | 3+/marked |
| variation in Hb content | anisochromia |
| directly proportional to the degree of anisocytosis | RDW |
| derived from histogram; coefficient of variation of the MCV | RDW |
| variation in size | anisocytosis |
| variation in shape | poikilocytosis |
| most preferred BM collection site | posterior superior illac crest |
| least preferred BM collection site | ribs and vertebrae |
| normal M:E ratio | 2:4 - 4:1 or 3:1 |
| BM diff count, at least __ cells but preferably ___ cells must be counted | 500 , 1000 |
| reflects body's tissue iron stores | Serum ferritin |
| 1st test to become abn when iron stores begin to decrease | Serum ferritin |
| indirect measure of transferrin | TIBC |
| micro-hypo anemia caused by inadequate supplies of iron to synthesize hb | IDA |
| dec: serum iron, serum ferritin, retics | IDA |
| inc: RDW, TIBC | IDA |
| smear shows ovalocytes/pencil forms | IDA |
| master regulator of Iron | Hepcidin |
| impaired release of storage iron associated with increased hepcidin levels | Anemia of Chronic Disease |
| dec: TIBC, serum iron inc or norm: ferritin | ACD |
| caused by BLOCKS in the protoporphyrin pathway resulting to ineffective hb synthesis and IRON OVERLOAD | Sideroblastic Anemia |
| EXCESS IRON accumulates in the mitochondrial region of the IMMATURE RBC | ringed sideroblast |
| EXCESS IRON accumulates in the mitochondrial region of the MATURE RBC | siderocytes |
| inclusions in the siderocyte using PERL'S PRUSSIAN BLUE | Siderotic granules |
| inclusions in the siderocyte using WRIGHT'S STAIN | Pappenheimer Bodies |
| genetic disorder characterized by a primary, quantitative REDUCTION OF GLOBIN CHAIN synthesis | Thalassemia |
| ABSENCE of both BETA CHAINS resulting in an excess of alpha chains | Major/homozygous / Cooley anemia |
| thalassemia compensated with up to 90% Hb F | Major homozygous / Cooley anemia |
| thalassemia which ONE BETA CHAIN IS NORMAL | Minor heterozygous |
| thalassemia compensated with HbA2 | Minor heterozygous |
| all four ALPHA chains are DELETED | Major alpha thalassemia |
| disease is known as HYDROPS FETALIS | Major alpha thalassemia |
| 80% Bart's Hb is produced | Major alpha thalassemia |
| THREE ALPHA chains are deleted, lead to BETA chain EXCESS | Hb H Disease |
| golf ball cells can be seen using BCB | Hb H Disease |
| TWO ALPHA genes are DELETED | Minor / Trait Alpha Thalassemia |
| up to 6% Bart's Hb | Minor / Trait Alpha Thalassemia |
| shows coarse BASOPHILIC STIPPLING | Lead Poisoning |
| causes multiple blocks in protoporphyrin pathway | Lead Poisoning |
| defective DNA synthesis causes ABN NUCLEAR MATURATION | Megaloblastic Anemia |
| macro-normo with OVAL MACROCYTES | Megaloblastic anemia |
| macro-normo with ROUND MACROCYTES | Non-megaloblastic anemia |
| associated with inc LDH, bilirubin, iron levels | Megaloblastic anemia |
| seen with Howell-Jolly bodies, Basophilic stippling, pappenheimer bodies and cabot rings | Megaloblastic anemia |
| causes flipped LDH | 1. Acute Myocardial Infarction 2. Renal Infarction 3. Hemolytic anemia |
| an AUTOIMMUNE DSE wherein NO INTRINSIC factor is produced by parietal cells. | Pernicious anemia |
| H. pylori and D. latum infection | Vitamin B12 deficiency |
| test detects pernicious anemia | Schilling's test |
| Bone Marrow: hypocellular hypoplastic with increased fat | Aplastic anemia |
| caused by CHLORAMPHENICOL, BENZENE, HERBICIDES, PARVO B19 | acquired aplastic anemia |
| associated with hypo/hyperpigmentention, hypogonadism, low birth weight | Fanconi Anemia |
| pure red cell aplasia | Diamond Blackfan Anemia |
| hypoproliferative anemia caused by replacement if BM hematopoietic cells by malignant cells or fibrotic tissue | Myelophthisic anemia / Myeloid Metaplasia |
