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Blood Heme
Hemoglobin
Hemoglobin Has Four Iron Hemes
Lots Of Hemoglobin In Red Blood Cells
High O2 Levels Increase O2 Binding
High Temperature Reduces O2 Binding
High CO2 Levels Reduce O2 Binding
Low pH Reduces O2 Binding
Myoglobin Binds Oxygen in Muscle
2 mins
Pyruvate Kinase Deficiency
Pathophysiology
Autosomal Recessive
Defect in Pyruvate Kinase
Decreased ATP
Extravascular Hemolysis
Presentation / Symptoms
Hemolytic Anemia in a Newborn
Diagnosis
RBC Enzyme Assay
Echinocytes
Burr Cells
Small Uniform Projections
Normocytic Anemia
2 mins
Hereditary Spherocytosis Disease
Northern Europeans
Mechanisms
Autosomal Dominant
Spectrin/Ankyrin Deficiency
Spherocyte Formation
Hemolytic Anemia
Symptoms
Bilirubin Gallstones
Jaundice
Splenomegaly
3 mins
Hereditary Spherocytosis Diagnosis & Treatment
Labs
Increased MCHC
Spherocytes
Normocytic Anemia
Diagnosis
Eosin-5-Maleimide (EMA) Binding Test
Osmotic Fragility Test
Glycerol Lysis Test
Treatment
Folic Acid
Splenectomy
2 mins
G6PD Mechanism
Turns NADP+ to NADPH
NADPH Used by Glutathione Reductase
Detoxifies Free Radicals and Peroxides
X-linked Recessive
2 mins
G6PD Deficiency
X-linked Recessive
Hemolytic Anemia
Inflammatory Response
Fava Beans
Sulfonamides
Primaquine
Anti TB Drugs
Heinz Bodies
Bite Cells
Prevalent Among African Americans due to Increased Malarial Resistance
Cis: Increased risk of sv. anemia in offspring | South Asians
Trans: mild anemia | West Africa
HbH Disease/Intermedia: 3 deleted genes
4 Beta-globin chains
Heinz Bodies: Beta-globin Tetramers
Hemolytic Anemia, Splenomegaly
Hb Barts: 4 deleted genes
4 Gamma-globin chains
Hydrops Fetalis
Beta Thalassemia
Mechanism
Microcytic, Hypochromic Anemia
Mediterranean Populations
Decreased Beta-Globin
Beta-Thalassemia Minor
Increased HbA2
No Intervention
Beta-thalassemia Major
Blood Transfusions
Hemochromatosis
Crew-cut on Skull X-Ray
Diagnosis
Electrophoresis
2 mins
Anemia Lab Values
Iron Deficiency Anemia
Serum Iron Decreased
Transferrin Increased
Ferritin Decreased
% Transferrin Saturation Decreased
Pregnancy and OCP Use
Serum Iron Normal
Transferrin Increased
Ferritin Normal
% Transferrin Saturation Decreased
Anemia of Chronic Disease
Serum Iron Decreased
Transferrin Decreased
Ferritin Increased
% Transferrin Saturation Decreased or Normal
Hemochromatosis
Serum Iron Increased
Transferrin Decreased
Ferritin Increased
% Transferrin Saturation Increased
3 mins
Normocytic Hemolytic Anemia Causes
Intrinsic Hemolytic
Hereditary Spherocytosis
RBC Enzyme Deficiency
Hemoglobin C Defect
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Sickle Cell Anemia
Extrinsic Hemolytic
Autoimmune
Microangiopathic
Mechanical Destruction
Prosthetic Cardiac Valves
Infection
Snake Venom
2 mins
Normocytic Nonhemolytic Anemia Causes
MCV 80-100
Nonhemolytic
Anemia of Chronic Disease/Inflammation
Aplastic Anemia
Chronic Kidney Disease
Early Iron Deficiency
1 min
Macrocytic Anemia Causes
MCV > 100
Megaloblastic
B12 Deficiency
Folate Deficiency
Orotic Aciduria
Non Megaloblastic
Alcoholism
Liver Disease
Reticulocytosis
2 mins
Microcytic Anemia Causes
MCV < 80
Causes
Thalassemias
Chronic Disease or Illness
Lead Toxicity
Late Iron Deficiency
Sideroblastic Anemia
1 min
Sideroblastic Anemia
Sideroblastic anemia
Defect in Heme Synthesis
X-linked defent in delta-ALA synthase gene
Decreased TIBC
Increased ferritin
Ringed sideroblasts
Basophilic stippling
C
Caused by Vitamin B6 deficiency
Caused by Copper deficiency
Isoniazid
Treat with pyridoxine
Microangiopathic Hemolytic Anemias
DIC
TTP
HUS
HELLP Syndrome
Iron Deficiency Anemia
Causes
Malnutrition/Malabsorption
Hemorrhage
Signs
Microcytic, Hypochromic Anemia
Decreased Heme Synthesis
Labs
Decreased Reticulocytes
Decreased Ferritin
Increased Red Cell Distribution Width (RDW)
Poikilocytosis
Anisocytosis
Increased Central Pallor
2 mins
Sickle Cell Anemia (Mechanism)
Intrinsic Normocytic Hemolytic Anemia
Point Mutation
Autosomal Recessive
African American
Dehydration or Decreased O2
Newborns Asymptomatic
Heterozygote Malarial Resistance
2 mins
Sickle Cell Anemia (Signs and Complications)
Signs
Crew-cut on Skull X-ray
Complications
Auto-splenectomy
Painful Crisis
Aplastic Crisis
Splenic-sequestration Crisis
Hyper-hemolytic Syndrome
Renal Papillary Necrosis
Salmonella osteomyelitis
Pulmonary Hypertension
Hyposthenuria
2 mins
Sickle Cell Anemia (Management)
Long Term Treatment
Hydroxyurea
Bone Marrow Transplant
Folate
Acute Treatment
Exchange Transfusion
Prophylaxis
Penicillin Prophylaxis
1 min
Vitamin B6 (Pyridoxine)
Pyridoxal Phosphate
Mechanism
Transamination
Decarboxylation
Glycogen Phosphorylase
Cystathionine Synthase
Functions
Heme Synthesis
Niacin Synthesis
Neurotransmitter Synthesis
2 mins
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