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Heme Final Part 2
MLS 310 with Ismail El-Amouri
| Question | Answer |
|---|---|
| Define Iron Deficiency Anemia. | Deficiency of iron due to malabsorption, malnutrition, or blood loss. |
| Define Anemia of Chronic Disease. | Iron will not leave the ferritin stores and cannot get to the RBC precursors. There is a defect in the hepcedin. |
| Define Sideroblastic Anemia. | Iron can leave the ferritin stores but cannot be inserted into the porphyrin ring. |
| Where does the iron accumulate during SA? | In the mitochondria, forming ringed sideroblasts. |
| What is the difference between a sideroblast and a siderocyte? | A sideroblast has a nucleus whereas a siderocyte does not. |
| What are the iron deposits on the ringed sideroblasts called? | Pappenheimer bodies |
| Which stain can stain the iron? | Prussian blue |
| What is the first stage of IDA? | Iron store depletion (exhaustion of iron stores) |
| What are the lab results with stage one IDA? | Normal serum iron Low serum ferritin N-sl high TIBC Normal saturation NO anemia RDW elevated Hgb normal normocytic, normochromic |
| What is the second stage of IDA? | Iron deficient erythropoiesis (insufficient iron to be inserted into protoporphyrin ring) |
| What are the lab results with stage two IDA? | Low serum iron Increased TIBC Low transferrin saturation No anemia yet |
| What is stage three of IDA? | Iron deficient Anemia |
| What are the lab results of stage three of IDA? | Low Hgb and Hct Low MCV and MCHC High RDW Anemia and hypochromia Microcytic, hypochromic |
| What is ferritin? | The iron stores |
| What is serum iron? | The amount of iron circulating in the blood |
| What is TIBC? | The amount of iron that is bound to transferrin |
| What is % saturation? | How much iron is saturated on the receptors |
| What is primary megaloblastic anemia? | Nutritional deficiency of B12 or folate |
| How does primary megaloblastic anemia affect DNA? | Leads to impaired DNA synthesis which impairs the nuclear maturation and cell division. ONLY AFFECTS DNA, NOT RNA |
| What is secondary megaloblastic anemia? | Due to malabsorption conditions such as pernicious anemia. |
| What is pernicious anemia? | Defect in HCL secretion and intrinsic factor. |
| Which cell types does megaloblastic anemia affect? | RBCs, WBCs, and PLTs (pancytopenia) |
| What is nuclear cytoplasm ansynchrony? | Young nucleus, old cytoplasm |
| What is non megaloblastic anemia? | Unrelated to nutritional deficiency. Due to liver disease, alcoholism...etc... |
| What does the intrinsic factor (IF) do? | Absorbs the B12 |
| What are some lab findings withe megaloblastic anemia? | Pancytopenia Macrocytic normochromic Macro ovalocytes and anisocytosis HJ bodies Hypersegmented neutrophils RPI <2 N:C ansynchrony Low serum folate/B12 High MMA and homocysteine |
| Why is MMA and homocysteine high in megaloblastic anemia? | Because B12 cannot convert them |
| Why is red cell folate a better indicator of MA? | Because RBCs survive for 120 days |
| What are some lab findings of nonmegaloblastic anemia? | Macrocytes round, not oval No hypersegmented neutrophils Normal WBC and PLT count Normal levels of B12 Normal levels of MMA and homocysteine |
| What are the two types of hemolytic anemia and which one is most common? | Intravascular (within blood vessels) and Extravascular (in the spleen, most common) |
| What are the lab findings with intravascular HA? | Low Hgb, Hct, and RBC count Schistocytes High serum bilirubin High LDH Low serum haptoglobin Hemoglobinemia Hemoglobinuria Hemosiderinuria |
| What does the serum haptoglobin do? | Carries bilirubin |
| What does hemosiderin do? | Breakdowns the porphyrin ring |
| What are some lab findings with extravascular HA? | Low Hgb, Hct, and RBC count Spherocytes High Serum bilirubin High LDH Hepatosplenomegaly |
