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Heme Part 1
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
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
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
2 mins
Hemochromatosis Pathophysiology
Pathophysiology
Hemosiderin Deposition
Autosomal Recessive
HLA-A3
Secondary to Transfusion Therapy
Increase Iron
Increase Ferritin
Increased Transferrin Saturation
2 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
Hereditary Spherocytosis Disease
Northern Europeans
Mechanisms
Autosomal Dominant
Spectrin/Ankyrin Deficiency
Spherocyte Formation
Hemolytic Anemia
Symptoms
Bilirubin Gallstones
Jaundice
Splenomegaly
3 mins
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
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
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
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Pathophysiology
Defective PIG-A Gene
Deficiency of DAF (Decay Accelerating Factor CD55)
Symptoms
Dark Urine in the Morning
Intravascular Hemolysis
Thrombosis
Diagnosis
Decreased CD55 and CD59
Treatment
RBC Transfusion
Eculizumab
Bone Marrow Transplant is Curative
3 mins
Polycythemia Vera Disease
Pathophysiology
JAK2 Mutation leading to increased red blood cells
Tyrosine Kinase
Symptoms
Erythromelalgia
Pruritus
Often After Hot Shower
Headache
Treatment
Phlebotomy
Aspirin
1 min
Polycythemia Vera Labs
Lab Findings (The 4 H's)
Hypervolemia
Histaminemia
Hyperviscosity
Hyperuricemia
Diagnosis
Elevated Hemoglobin or Hematocrit
Positive Jak2 Mutation
1 min
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
Sickle Cell Anemia (Management)
Long Term Treatment
Hydroxyurea
Bone Marrow Transplant
Folate
Acute Treatment
Exchange Transfusion
Prophylaxis
Penicillin Prophylaxis
1 min
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
Thrombotic Thrombocytopenic Purpura (TTP)
Inhibition or deficiency of ADAMTS 13
vWF Multimers are Not Degraded
Microthrombi (and Emboli)
Symptoms
FAT RN Pentad
Fever
Anemia
Thrombocytopenia
Renal Dysfunction
Neurologic Abnormalities (Altered Mental status)
Treatment
Plasmapheresis
Corticosteroids
Splenectomy
2 mins
Von Willebrand Disease
Mixed platelet and coagulation disorder
Mechanism
Autosomal Dominant Condition Leading to Reduced vWF
Defect in platelet plug formation
Labs
Increased Bleeding Time
PTT increased
Factor VIII decreased
Normal platelet count
Treatment
DDAVP (synthetic vasopressin)
1 min
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