CM- Hem-4 - Normocytic anemias
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| Name anemai where size of erythrocytes are normal and Hgb content is normal problem is insufficient number of erythrocytes | Normocytic Anemia
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| What are the 4 distinct conditions comprising normocytic anemias | Aplastic Anemia (damage to bone marrow), posthemorrhagic anemia, hemolytic anemia, sickle cell anemia
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| How can causes of hemolytic anemias be seperated | seperated into Intrinsic and Extrinsic causes
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| What are the intrinsic causes of hemolytic anemia | MAD- Membrane Defects, Abnormal hemoglobin, or Deficient enzymes
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| What ere the extrinsic causes of hemolytic anemia | MAD- Microangiopathic, Autoimmune, and Drugs
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| What is intravascular hemolysis | where defect of the RBC results in its lysis during normal circulation
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| What are causes of intravascular hemolysis | Paroxysmal Nocturnal Hemoglobinuria, Prosthetic Heart Valves, G6PD-Glucose 6 phosphate Dehydrogenase deficiency, Disseminated intravascular coagulopathy, Antibody complement complexes
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| What type of lysis occurs in the splenic reticuloendothelial system | Extravascular hemolysis
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| What causes extravascular hemolysis | increased splenic red blood cell destruction, especially of congenital spherocytes, warm agglutinins and sickle cell disease (abnormal RBCs) remember Warm, round Genitals, sickle
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| What will your lab values show on hemolytic anemia | MCV-normal, Reticulocyte index increased, Increased indirect bilirubin, Increasd LDH, Abnormal cell morphology (schistocytes, bite cells or heinz bodies, spherocytes, acanthocytes)
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| Autosomal dominant disease where there is a membrane defect in RBC due to lack of spectrin | Congenital Spherocytosis
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| What are the s/sx of congenital spherocytosis | Splenomegaly, jaundice (due to release of indirect bilirubin)
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| What special lab will you order if you suspect pt has congenital spherocytosis | osmotic fragility test- spherocytes are more likely to lysis in hypotonic saline
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| What is the tx for congenital spherocytosis | Splenectomy, (make sure you give pneumovax vaccine to prevent pneumococcal diseas) this keeps spleen from removing abnormal spherocytes
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| You get this disease with a genetic mutation that causes membrane defect in stem cells and their progeny (WBC, RBC and platelets). You get sensitivity to C3 in plasma causing intravascular hemolysis and decreased production of WBC and platelets | Paroxysmal Nocturnal Hemoglobinuria
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| What are the s/sx of paroxysmal nocturnal hemoglobinuria | morining urine has Hgb, decreased respiratory rate while sleeping respiratory acidosis
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| What specialty labs will you order if you suspect pt has paroxysmal nocturnal Hemoglobinuria | sugar water test- RBC exposed to sucrose fix complement to their cell surface and will hemolyze; HAM- acidified serum test where cell lyse in acidified normal serum
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| What is the tx for paroxysmal nocturnal hemoglobinuria | Transfusion dependency
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| On peripheral blood smear you see Heinz Bodies(olive looking RBCs) and Bite cells what is pt likely suffering from | Glucose-6-Phosphate Dehydrogenase Deficiency
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| What group of people are most likely to suffer from Glucose-6-Phosphate Dehydrogenase Deficiency? | pt of Greek (they eat a lot of olives=heinz bodies) or African decent
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| What are the s/sx of Glucose-6-Phosphate Dehydrogenase Deficiency | Fatigue, lethargy- episode is started by certain medications (bactrim/septra, primaquine) or Fava Beans
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| If you hear Fava Beans in a question what should you think of first | Glucose-6-Phosphate Dehydrogenase Deficiency
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| What will you see on labs in Glucose-6-Phosphate Dehydrogenase Deficiency | Sudden drop in Hgb w/ mild increase in indirect bilirubin and LDH. Heinz bodies and bite cells seen on peripheral blood smear
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| What is the tx for Glucose-6-Phosphate Dehydrogenase Deficiency | stop medication or food that is precipitating the disease and give supportive care
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| Disease where you have a valine in place of glutamine on the beta strand in the sixth position of Hgb making Hgb S. Autosomal Recessive inheritance pattern | Sickle Cell Anemia
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| What is the trigger for RBCs to sickle in sickle cell anemia | oxygen removal from the cell so hypoxemic states, high altitude, exercise can cause sickling
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| What groups are most likely to present with sickle cell anemia | african, indian, arabian and eastern mediterranean decent individuals
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| what are the s/sx of sickle cell anemia | severe pain from local hypoxia and tissue infarction. Bone and abdominal pain. Splenic, Marrow, pulmonary, hepatic, Cerebral infarctions are common as is renal tubular dysfunction
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| What will be see in lab evaluation of sickle cell anemia | sickle shaped cells on peripheral smear, increased Hgb S and Hgb F vs Hgb A on Hgb electrophoresis.