| What is DAT used for? | To seperate HA's into immune mediated (inherited) and non immune mediated (aquired) |
| What membrane proteins are defected with HA? | HS (ankryin and Spectrin) HE (protein 4.1 and Spectrin) Hereditary Stomatocytosis PNH (CD55 and CD59) |
| What enzyme defects are associated with HA? | G6PD and PK |
| What Hgb defects are associated with HA? | Hemoglobinapathies Thalassemias |
| What is hereditary spherocytosis? | Vertical protein defect. Blood film shows spherocytes and MCHC is high whereas MCV is normal. |
| How do you diagnose HS? | Osmotic fragility test (will be increased) and DAT (will be neg) |
| What is hereditary elliptocytosis? | Horizontal protein defect |
| What is hereditary pyropoikilocytosis? | Severe form of HE |
| Explain the overhydrated form of Hereditary stomatocytosis. | More Na+ in than K+ out. Water rushes in. Uncompensated HA |
| Explain the dehyrdrated form of Hereditary stomatocytosis. | More K+ out than Na+ in. Water rushes out. Well compensated HA. |
| What is PNH? | defect in the GPI anchor for CD55 and CD59 proteins. |
| How do you diagnose PNH? | Sucrose hemolysis test (sugar water) Ham's test (acid serum test which will activate C') Flow analysis |
| What is the role of G6PD? | Catalyzes the formation of NADPH that is need to keep glutathione in the reduced state (GSH) to prevent oxidative damage to Hgb |
| What does G6PD deficiency cause? | Impaired generation of GSH -> cellular oxidants accumulate -> Hgb is oxidized to methemoglobin -> Heinz bodies formed and are removed by macrophages of spleen |
| In G6PD, what does the blood film and enzyme assay look like? | Blood film has bite cells, blister cells, and Heinz bodies. Enzyme assay has decreased flourescence. |
| Explain warm antibodies (Warm AIHA). | Mostly extravascular hemolysis via the spleen. Blood film shows spherocytes with a pos DAT |
| Explain cold antibodies (Cold AIHA). | Mostly intravascular hemolysis via C'. Blood film shows red cell agglutination |
| DAT can help you differentiate between which two conditions? | Heriditary spherocytosis (neg DAT) and Warm AIHA (pos DAT) |
| What does DAT and IAT look for? | DAT looks for antibodies whereas IAT looks for alloantibodies |
| What is the substitution for Hgb S? | B6 glu->val |
| What is the substitution for Hgb C? | B6 glu->lys |
| What is the substitution for Hgb E? | B26 glu->lys (microcytic, hypochromic anemia) |
| What is sickle cell trait? | The heterozygous state (SA). Mostly asymptomatic |
| What is sickle cell anemia? | The homozygous state (SS). Symptoms range from mild to severe |
| What causes the cells to sickle? | When oxygen tension decreases (deoxygenated) because Hgb S is soluble. |
| What is vaso-occlusive crisis? | The hallmark of sickle cell anemia. Very intense pain (body biting) |
| Recurrent vaso-occlusive crisis can lead to what? | Organ damage causing autosplenectomy |
| What type of anemia is sickle cell? | Normocytic Normochromic |
| What does the peripheal blood smear show in the homozygous and heterozygous sickle cell. | Hetero is mostly normal with some possible target cells Homo has target cells, sickled cells, nRBCs, polychromasia and possible HJ bodies and basophilic stippling from autosplenectomy |
| How is sickle cell diagnosed? | Solubility testing and Hgb electrophoresis. |
| What is the downfall to solubility testing? | It is just a screening test, it does not differentiate between trait and disease. |
| What is the reagent used in solubility testing and what does it do? | Sodium dithionite. Removes the O2 |
| What is thalassemia? | Inherited disorder caused by a mutation of one or more globin genes leading to decreased or absent synthesis of a structurally normal globin chain. |
