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132 Cards in this Set

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What blood cell?
Bilobed nucleus
Basophils & eosinophils
What blood cell?
Involved in type I hypersensitivity
Mast cell
What blood cell?
Important in defense against helminths & protozoans
Eosinophil
Uses Major basic protein
What blood cell?
Polymorphonuclear leukocyte
Mainly describes Neutrophils
Also technically includes basophils & eosinophils
What blood cell?
Kidney shaped nucleus
Monocyte
What blood cell?
"Frosted glass" cytoplasm
Monocyte
What blood cell?
Cell markers are CD19 & CD20
B lymphocyte
What blood cell?
Cell markers are CD3 & CD4
T helper cell
What blood cell?
Cell markers are CD3 & CD8
Cytotoxic T cell
What blood cell?
Clock face chromatin distribution
Plasma cell
What blood cell?
Professional APC's
Dendritic cell
What blood cell?
Specialized dendritic cell in the skin
Langerhans cell
What factors make up the intrinsic pathway?
XII▶XI▶IX▶X(Common pathway)
What factors make up the extrinsic pathway?
Tissue factor & VII ▶ X(Common pathway)
What factors make up the common pathway?
X converts prothrombin to thrombin, which converts fibrinogen to fibrin, which is then cross linked by XIII
What is factor III more commonly known as?
Tissue factor
What is factor II more commonly known as?
Prothrombin
What is factor I more commonly known as?
Fibrinogen
What factor increases the speed of factor IX activation of factor X?
Factor VIII
What actions does Thrombin perform?
Converts fibrinogen to fibrin
Activates factors V, VIII, XI & XIII
What factor increases the speed of factor X conversion of prothrombin to thrombin?
Factor V
What factor cross links the fibrin clot?
Factor XIII
What conversions require Calcium?
Activation of factor IX, factor X, factor XIII, Prothrombin & Fibrinogen
What do Proteins C & S do?
Inactivate factors V & VIII
What does antithrombin III do?
Inactivates thrombin, IX, X & XI
What does tPA do?
Generates Plasmin, which cleaves fibrin
What is factor XII more commonly known as?
Hageman factor
What are the Vitamin K dependent factors?
II(Prothrombin), VII, IX & X
How does Heparin work?
Binding & activation of antithrombin III
This RBC form indicates?
Spherocyte
Hereditary spherocytosis, autoimmune hemolysis
This RBC form indicates?
Elliptocyte
Hereditary elliptocytosis
This RBC form indicates?
Macro-ovalocyte
Megaloblastic anemia, marrow failure
This RBC form indicates?
Helmet cell
DIC, traumatic hemolysis
This RBC form indicates?
Schistocyte
DIC, traumatic hemolysis
This RBC form indicates?
Teardrop cell
Myeloid metaplasia w/ myelofibrosis
This RBC form indicates?
Acanthocyte
Spiny appearance in abetalipoproteinemia
This RBC form indicates?
Target cell
HbC disease, Asplenia, Liver disease, Thalassemia
This RBC form indicates?
Poikilocyte
Nonuniform shapes in TTP/HUS, microvascular damage, DIC
This RBC form indicates?
Burr cell
TTP/HUS
When do you see basophilic stippling?
Thalassemias, Anemia of chronic disease, Iron deficiency anemia & Lead poisoning
What type of anemia?
Iron deficiency
Microcytic anemia
What type of anemia?
Thalassemias
Microcytic anemia
What type of anemia?
Lead poisoning
Microcytic anemia
What type of anemia?
Sideroblastic anemia
Microcytic anemia
What type of anemia?
Anemia of chronic disease
Microcytic anemia or Normocytic anemia
What type of anemia?
Hemolytic anemia
Normocytic anemia
What type of anemia?
Acute hemorrhage
Normocytic anemia
What type of anemia?
Renal disease
Normocytic anemia
What type of anemia?
Bone marrow disorders
Normocytic anemia
What type of anemia?
G6PD deficiency
Normocytic anemia
What type of anemia?
Sickle cell anemia
Normocytic anemia
What type of anemia?
B12 deficiency
Macrocytic megaloblastic anemia
What type of anemia?
Folate deficiency
Macrocytic megaloblastic anemia
What type of anemia?
