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Heme/Onc Part 1
Blood Types and Compatibilities
ABO Compatibility
No Antigens (Type O Blood)
Universal Donor
A Antigen (Type A Blood)
B Antigen (Type B Blood)
AB Antigen (Type AB Blood)
Universal Recipient
Rhesus (Rh) Compatibility
Rh (Rhesus) Antigen
Never Rh+ to Rh-
Considerations
Blood Transfusion
Obstetric Patient
2 mins
Coagulation Cascade Overview
Secondary Hemostasis
Intrinsic Pathway
Partial Thromboplastin Time (PTT)
Heparin
Extrinsic Pathway
Prothrombin Time (PT)
Warfarin
Common Pathway
Thrombin
Convert Fibrinogen to Fibrin
Stabilizes the Platelet Plug
2 mins
Microcytic Anemia Causes
MCV < 80
Causes
Thalassemias
Chronic Disease or Illness
Lead Toxicity
Late Iron Deficiency
Sideroblastic Anemia
1 min
Normocytic Nonhemolytic Anemia Causes
MCV 80-100
Nonhemolytic
Anemia of Chronic Disease/Inflammation
Aplastic Anemia
Chronic Kidney Disease
Early Iron Deficiency
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
Macrocytic Anemia Causes
MCV > 100
Megaloblastic
B12 Deficiency
Folate Deficiency
Orotic Aciduria
Non Megaloblastic
Alcoholism
Liver Disease
Reticulocytosis
2 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
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
Folate Deficiency
Clinical Features
Macrocytic Anemia
Megaloblastic Anemia
Etiologies
Malabsorption
Dietary
Tea and Toast
Alcoholics
Medication-induced
Methotrexate
Trimethoprim
Increased Requirement
Hemolytic Anemia
Pregnancy
2 mins
Orotic Aciduria
Pathophysiology
Autosomal Recessive
Defect in UMP Synthase
De Novo Pyrimidine Synthesis Pathway
Symptoms
Increased Orotic Acid in Urine
Megaloblastic Anemia
No Response to B12 or Folate
Non-Hyperammonemic
Failure to Thrive
Treatment
Oral Uridine Monophosphate
2 mins
Vitamin B12 (Cobalamin) Mechanism and Deficiency
Mechanism
Cobalamin
Large reserve pool in the liver
Homocysteine to methionine
Methylmalonyl CoA to succinyl CoA
Deficiency Signs and Symptoms
Macrocytic Megaloblastic Anemia
Hypersegmented Neutrophils
Neurologic defects
Posterior column
Lateral corticospinal tract
Spinocerebellar tract
5 mins
Vitamin B12 (Cobalamin) Deficiency Causes
Causes
Vegans
Pernicious Anemia
Gastric Bypass Surgery
Malabsorption
Sprue
Enteritis
Crohn's Disease
Diphyllobothrium latum
4 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 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
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
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
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
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