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Heme Final Part 2

MLS 310 with Ismail El-Amouri

QuestionAnswer
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)
Created by: roni_robinson56