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| What is the tx for sickle cell anemia | hyper transfusion, hydroxyurea increasing Hgb F inhibiting sickling. Narcotics for pain from sickle cell crisis
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| How are autoimmune hemolytic anemias divided | into Cold and Warm RBC cell agglutination
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| Pt has IgG antibody that is causing tissue damage and anemia what type of anemia are they likely suffering from | Warm Autoimmune Hemolytic Anemia
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| What type of autoimmune hemolytic anemai can the following diseases cause- Systemic Lupus, Chronic Lymphocytic anemia, and hodgkins lymphoma | Warm Autoimmune Hemolytic Anemia
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| What are the s/sx of warm autoimmune hemolytic anemia | drop in Hgb level, Splenomegally from extravascular lysis of RBC's by the spleen
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| What lab test will you order to test for warm autoimmine hemolytic anemia | Direct Coombs test- tests for IgG adhering to RBC's resulting in agglutination
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| What is the tx for Warm Autoimmune hemolytic anemia | stop offending meds and give high dose of corticosteroid, splenectomy if corticosteroid therapy isn't tolerated. Cyclophosphamide if none of the above works
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| What type of anemia is associated w/ mycoplasma pneumonia or infectious mononucleosis and IgM production | Cold Autoimmune Hemolytic Anemia
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| Where is the hemolysis of Cold autoimmune hemolytic anemia likely to take place | the reticuloendothelial system of liver and spleen.
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| What determines severity of cell lysis in Cold Autoimmune hemolytic anemia | Temperature- Ab causes hemolysis when exposed to cold hence the name
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| What is the tx for cold autoimmune hemolytic anemia | Alkylating agents (cyclophosphamides and chlorambucil) because disease doesn’t respond to steroid therapy
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| What type of anemia can be caused by prosthetic heart valves, vasculitis, DIC, consumptive coagulopathy. | Microangiopathic hemolytic anemia an extrinisic hemolytic anemia
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| What is the tx for microangiopathic hemolytic anemai | Treat underlying cause antibiotics for sepsis steroids for vasculitis
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| What will you see in lab results testing for microangiopathic hemolytic anemia | Low haptoglobin level, schistocytes on peripheral smear, hemoglobinuria, hemosiderinuria
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| What is erythroblostosis fetalis | hemolytic disease where incompatiblity between newborns blood group and mother's
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| What must be present for erythroblastosis fetalis to occur | mom must be Rh- dad Rh+ 1st pregnancy must be Rh+ mother gets exposed to babies blood and develops antibodeis to Rh factor. Future pregnancies mothers Ab will attack fetus resulting in hydrops fetalis
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| What are the s/sx of erythroblastosis fetalis | excess unconguated bilirubing, kernicterus, jaundice, hydrops fetalis
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| What is the tx for erythroblastosis fetalis | Rhogam shot for mother at 28 weeks of pregnancy; Infant needs to undergo exchange transfusion at birth or intrauterine transfusions can be given
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| How will patients RBCs appear in thallasemia, sideroblastic, iron deficiency anemias | microcytic hypochromic
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| how will patients RBCs appear in andemia of chronic disease | Microcytic normochromic
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| How will patients RBCs appear in infection caused anemia | normocytic hypochromatic
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| How will patients RBCs appear in acute blood loss, marrow failure and sickle cell | Normocytic Normochromatic
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| How will pts RBCs appear in folate or B12 deficiency and chronic alcoholism caused anemia | Macrocytic Normochromatic
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