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Anemia SMD
Micro/macro/normocytic anemias
| Question | Answer |
|---|---|
| 4 causes of microcytic anemia | 1. Fe deficiency 2. Thalassemia 3.Pb poisoning 4.Anemia of chronic disease (10%) |
| Causes of Fe deficiency | 1. dietary 2. malabsorptio n3. bleeding 4.increased need eg. pregnancy, rapid growth |
| Clinical features of Pb poisoning | -microcytic anemia-irritability-cognitive impairment-abdominal pain |
| Clinical presentation of Fe deficiency | -pallor- skin creases, conjunctiva-irritability, fatigue, tachycardia-angular stomatitis-blue sclera-pica |
| Lab results of Fe deficiency | -Gold standard: bone marrow biopsy (rarely used)-Ferritin LOW-Serum Fe LOW-TIBC HIGH-Transferrin saturation LOW-Soluble transferrin receptor HIGH |
| 7 Causes of macrocytic anemia | 1. B12 deficiency 2. Folate deficiency 3. Drugs 4. Myelodysplasia 5. Liver disease 6. Reticulocytosis 7. Hypothyroidism |
| 5 factors required for B12 absorption | 1. Diet 2. Acid and pepsin in stomach 3. Pancreatic proteases 4. IF5. Intact ileum with B12-IF receptors |
| Daily vitamin B12 requirements/ total body store | 2ug/day, 2-5mg body stores |
| Causes of B12 deficiency | Lack of IF-autoimmune-gastrectomy-disease of small bowel (Crohn's) |
| Clinical features of B12 deficiency | -macrocytic anemia -other cytopenias -mild jaundice -neurological changes -malabsorption -smooth red tongue |
| Lab tests for B12 deficiency | -Homocysteine HIGH -MMA HIGH -B12 LOW -anti-parietal or IF |
| Peripheral blood smear of B12 deficiency | -anemia -oval macrocytes -low WBC -low Plts -hypersegmented neutrophils |
| MCV predictive value for B12/folate deficiency | 80-100 <25%, 115-129 50%, >130 100% |
| Causes of false low B12 levels | -folate deficiency -pregnancy -oral contraceptives -AIDS |
| Clinical features of folate deficiency | -macrocytic anemia -otehr cytopenias |
| Diagnosis of folate deficiency | -low serum folate or RBC folate -high Hcy -normal MMA -hypersegmented neutrophils -megaloblastic marrow |
| Storage of Fe in: serum? tissue? muscles? storage? | hemoglobin/transferrin, cytochromes/enzymes, myoglobin, ferritin/hemosiderin |
| What is hemosiderin? | Form of Fe stored in macrophages visualized by Prussian Blue stain. |
| Daily Fe requirement? | 10-15mg/day |
| Normocytic anemias | 1. Acute blood loss 2. Anemia of Chronic Disease 3. Hemolysis 4. Renal failure 5. Myelodysplasia |
| Pathophysiology of ACD | Decreased RBC survival, decreased RBC production, EPO "inappropriately normal", altered Fe metabolism |
| What happens to the Fe in ACD? | sequestration of Fe into macrophages |
| What are cytokines role in ACD? | IL-1, TNF, IL-6 decrease EPO and increase hepcidin |
| how does hepcidin affect Fe? | Blocks ferroportin from releasing Fe so it is stored in bone marrow. Also decreases Fe absorption. Induced by IL-6 |
| Lab findings of ACD | Hb 85-115, normochromic, normocytic, serum Fe LOW, transferrin sat LOW, Ferritin NORMAL or ELEVATED, retic NORMAL, bone marrow NORMAL, CRP HIGH |
| Significance of reticulocyte count in ACD? | Inappropriately normal/LOWER than expected for anemia. |
| Special cell tyeps in ACD | Burr Cells, kidney disease - Target Cells, liver disease - |
| Thrombocytosis is indicative of what? | Increased platelets, usually reactive and increased with chronic inflammation, iron deficiency, or myeloproliferative disorders |
| How does reticulocyte count help DDX of normocytic anemia? | ACD LOW, bleeding HIGH, hemolysis HIGH, marrow failure LOW |
| What is HbH disease? | Only one functional alpha globulin gene, beta4 globulin |
| What is Hydrop fetalis? | no functional alpha globulin, Hb Barts gamma4 globulin |
| What are H bodies? | Aggregated beta globulin chains seen in alpha thalassemia |
| What is beta thalassemia minor? | heterozygous for beta globin, increase levels of HbA2 and HbF |
| Diagnosis of thalassemia? | hypochromic, microcytic,splenomegaly, increased HbA2 or HbF |
| Physical exam of hemolytic disease | jaundice, splenomegaly, expansion of bone marrow space |
| Lab tests for hemolytic disease | normocytic anemia- increased erythropoeisis, reticulocytes, polychromasia, bone marrow hyperplasia -increased unconjugated bilirubin, increased LDH |
| Lab tests for intravascular hemolysis | LOW serum haptoglobin, HIGH plasma Hb, HIGH urobilinogen, hemoglobinuria, hemosiderinuria, methemalbumin |
| Three main causes of intracorpuscular defects leading to hemolysis | hemoglobin, enzymes, membrane proteins |
| two RBC membrane conditions leading to hemolytic anemia | Spherocytosis, elliptocytosis |
| 2 RBC metabolism defects leading to hemolytic anemia | G6PD deficiency, pyruvate kinase deficiency |
| Special cell types seen in G6PD deficiency | heinz bodies, bite cells |
| Two main causes of extracorpuscular disorders of RBCs | Immune hemolytic and non-immune hemolytic anemia |
| Lab test for autoimmune hemolytic anemia? | direct antiglobulin test (Coomb's test) |
| Two types of autoimmune hemolytic anemias | cold-acting antibody and warm acting antibody |
| 4 cell lineages of hematopoietic stem cells | granulocyte, erythrocyte, monocyte, megakaryocyte |
| Why would the reticulocyte be low? | Decreased production or release of RBCs. ACD, Fe/folate/B12 deficiency, aplastic anemia |
| When would EPO and retic count be inappropriately normal? | anemia of inflammation |
| How are lab tests different for Fe vs ACD? | Fe: microcytic, LOW Fe, ferritin, bone marrow stores, transferrin sat, HIGH TIBCACD: normo/microcytic, LOW serum Fe, TIBC, transferrin sat, HIGH bone marrow stores, ferritin |
| What are two lab tests for anemia of inflammation? | ESR or CRP |
| Therapeutic option for ACD? | erthroid stimulating agents |
| What is HbH disease caused by genotypically? What will the distribution of Hb types be? | only one functional alpha globin allele. will get HbH (4 beta), low HbA. Get H bodies |
| What are H bodies? | Accumulation of beta globin chains due to excess. In HbH disease due to inability to produce much alpha globin. |
| What is Hb Barts? | 4 gamma chains in alpha thalassemia. |
| What lab results indicate beta thalassemia major? | absence of HbA, increase in HbF and HbA2. microcytic, hypochromic, mild anemia |
| When can thalassemia be diagnosed? | alpha: at birth. beta: 6 mo |
| How do you distinguish between Fe deficiency and thalassemia? | serum ferritin, TIBC |
| what is the phenotype of a HbE/B-thal compound heterozygote? | severe anemia like beta-thal major |
Created by:
baby_manatee
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