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Final Units 3-5 Heme

Hematology Final for El Amouri

QuestionAnswer
What is the cause of Iron Deficiency Anemia (IDA)? It is a deficiency of iron due to malnutrition, malabsorption, or blood loss
What is the cause of Anemia of Chronic Diseases (ACD)? Iron is blocked from reaching the RBC precursors due to impaired release from stores
The stain used to view unused iron Prussian blue
What is the cause of Sideroblastic Anemia (SA)? Iron leaves the stores but cannot be inserted to protoporphyrin ring. It accumulates in the mitochondria and forms ringed sideroblasts.
What are the three progressions (stages) of IDA? Stage 1: Iron store depletion Stage 2: Iron deficient erythropoiesis Stage 3: Iron deficiency anemia
What is the red cell morphology for stage 1 IDA? Normocytic normochromic
What is the red cell morphology for stage 3 IDA? Microcytic hypochromic
What are the iron studies for IDA? (ferritin, serum iron, TIBC, and % saturation) Ferritin: decreased Serum iron: decreased TIBC: increased Transferrin saturation: decreased
What are the iron studies for ACD? (ferritin, serum iron, TIBC, and % saturation) Ferritin: normal-increased Serum iron: decreased TIBC: normal-decreased Transferrin saturation: normal-decreased
What are the iron studies for SA? (ferritin, serum iron, TIBC, and % saturation) Ferritin: normal-increased Serum iron: normal-increased TIBC: normal-decreased Transferrin saturation: increased
Primary megaloblastic anemia Nutritional deficiency of B12 or folate that leads to impaired DNA synthesis. Causes impaired nuclear maturation and cell division.
Secondary megaloblastic anemia Due to malabsorption condition such as pernicious anemia where there is a defect in HCl secretion and Intrinsic Factor (IF)
Cell types affected by megaloblastic anemia All cell types (pancytopenia)
Nuclear cytoplasm asynchrony Nuclear maturation defect characterized by high N:C ratio; immature nucleus with mature cytoplasm. Usually results in ineffective erythropoiesis.
Non-megaloblastic anemia Unrelated to nutritional deficiency. Characterized by high liver enzymes from liver disease or alchoholism.
Peripheral blood findings in megaloblastic anemia Pancytopenia Macrocytic normochromic cells Macro-ovalocytes Anisocytosis Hypersegmented neutrophils HJ bodies Ineffective erythropoiesis
BM findings in megaloblastic anemia Marked N:C asynchrony
Chemistry findings in megaloblastic anemia Decreased serum folate and/or B12 Increased MMA Increased homocysteine
Laboratory findings in non-megaloblastic anemia Round macrocytes No hypersegmented neutrophils Normal WBC, and PLT Normal B12 and folate Normal MMA and homocysteine
Intravascular hemolytic anemia Occurs within blood vessels Increased schistocyes Increased serum bilirubin Decreased haptoglobin Hemoglobinuria
Extravascular hemolytic anemia Occurs mainly in spleen Increased spherocytes Increased serum bilirubin Hepatosplenomegaly
What is the job of haptoglobin in intravascular hemolysis? To transport and elminate serum bilirubin
How is bilirubin eliminated in extravascular hemolysis? By the spleen
DAT seperates hemolytic anemias into what two groups? Immune-mediated (allo or auto immune) or non-immune (inherited)
Compensated hemolytic anemia No anemia unless RBC destruction exceeds production
What are the three types of intrinsic RBC defects? Membrane protein defects Enzyme defects Hgb (globin chain) defects
What are the intrinsic membrane protein defect HA's? Hereditary spherocytosis (HS) Hereditary elliptocytosis (HE) Hereditary stomatocytosis PNH (CD55 and CD59)
What are the intrinsic enzyme defect HA's? Glucose-6-phosphate dehydrogenase(G6PD Pyruvate kinase (PK) deficiency
What is hereditary spherocytosis (HS)? Defect in vertical proteins. Shows elevated number of spherocytes and MCHC <36%.