Liver disease
Macrocytic non-megaloblastic anemia
What type of anemia?
Myelodysplasia
Macrocytic megaloblastic anemia
What viruses can cause aplastic anemia?
Parvovirus B19, EBV, HIV
What environmental exposures/drugs can cause aplastic anemia?
Radiation, benzene, chloramphenicol, alkylating agents, antimetabolites
What mutation has occured in sickle cell anemia?
Single amino acid replacement in beta chain
What mutation has occured in alpha-thalassemia?
Deletion of the alpha globin gene; There are 4 genes and the severity depends on the number of genes deleted
What mutation has occured in beta-thalassemia?
Point mutation causing decreased/no expression of the beta globin gene
What is HbH?
Hemoglobin that is a tetramer of beta globin chains; seen in alpha-thalassemia w/ 3/4 genes deleted
Causes Heinz bodies
What is HbBarts?
Hemoglobin that is a tetramer of gamma globin chains; seen in alpha-thalassemia w/ 4/4 genes deleted
What is HbC?
The mutated form of alpha-2/beta-2 hemoglobin seen in a milder form of sickle cell anemia
What is HbS
The mutated form of alpha-2/beta-2 hemoglobin seen in sickle cell anemia
What is a warm agglutinin?
IgG autoantibody that causes anemia
What is a cold agglutinin?
IgM autoantibody that causes anemia
What causes warm agglutinins?
SLE, CLL, certain drugs(e.g. alpha-methyldopa)
What causes cold agglutinins?
Mycoplasma pneumoniae or infectious mononucleosis
What is erythroblastosis fetalis?
When mother's antibodies attack fetal RBC's due to Rh or other blood antigen incompatibility
What defect causes spherocytosis?
Defect in spectrin or ankyrin
How is hereditary spherocytosis confirmed?
Osmotic fragility test
What is a direct Coombs' test?
anti-Ig Ab is added to patient's RBC's to see if Ig's are attached to RBC's
What is an indirect Coombs' test?
Normal RBC's are added to patient's serum to determine if anti-RBC surface Ig's are present in the serum
Pluripotent stem cells differentiate into what?
Either Mixed myeloid progenitor cells OR Lymphoid stem cells
What is made by the Mixed myeloid stem cells?
Basophils, Neutrophils, Monocytes, Eosinophils, Erythrocytes & Megakaryocytes/platelets
What is made by the Lymphoid stem cells cells?
NK cells and B/T lymphocytes
What is the growth factor for Neutrophils
G-CSF
What is the growth factor for Monocytes?
M-CSF
What is the growth factor for Eosinophils?
IL-5
What is the growth factor for Erythrocytes?
IL-3 and Erythropoietin
What is the growth factor for Megakaryocytes?
Thrombopoietin
What is the growth factor for T Cells?
IL-7
What is the growth factor for B cells?
IL-7
GPIa/IIa
Receptor that allows platelets to bind to collagen
GPIb/IX
Binds platelets to vWF multimers
GPIIb/IIIa
Binds platelets to collagen, fibrinogen and vWF multimers
Bernard Soulier Syndrome
Deficiency of GPIb/IX/V
Triad of large platelets, moderate thrombocytopenia and a prolonged bleeding time
Autosomal recessive
Glanzmann’s disease
Deficiency of GPIIB/IIIa
Autosomal recessive
Type 1 Von Willebrand disease
↓ quantity of vWF
Type 2 Von Willebrand disease
Qualitative defect of vWF
Type 3 Von Willebrand disease
Absent vWF
Genetics of Von Willebrand disease
Mostly autosomal dominant, but rarely autosomal recessive
Coded on chrom 12
Hemophilia A
a.k.a. Classic Hemophilia
Factor VIII deficiency
Platelets <30% of normal
Hemophilia B
a.k.a. Christmas Disease
Factor IX deficiency
Platelets <1% of normal
Cyclic Neutropenia
defect in neutrophil elastase
Severe Congenital Neutropenia
a.k.a. Kostmann Disease
defect in neutrophil elastase (more severe defect than in Cyclic Neutropenia)
Shwachman-Diamond Syndrome
Classic Triad: Neutropenia, exocrine pancreatic insufficiency and skeletal abnormalities
↑ apoptosis of bone marrow cells via Fas(pro-apoptotic)
Myelokathexis
↑ apoptosis of neutrophils via bcl-x (anti-apoptotic that gets down regulated
Reticular Dysgenesis
Failure of stem cell committed to myeloid and lymphoid development
Leukocyte Adhesion Deficiency Type I (LAD I)
Defect in CD18 (beta subunit) of mac-1; defect in adhesion and ingestion
Auto. Recessive
Chediak-Higashi Syndrome
Mutant lysosomal trafficking protein (CHS or LYST) leads to ↓ transfer of lysosomal enzymes to phagocytic vacuoles; defect in chemotaxis and degranulation
Auto. Recessive
Chronic Granulomatous Disease
Functional defect in oxidative metabolism (no respiratory burst)
Susceptibility for catalase-positive organisms
2/3 X-link; 1/3 Auto Recess
Disease a/w Burkholderia and Serratia infections
Chronic Granulomatous Disease
Test for Chronic Granulomatous Disease
Nitro Blue Tetrazolium Test
Will be negative in CGD
This RBC form indicates?