| shows leukoerythroblastic blood picture and DACROCYTES | Myelophthisic anemia / Myeloid Metaplasia |
| earliest hematological in acute blood loss anemia | transient fall in plt count |
| characterized by gradual LONG TERM of blood loss, often caused by GI bleeding | chronic blood loss anemia |
| anemia with dec HB and HAPTOGLOBIN | Hemolytic anemia |
| defect in membrane SKELETAL PROTEIN | Hereditary spherocytosis |
| membrane defects caused by polarization of CHOLE at the END of the cell | Hereditary Elliptocytosis |
| abnormal PERMEABILITY both to Na and K causes RBC swelling | hereditary stomatocytosis |
| most common enzyme deficiency in EMB | Pyruvate Kinase Deficiency |
| acquired defect in which RBC membrane has an increased sensitivity for complement binding as compared to normal RBCs | PNH |
| an IgG biphasic DONATH-LANDSTEINER antibody | PNH |
| linked proteins such as CD 55 and CD 59 | PNH |
| attachment at cold, hemolysis at warm | PNH |
| classification of Immune Hemolytic anemia | Isoimmune, Autoimmune, Drug Induced |
| RBCs are coated with IgG or complement. macrophages PHAGOCYTIZE the RBCs. | Warm Autoimmune Hemolytic Anemia |
| RBCs are coated with IgM or complement. RBCs are LYSED by complement | Cold Autoimmune Hemolytic Anemia |
| systemic clotting is initiated by the activation of the coagulation cascade. fibrin is deposited in small vessels, causing RBCs to fragment. all coag test are abn | DIC |
| deficiency of the enzyme ADAMTS13 that is responsible for the breakdown of large vWF | TTP |
| hemoglobinopathies: 80% HbS and 20% HbF | Sickle Cell Dse (Hb SS) |
| hemoglobinopathies: 60% HbA and 40% HbS | Sickle Cell Trait (Hb AS) |
| shows folded cell. No HbA is produced | Hb C Disease |
| double heterozygous condition where an abn sickle cell and abn C gene is inherited | Hb SC Disease |
| presence of HEMOGLOBIN CRYSTAL | Hb SC Disease |
| Hb S: 6th amino acid, glutamate is replaced by | valine |
| Hb C: 6th amino acid,glutamate is replaced by | Lysine |
| Hb D: 121st amino acid, glutamate is replaced by | Glycine |
| Hb E: 26th amino acid, glutamate is replaced by | Lysine |
| Hb O: 121st amino acid, glutamate is replaced by | lysine |
| poikilocytosis seen in MAHA | Helmet cells / Horn/ Keratocytes |
| small round DNA FRAGMENTS | Howell-Jolly Bodies |
| seen in, Sickle cell anemia, beta thalassemia major, severe hemolytic anemia, megaloblastic anemia, alcoholism, post-splenectomy | Howell-Jolly Bodies |
| figure of 8 shape | Cabot rings |
| stacking or coining pattern of RBCs due to abn or inc plasma protein | rouleaux |
| characterized by CLUMPING of erythrocytes with NO PATTERN | agglutination |
| deficient in NADPH oxidase | Chronic granulomatous disease |
| nucleus has a dumbbell or peanut shape; pince-nez appearrance | Pelger-Huet Anomaly |
| nucleus is usually ROUND, instead dumbbell | pseudo pelger-huet anomaly |
| has DOHLE-like inclusions | May-Hegglin Anomaly |
| associated with GIANT PLATELETS | 1. Bernard-Soulier 2. Gray plt 3. May-Hegglin Anomaly 3. May Hegglin anomaly |
| granules contain DEGRADED MUCOPOLYSACCH | alder-riley anomaly |
| large AZUROPHILIC granule | alder-riley anomaly |
| most common lipid storage disorder | Gaucher disease |
| CRUMPLED TISSUE PAPER appearance on macrophage | Gaucher disease |
| deficiency in sphyngomyelinase | Niemann-pick dse |
| FOAMY appearance in macrophages | Niemann-pick dse |
| kissing dse; caused by EBV | IM |
| most common transmitted infx from mother to fetus | Cytomegalovirus |
| associated with adenovirus and coxackie A virus | Infectious Lymphocytosis |
| WHO defines acute leukemia as __% bone marrow blasts | >20% |