What is the diagnostic test for HS? Osmotic fragility test for spherocytes and negative DAT
What is hereditary elliptocytosis and it's severe form? Horizontal protein defect. When fragmentation begins to occur it is called hereditary pyropoikilocytosis (HPP).
What is hereditary stomatocytosis? Defect in membrane protein stomatin which helps regulate ions across the red cell channel
What two types of hereditary stomatocytosis are there? Overhydrated: Excessive gain of Na+ and water rushes in causing uncompensated HA Dehydrated: Excessive loss of Na+ and water rushes out causing compensated HA
What is PNH? Defect in GPI anchor for complement regulator proteins CD55 and CD59. C' reg. proteins missing = C' activated, cells destroyed
Tests for diagnosing PNH Sucrose hemolysis (sugar water), Ham's (acid serum) and flow analysis
What is G6PD deficiency? Causes impaired generation of glutathione in reduced state (GSH) that helps to prevent oxidative damage to Hgb. Causes denatured, oxidized Hgb forming Heinz bodies.
What type of stain is used to view Heinz bodies? Supravital stain
What will the blood film of G6PD deficient patient look like? Bite cells and blister cells
What is a source of error in testing for G6PD deficiency? Reticulocytosis because retics have high G6PD activity causing false increase in levels
DAT tests for what antibodies associated with HA? Autoimmune antibodies (Warm or cold)
IAT tests for what antibodies associated with HA? Alloimmune antibodies (transfusion reaction or hemolytic disease of the newborn - HDN)
Warm and cold AIHA are associated with which HA? Autoimmune hemolytic anemia
Amino acid substitution for Hgb S B6(Glu) -> (Val)
Amino acid substitution for Hgb C B6(Glu) -> (Lys)
Amino acid substitution for Hgb E B26(Glu) -> (Lys)
Is Hgb SA sickle cell anemia or trait? Trait; asymptomatic
Is Hgb SS sickle cell trait or anemia? Anemia (also known as sickle cell disease)
Is Hgb S soluble in the deoxygentated state or the oxygenated state? Oxygenated
Under what conditions does Hgb S turn into insoluble aggregates? Under deoxygenated, deoxyhemoglobin state; Hgb S polymerizes
What clinical condition is the hallmark manifestation of sickle cell disease? Vaso-occlusive crisis; causes very intense pain termed "body biting"
Deterioration of tissues from recurring vaso-occlusive episodes can cause what two things? Autosplenectomy and overwhelming infections
Screening test for sickle cell and it's reagent Solubility test - Sodium Dithionite (reducing agent that takes O2 from cells and causes sickling = turbidity)
Major difference in Thalassemia and Hemoglobinopathies? Thalassemia is decreased or absent synthesis of structurally NORMAL globin chains. Hgbopathies are synthesis of structurally ABNORMAL globin chains.
Thalassemias are characterized by what RBC morphology and iron status Microcytic hypochromic and NORMAL iron status
Types of alpha Thalassemia Intermedia (Hgb H) [4 beta chains] Major (Hgb Barts/Hydrops Fetalis) [4 gamma chains]
Which alpha Thalassemia is incompatible with life? Hydrops Fetalis
Type of beta Thalassemia Major (Cooley's or Mediterranean anemia) [Little or no Beta chain synthesis; Majority are Hgb F]
Classification of anemia deals with stem cell defects Aplastic anemia (Fanconi anemia) causing hypocellular BM and pancytopenia
Classification of anemia that deals with progenitor cell defects Pure red cell aplasia (PRCA) where there is failure to produce erythroid progenitor cells but WBC's and PLT's are normal [Diamond Blackfan syndrome]
Classification of anemia dealing with bone marrow replacement Myelophthisic anemia
Stages of neutrophil development Myeloblast Promyelocyte Myelocyte Metamyelocyte Band Neutrophil
First stage of neutrophil development incapable of division Metamyelocyte
Which pool are dividing cells located? Mitotic pool
Which pool are non-dividing cells located? Post-mitotic pool