| What are the differences between thalassemias and hemoglobinopathies? | Both the same except thalassemia has structurally normal globin chains whereas Hemogloblinopathy has structurally abnormal. Thalassemia is microcytic hypochromic and hemoglobinopathy is normochromic normocytic |
| What is Intermedia alpha thalassemia? | Hgb H disease. No Hgb A, only four beta chains. Pitted golf ball inclusions. --/-a |
| What is Major alpha thalassemia? | Hgb Barts/Hydrops Fetalis. No Hgb A only four gamma chains. Incompatible with life. --/-- |
| What is major beta thalassemia? | Homozygous, severe form of HA. Cooley's or Mediterranean anemia. Little or no beta chain sythesis (No Hgb A), mostly are Hgb F |
| What are the differences between Thalassemia and IDA? | Thalassemia has basophilic stippling, IDA does not IDA has anisocytosis, Thalassemia does not IDA has abnormal iron studies, Thalassemia does not |
| What is aplastic anemia and give an example? | Stem cell defect. Pancytopenia and hypocellular BM. EX: Fanconi Anemia |
| What is Pure Red Cell Aplasia and give an example? | Progenitor Cell Defect. Failure to produce erythroid and progenitor cells while leukocytes and platelets are normal. EX: Diamond Blackfan Syndrome |
| What is myelophthisic anemia? | From a bone marrow replacement |
| List the stages of leukocyte maturation. | Myeloblast: earlist recongnizable form of GM Promyelocyte: first recognizable of granulocyte Myelocyte: first commited to three types Metamyelocyte: first not capable of divison Band Seg |
| What is in the mitotic pool? | Myeloblasts, Promyelocytes, and Myelocytes |
| What is in the post mitotic pool? | Metamyelocytes, Bands, and Segs |
| What is in the circulating and marginal neutrophil pools? | 1/2 neutrophils in circulating, 1/2 neutrophils in marginal |
| Neutrophils move into the tissues from which pool? | Marginal |
| What is blastogenesis? | When B and T cells are activated and make fighter/end cells. |
| What are some fighter/end cells? | Reactive lymphs, large granular lymphs, and plasma cells |
| What are dohle bodies and what anomaly is often confused with it? | Laminar precipitates of rough ER (RNA). Confused with May-Hegglin |
| What is toxic granulation and what is often confused with? | Enhanced lysosome enzyme production. Confused with Alder-Reiley |
| What is Chediak Higashi Syndrome? | Defective chemotaxis and phagocytosis causing recurrent infections in children and even death |
| What kind of shape does Pelger-Huet form? | Dumbbell |
| How can you tell when it's just an artifact and not toxic neutrophils? | All cells will be effected |
| What can cause an artifact? | Delay in EDTA or poor staining |
| How do you calculate and absolute count? | Relative count (decimal) x WBC count |
| What is leukomoid reaction? | Benign leukocyte proliferation characterized by high WBC count with many circulating immature WBC precursors (left shift) |
| What is a proto-oncongene? | Good gene |
| What is an oncongene? | Malignant cell |
| What happens if you lose a tumor suppressor gene? | Cell becomes malignant |
| What morphologies could indicate malignancy? | Clustering Prominent Nucleoli Multiple Nucleoli Auer rods (myeloid only) Clonal morphology Cytoplasmic fragmentation |
| What is the major difference between acute and chronic leukemias? | Acute has more immature cells whereas chronic has more mature cells |
| What is the biggest difference between the FAB and WHO classification systems of Acute leukemias? | FAB says >30% blasts WHO says >20% blasts |
| Give the name of M0 | Acute myeloblastic Leukemia without differentiation |
| Give the name of M1 | Acute myeloblastic Leukemia with minimal differentiation |
| How do you differentiate between M0 and M1? | Flow analysis |
| Give the name and translocation of M2. | Acute myeloblastic leukemia with maturation t(8;21) |