| standard for diagnosis in malignant leukocyte | WHO classification |
| stain used to help in the diagnosis of DiGuglielmo's syndrome (FAB M6) | Periodic Acid Schiff Stain |
| most common leukemia in adult | acute myelogenous leukemia |
| most common ALL in childhood. most T-cell ALLs | FAB L1 |
| most common ALL in adult | FAB L2 |
| leukemic phase of Burkitt's lymphoma. B cell | FAB L3 |
| the only chronic myeloproliferative disease NEGATIVE for JAK2 | CML / CGL |
| result of T(9,22) or BCR/ABL gene mutation | philadelphia chromosome |
| pathognomic cell is Reed-Sternberg cells | Hodgkin's Lymphoma |
| presence of BJP in the urine and precipitates at 40-60C and dissolves at 100C | Multiple myeloma |
| presence of Sezary cells/ ceribriform cell. Malignancy of T cells | Mycosis Fungoides |
| Flower cells. caused by HTLV-1 | Adult T-cell leukemia |
| computed values | mch, mchc, hct |
| derived values | mcv, rdw, mpv, pdw |
| directly measured by electrical impedance | rbc, wbc, plt |
| forward angle light scatter measures | cell size |
| in platelet histogram, what is the reference range | 2-20fL |
| leukocytes have gigantic granules that are peroxidase positive | Chediak-Higashi syndrome |
| 5 or more lobes in the neutrophil | Hypersegmentation |
| blood picture MIMICS CML; Increased LAP score | Neutrophilic Leukemoid Reaction |
| ABNORMAL random and chemotactic activity | Lazy Leukocyte Syndrome |
| NORMAL random activity but characterized by ABNORMAL chemotactic activity | Job's Syndrome |
| Fabry's Disease enzyme deficient | Alpha galactosidase |
| Gaucher Disease enzyme deficient | glucocerobrosidase |
| Krabbe enzyme deficient | Cerebrosidase beta galactosidase |
| Neimann-Pick enzyme deficient | Sphingomyelinase |
| Metachromatic leukodystrophy enzyme deficient | Arylsulfatase A |
| Sandoff enzyme deficient | Hexosaminidase A and B |
| Tay Sach enzyme deficient | Hexosaminidase A |
| platelets can reach up to >1000 x 10^9/L but abnormal plt function | essential thrombocythemia |
| pancytosis and Low EPO, inc blood viscosity | Polycythemia Vera |
| increased presence of SMUDGE CELLS in the PBS | Chronic Lymphocytic Leukemia |
| Waldenstrom's Macroglobulinemia increased Ig | IgM |
| Lymphoplasmacytic infiltration of the B.M | Waldenstrom's Macroglobulinemia |
| owl's eye appearance of nucleus | Reed-Sternberg cells |
| in ELECTRICAL IMPEDANCE, the number of pulses is proportional to | cell count |
| in ELECTRICAL IMPEDANCE, the amplitude of pulses is proportional to | cell size |
| uses flow cytometer with laser to measure light scattering properties of cells | light scattering optical method |
| internal complexity or cell granularity | orthogonal / slide angle |
| combination of forward low-angle and forward high-angle scatter which correlates to cell volume and refractive index or with internal complexity | differential scatter |
| representation of cell number versus one measured property, usually cell size | histogram |
| 1st peak in histogram | lymphocyte |
| 2nd peak in histogram | monocyte |
| 3rd peak in histogram | granulocyte |
| measurement of lymphocyte in histogram | 35-90fL |
| measurement of monocyte in histogram | 90-160fL |
| measurement of granulocyte in histogram | 160-450 fL |
| rbc histogram reference size range | >36fL |
| normal RBC histogram will show single peak between | 70-110 fL |
| RBC histogram two peaks indicate | dimorphic population |
| histogram: MVC shift to the right | macrocytic |
| histogram: MCV shift to the left | microcytic |
| measures the cell interior granules of cell | Radiofrequency |
| most common problem instrumental error | Aperture plugs |
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