Red Blood Cells And Bleeding Disorders Flashcards
Average content (mass) of hemoglobin per red cell expressed in picograms
Mean Cell Hemoglobin
Average volume of a red cell expressed in femtoliters
Mean Cell Volume
Average concentration of hemoglobin in a given volume of packed red cells expressed in grams per deciliter
Mean Cell Hemoglobin Concentration
Coefficient of variation of red cell volume
Red Cell Distribution Width
Inc RDW
Dec RDW
Iron deficiency
Thalassemia
Hemolytic Anemias
Hereditary spherocytosis G6PD deficiency Sickle cell anemia Thalassemia Autoimmune HA Microangiopathic HA Macroangiopathic HA
Mutations of ankyrin, spectrin, band 3 and band 4.2
Hereditary spherocytosis
Hemolytic anemia
Aplastic crisis
Hemolytic crisis
Parvovirus B19
EBV mononucleosis
Small dark nuclear remnants present in RBCs of asplenic patients
Howell Jolly bodies
Hereditary spherocytosis
Symptoms of extravascular hemolysis
Anemia
Splenomegaly
Jaundice
Symptoms of intravascular hemolysis
Anemia Hemoglobinemia Hemoglobinuria Hemosiderinuria Jaundice
Episodic hemolysis
G6PD deficiency
Triggers of G6PD deficiency
INFECTIONS
Drugs antimalarials sulfonamides
Fava beans
Membrane bound precipitates on denatured globin chains
Heinz bodies
G6PD def
RBCs with damaged membranes removed by splenic macrophages
Bite cells
Recovery phase of G6PD deficiency heralded by
Reticulocytosis
6th codon of B globin: glu to val
Sickle cell anemia
RBCs shaped like curved blades
Sickled cells
Sickle cell anemia
Reticulocytosis
Howell Jolly bodies
Trapping of sickled red s leading to splenic infarction
Autosplenectomy
Sickle cell anemia
Types of crisis in Sickle cell anemia
Vasoocclusive
Sequestration
Aplastic
Chronic tissue hypoxia Hyposthenuria - cannot concentrate urine Infections Prominent cheekbones Crewcut skull appearace Chronic hyperbilirubinemia
Sickle Cell Anemia
Sickle cell anemia
Initial dx test
Periph blood smear
Sickle cell anemia
Gold std
Hb electrophoresis
Sickle cell anemia
DOC
Hydroxyurea
Alpha thalassemia 1 2 3 4
Silent carrier state
A thalassemia trait
Hemoglobin H disease
Hydrops fetalis
Beta thalassemias
Transfusion dependence. HbF
Severe but no transfusion
Asx
Major cooley’s anemia
Intermedia
Minor
Dehydrated RBCs with bull’s eye appearance
Target cells or codocytes
Thalassemia
Basophilic stippling
Anisocytosis
Poikilocytosis
Microcytic hypochromic anemia
Thalassemia
Crew cut skull appearance
Chipmunk facies
Hemosiderosis
Hemochromatosis
Thalassemia
Treatment for hemochromatosis
Phlebotomy
DOC for hemochromatosis
Deferoxamine chelation
Leading cause of death in PNH
Thrombocytosis
Warm agglutinin
Cold agglutinin
IgG
IgM
Autoimmune HA seen in SLE, CLL or methyldopa
Warm agglutinin
Autoimmune HA seen in Mycoplasma and mononucleosis
Cold agglutinin
Anti Ig antibody added to patient’s RBCs agglutinate if RBCs are coated with Ig
Direct Coombs’ Test
Normal RBCs added to serum agglutinate if serum has Ig
Indirect Coombs’ Test
Damaged RBCs upon passing through obstructed or narrowed vessel lumina
Cardiac valve prosthesis
Microangiopathic HA
RBCs with spikes
Burr cells or echinocytes
Microangiopathic HA
Fragmented RBCs
Halved RBCs
Schistocytes
Helmet cells
Microangiopathic HA
Intrinsic hemolysis
Extravascular site
Hereditary spherocytosis
Sickle cell anemia
Thalassemia
Intrinsic hemolysis
Both intra and extravascular
G6PD deficiency
Intrinsic hemolysis
Intravascular site
PNH
Extrinsic hemolysis
Intravascular site
Autoimmune HA
Microangiopathic HA
Macroangiopathic HA
Anemias of diminished erythropoiesis
Megaloblastic Pernicious IDA Chronic disease Aplastic Pure red cell aplasia
Macroovalocytes
Hyper segmented neutrophils
Giant metamyelocytes
Megaloblastic anemia
Autoimmune gastritis resulting to failure of intrinsic factor production
Pernicious anemia
Atrophy of fundic glands: chief and parietal
Gastric mucosa replaced by goblet cells
Beefy tongue
Demyelination of dorsal and lateral tracts of spinal cord
Intestinalization
Atrophic glossitis
Pernicious anemia
Schilling test
Pernicious anemia
Schilling test
Stage 1 normal
B12 deficiency
Schilling test
Stage 1 low
Stage 2 normal
Pernicious anemia
Schilling test
Both stages are low
Malabsorption
Iron absorbed primarily in the
Proximal duodenum
Koilonychia spoon nails
Pica
Plummer Vinson syndrome
IDA
Esophageal webs
Microcytic hypochromic anemia
Atrophic glossitis
Plummer Vinson syndrome
IL6 stimulates inc in _ causing reduced iron transfer from storage pool to BM
Hepcidin
Inc serum iron
Low TIBC
Inc ferritin
Inc transferrin sat
Hemochromatosis
Thalassemia
Low serum iron
Low TIBC
Inc ferritin
Chronic Disease
Low serum iron
Inc TIBC
Low ferritin
Low transferrin salt
IDA
Idiosyncratic effect of this drug is aplastic anemia
Chloramphenicol
Occupational chemical exposure causes aplastic anemia
Benzene
Hypocellular BM with fat cells
Hypercellular BM with fat cells
Aplastic anemia
Polycythemia vera
Anemia
Thrombocytopenia
Neutropenia
Without splenomegaly
Aplastic anemia
Non thrombocytopenic purpura due to increased fragility of cells
T
PC<100,000
Thrombocytopenia
Thrombocytopenia
Immune thrombocytopenic purpura
Thrombotic microangiopthies
Most feared complication of immune thrombocytopenic purpura
Intracranial bleeding
Thrombotic microangiopathies
Thrombotic thrombocytopenic purpura
HUS
Renal failure Anemia Thrombocytopenia Fever Neurologic deficit
Thrombotic thrombocytopenic purpura
Renal failure
Anemia
Thrombocytopenia
HUS
Platelet dysfunction
Bernard Soulier
Glanzmann’s thrombasthenia
Inc bleeding time
Dec PC
Bernard Soulier
Inc bleeding time
Normal PC
Glanzmann’s thrombasthenia
Coagulation disorders
Von Willebrand
Hemophilia
DIC
Most common inherited bleeding disorder
Treatment
Von Willebrand
Desmopressin
Most common hereditary disease with life threatening bleeding
Hemophilia A
Factor VIII
Christmas disease
Hemophilia B
Factor IX deficiency
Most specific test for DIC
D dimer
Associated with obstetrics and trauma
Acute DIC
Occurs in cancer patients
N PC and clotting factors
Chronic DIC