Pencil cell
Iron deficiency
Uncommon manifestation
This RBC form indicates?
Basket cell
Oxidative damage (G6PD deficiency); unstable hemoglobin
Diamond-Blackfan
Congenital red cell aplasia
Intrinsic defect of erythroid progenitor cells
Autosomal dominant
Drugs that can cause red blood cell aplasia
Chloramphenicol, Chemotherapy drugs, Benzene
Infectious causes of red blood cell aplasia
Parvovirus B19; Transient Erythroblastopenia of childhood (Catch all for all non-parvo viruses causes)
What molecule becomes built up if you are deficient of folate?
Homocysteine(converted to methionine by methyl THF)
Where is B12 absorbed?
Distal ileum
What molecule becomes built up if you are deficient of B12?
Methylmalonic acid
What causes Sideroblastic anemia?
ALA synthase deficiency; leads to diminished production of protoporphyrin rings which leads to diminished heme
What enzymes does lead poisoning inhibit?
ALA dehydratase, Ferrochelatase, Pyrimidine-5’nucleotidase
What is haptoglobin?
Molecule that binds to free Hgb to remove it from the blood upon lysis or RBC's
↓ in intravascular hemolysis
Where is the Alpha globin gene?
Chrom 16; 2 copies on each chromosome
Where are Beta, delta & gamma globulin genes?
Chrom 11; 1 copy on each chromosome
When does HbA become the predominant form of Hgb instead of HbF
By age 3-6 months
Sickle cell patients are susceptible to what infections?
Encapsulated organisms
Streptococcus pneumonia, Haemophilus influenza, and Neisseria meningitides
What causes aplastic crisis in sickle cell patients?
Parvovirus B19
Causes bone marrow to stop production of RBC's
Since RBC life span is shortened in sickle cell, pt ends up w/ no RBC's
Why do you give Hydroxyurea to sickle cell patients?
Increases concentraion of fetal Hgb
Usual mutation in alpha-thalassemia
Deletions
Usual mutation in beta-thalassemia
Point mutations (usually in promoter area)
Most common form of thalassemia in Southeast Asia
Alpha Thalassemia
Most common form of thalassemia in Mediterranean regions and in the US
Beta Thalassemia
Glucose–6 phosphate dehydrogenase (G6PD) deficiency
X-linked recess(point muta.)
Anemia from RBC inability to respond to oxidative stress
What is a heinz body?
Intracellular inclusions of denatured hemoglobin
a/w thalassemias, sickle cell, G6PD, splenectomy
What sets off a G6PD deficiency?
Infections, Drugs(sulfa's & anti-malarials are most common ones), Fava beans, moth balls(naphthalene)
Hereditary Spherocytosis
Defect of RBC membrane skeletal protein(spectrin, ankyrin or pallidin)
autosomal dominant
What kind of hemolysis do you get w/ IgG autoimmune hemolytic anemia?
Extravascular hemolysis
What kind of hemolysis do you get w/ IgM autoimmune hemolytic anemia?
Intravascular hemolysis
What Ig is involved in Hemolytic disease of the newborns?
IgG