| Give the name and translocation of M3. | Acute promyelocytic leukemia t(15;17) DIC and Auer rods |
| Give the name and translocation of M4. | Acute myelomonocytic leukemia. Inv (16) M4eo: with eosinophils |
| Give the name and translocation of M5. | Acute monocytic leukemia t(9;11) |
| Give the name M6. | Acute erythroblastic leukemia |
| Give the name and translocation of M7. | Acute megakaryblastic leukemia t(1;22) |
| At what age is there a good prognosis for ALL? | 2-8 years |
| What is L1? | Homogenous blasts Children 2-10 |
| What is L2? | Heterogenous blasts Adolescence and adults |
| What is L3? | Homogenous Large blasts Children and Adults AKA: Burkitts type leukemia |
| What are the differences between a lymphoblast and myeloblast? | Lymphoblast is smaller and has scant cytoplasm, dense chromatin, indistinct nucleoli, and NO auer rods |
| What is the importance of immunophenotyping? | To determine whether the leukemia is B or T cell origin. Very important in prgonosis. |
| Which malignancies are normally more agressive: T cell, NK cell, or B cell? | T cells and NK cells are more aggressive than B cells |
| What is the Philadelphia chromosome? | t(9;22) Associated with CML Leads to the formation of BCR-ABL fusion gene->P210 protein->increase tyrosine kinase->increase proliferation and apoptosis |
| What is blast crisis? | Nearly all myeloid cells are Ph'+ Patients usually die Most blast crisis' resemble AML |
| What are the main differences between CML and Leukomoid reaction? | CML has low LAP, RXN has high LAP CML has eosos and basos, RXN doesn't CML has Ph' and RXN doesn't |
| What is Polycythemia Vera (PV)? | Have the JAK2 mutation Overproduction of RBCs, WBCs, and PLT High Hgb, Hct, RCM |
| What is relative polycythemia? | Low plasma volume due to vomiting or dehydration |
| What is seondary polycythemia? | Increased EPO from renal disease, tissue hypoxia, pulmonary disease, or high O2 affinity Hgb |
| How do you differentiate primary and secondary polycythemia? | Primary has normal O2 and EPO Secondary has abnormal O2 and EPO |
| What is CLL? | A disease in the elderly. Has a high lymphocyte count with non functional lymphocytes. Mostly a B cell abnormality |
| What is the key morphology with CLL? | Smudge Cells |
| What is Prolymphocytic leukemia (PLL)? | A severe form of CLL |
| Which test is positive in Hairy Cell leukemia? | TRAP positive. Uses tartrate which HCL is resistant to |
| What is the difference between a leukemia and a lymphoma? | Lymphoma is in the tissues and leukemia is in the blood. |
| How do you diagnose Hodgkin Lymphoma? | Presence of Reed Sternberg cells (owl eye cells) |
| What is the translocation in Burkitt's lymphoma (Non hodgkin)? | t(8;14) C-MYC gene rearrangement |
| What are some characteristics of multiple myeloma? | Presence of rouleaux Bence jones proteinuria (light chains in urine) M spike in serum electrophoresis |
| What is Sudan Black B? | Stains cellular components containing lipids. Pos for AML, negative for ALL. Differentiates myeloid from lymphoid. |
| What is myeloperoxidase (MPO)? | Sensitive and specific for granulocytes. Differentiates blast of AML (pos) and ALL (neg). Faster than SBB but reagent is carcinogenic |
| What reagent is used in Specific esterase? | Chloroacetate esterase (M4 and M5 pos) |
| What reagent is used in Non Specific esterase? | Alpha-naphthyl esterase (M4 pos) |
| What is Periodic Acid Schiff (PAS)? | Glycogen causes a pos PAS which makes ALL postive and AML negative. |
| What is Terminal deoxynucleotidyl transferase? | Recognized as a marker for B and T primitive lymphs. ALL pos, AML neg (usually) |
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roni_robinson56
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