Where do cells travel in the event of infection? Directly from marginal pool to tissues
Formula for absolute count of WBC's Absolute count = Relative count (in decimal)*WBC count
Is LAP higher in Leukemoid reaction or CML Leukemoid reaction
Is Philidelphia chromosome associated with CLL or CML? CML - Chronic Myeloid Leukemia
Causes malignancies and abnormal proliferation of cells after mutation Proto-oncogene -> Oncogene
Causes inability to suppress malignant clones of cells Loss of a tumor suppressor gene
Are Auer rods found in lymphoid or myeloid leukemias? Myeloid leukemia
Organization classifies leukemia by someone having >30% blasts and relies on morphology French-American-British (FAB)
Organization classifies leukemia by someone having >20% blasts and relies on immunophenotyping The World Health Organization (WHO)
AML classified by t(8;21) M2: Acute Myeloblastic Leukemia with maturation
AML classified by t(15;17) and multiple Auer rods M3: Acute Promyelocytic (PG) Leukemia
AML classified by Inv (16) M4: Acute Myelomonocytic leukemia
AML classified by t(9;11) M5: Acute Monocytic leukemia
M6 also goes by what other name? Acute Erythroblastic leukemia
AML classified by t(1;22) Acute Megakaryoblastic leukemia
ALL classification with homogenous population of blasts and seen in children 2-10 years old L1
ALL with heterogeneous population of blasts seen in adolescents and adults L2
Burkitt's type leukemia with homogenous populations of LARGE blasts; seen in adults and children L3
Importance of immunophenotyping in ALL To determine B or T cell origin; B cell has better prognosis and T cells more aggressive
Most important prognostic indicators of ALL Age WBC count Cell type/Lineage
MPO, SBB, Specific esterase (SE) and NSE are all positive for which leukemia? Acute Myeloid Leukemia (AML/ANLL) [SE pos for granuloblast; NSE pos for monoblast]
MPO, SBB, Specific esterase (SE) and NSE are all negative for which leukemia? Acute Lymphoid Leukemia (ALL)
What is cytochemical in NSE? a-naphthyl Butyrate/Acetate
TdT and PAS usually negative for what? Acute Myeloid Leukemia (AML/ANLL)
TdT and PAS usually positive for what? Acute Lymphoid Leukemia (ALL)
Chronic myelogenous/granulocytic leukemia is characterized by what? Marked leukocytosis (>50,000/ul) and includes all three granulocytes maturation stages [immature granulocytes] (VS leukemoid with only neutrophils)
Philidephia (Ph') chromosome deals with which leukemia and is what translocation? CGL and t(9;22)
What are other names for Ph' chromosome? BCR-ABL fusion gene P210 protein Philidelphia chromosome Chronic granulocytic leukemia
Blast crisis Where CGL progresses to produce 90% blasts and resembles AML. Must know of previous CML condition and patients usually die.
What mutation do Polycythemia Vera (PV) patients have? JAK2 mutation
What is primary PV? Polycythemia Vera; overproduction of all cells (no correlation with EPO)
Relative PV Decreased plasma volume from vomitting, diarrhea, dehydration
Secondary PV Appropriate and inappropriate increase in EPO; O2 elevated and EPO abnormal; associated with: Renal disease Tissue hypoxia Pulmonary disease High O2 affinity Hgb
What is Chronic Lympohocytic Leukemia (CLL)? Overproduction of non-functional lymphs; immune system doesn't respond properly to infection
Hallmark blood film characteristic of absolute lymphocytosis patient Smudge cells
Owl eyes are characteristic of what lymphoma? Hodgkin Lymphoma (HL)
Difference between leukemia and lymphoma Lymphoma is solid tumor involving lymphatic tissue Leukemia is liquid tumor where malignant cells circulate
Burkitt lymphoma with C-MYC gene rearrangement [t(8;14)] Non-hodgkin Lymphoma (NHL) - Similar to L3
Aggressive form of CLL Prolymphocytic leukemia (PLL)
TRAP positive leukemia Hairy Cell Leukemia (HCL)
What is TRAP? Tartrate resistent acid phosphatase stain for HCL
Presence of Rouleaux Bence Jones Proteinuria M spike Multiple Myeloma (plasma cell myeloma)
Created by: 1078269716