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132 Cards in this Set
- Front
- Back
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What are the different classifications of anemias?
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Microcytic
Normocytic Macrocytic |
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What is the definition of a microcytic anemia?
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MCV < 80 fL
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What is the definition of a normocytic anemia?
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MCV<80-100 fL
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What is the definition of a macrocytic anemia?
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MCV>100
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What are the different microcytic anemias?
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Iron deficiency
ACD Thalassemias Lead poisoning Sideroblastic anemia |
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What are the different classes of normocytic anemias?
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Hemolytic
Nonhemolytic |
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What are the different classes of hemolytic anemias?
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Intrinsic
Extrinsic |
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What are the different nonhemolytic normocytic anemias?
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ACD
Aplastic anemia Chronic kidney disease |
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What are the intrinsic hemolytic anemias?
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RBC membrane defects: hereditary spherocytosis
RBC enzyme deficiency HbC Sickle cell anemia Paroxysmal nocturnal hemoglobinuria |
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What are the extrinsic hemolytic anemias?
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Autoimmune
Microangiopathic Macroangiopathic Infections |
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What are the different calsses of macrocytic anemia?
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Megaloblastic
Non-megaloblastic |
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What are the megaloblastic, macrocytic anemias?
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Folate deficiency
B12 deficiency Orotic aciduria |
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What are the non-megaloblastic, macrocytic anemias?
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Liver disease
Alcoholism Reticulocytosis |
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What's the cause of iron deficiency anemia?
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Decreased iron due to chronic bleeding: (GI loss; menorrhagia)
Malnutrition/absorption disorders Increased demands |
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What is the mechanism of iron deficiency anemia?
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No reagents for the final step of heme synthesis.
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What are the findings in iron deficiency anemia?
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Decreased iron
increase in TIBC Decreased ferritin |
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What are the histologic findings in iron deficiency anemia?
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Microcytosis
Hypochromia |
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What are the symptoms of Plummer Vinson syndrome?
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Anemia
Esophageal webs Atrophic glossitis |
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What is defective in alpha-thalassemia?
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Defect in the alpha globin gene: lack of synthesis
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What kind of deletion is present in the Asian alpha thallasemias?
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Cis deletion
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What kind of deletion is present in the African alpha thallasemias?
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Trans deletion
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What are the different kinds of the alpha-thallasemias?
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4 gene deletion: hydrops fetalis
3 gene deletion: HbH disease 1-2 genes: no anemia |
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What kinds of hemoglobin form if you have a 4 gene deletion of the alpha genes?
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Gamma4 (Hb Barts)
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What problem underlies the beta thallasemias?
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Point mutations in the splice sits and promoter sequences of the beta-globin gene, leading to decreased synthesis.
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What populations have a high incidence of thalassemias?
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Mediterranean population
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What is the genotype in beta-thallasemia minor?
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Heterozygote
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What are the symptoms of beta-thalassemia minor?
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Asymptomatic
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How do you diagnose the thalassemias?
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Hb electrophoresis
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What indicates a diagnosis of beta-thalassemia minor on Hb electrophoresis?
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Increased HbA2 (>3.5%)
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What is the phenotype of beta thalassemia major?
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Homozygote
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What is the therapy for beta-thalassemia major?
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Chronic blood transfusion
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What is the appearance of beta-thalassemia major on an xray?
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Marrow expation-->deformities
"Chipmunk facies" |
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What types of hemoglobin are found in beta-thalassemia major?
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Increased HbF (alpha2, gamma2)
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What type of beta-thalassemia is a hybrid disease?
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HbS/Beta-thalassemia heterozygote
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What is the cause of toxicity with lead poisoning?
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Lead inhibits the ferrochelatase and ALA dehydratase-->decreased heme synthesis
Inhibits rRNA degradation |
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What is the impact on lead inhibiting rRNA breakdown in blood cells?
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Basophilic strippling
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What are the findings of lead poisoning?
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Lead Lines on gingivae (and metaphyses of long bones on xray)
Encephalopathy Abdominal colic Drops (wrist and foot) |
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What are the histologic findings of lead poisoning?
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Erythrocyte basophilic strippling
Sideroblastic anemia |
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What's the treatment for lead poisoning?
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Dimercaprol
EDTA Succimer for chelation |
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What is the purpose of succimer?
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Chelate lead that a person has ingested.
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Generally, what is the cause of sideroblastic anemia?
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Defects in heme synthesis
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What's a hereditary cause of sideroblastic anemia?
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X-linked defect in the delta-ALA synthase gene
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What are some reversible causes of sideroblastic anemia?
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Alcohol
Lead Isoniazid |
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What are histologic findings in sideroblastic anemia?
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Ringed sideroblasts (with iron laden macrophages)
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What are the lab findings in sideroblastic anemia?
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Increased iron
Normal TIBC Increased ferritin |
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What's the treatment for sideroblastic anemia?
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Pyridoxine (B6; a cofactor for ALA synthetase)
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Generally, what is the cause of megaloblastic anemia?
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Impaired DNA synthesis leads to a delay in the maturation of the nucleus relative to the maturation of the cytoplasm
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What are causes of folate deficiency?
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Malnutrition
Malabsorption Antifolates Increased requirements |
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What are some causes that place an increased folated burden on the body?
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Pregnancy
Hemolytic anemia |
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What are the findigns in folate deficiency?
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Hypersegmented neutrophils
Glossitis Decreased folate Increased homocystine Normal methylmalonic acid |
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What are the causes of B12 deficiency?
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Insufficient intake (vegans!)
Malabsorption Pernicious anemia Diphyllobohrium latum PPIs |
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What are the findings in B12 deficiency anemia?
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Hypersegmented neutrophils
Glossitis Decreased B12 levels Increased homocystine Increased methylmalonic acid NEUROLOGIC SYMPTOMS |
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What are the neurologic symptoms in B12 deficiency?
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Peripheral neuropathy with sensorimotor dysfunction
Posterior column problems (vibration/proprioception) Lateral corticalspinal problems (spasticity) Dementia |
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If there are problems with the posterior columns, what senses will be impaired?
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Vibration
Proprioception |
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What is the cause of ortic aciduria?
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Genetic mutation in the enzyme that synthesizes uridine from orotic acid
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What's the presentation of orotic aciduria?
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Megaloblastic anemia in kids that can't be cured with folate or B12
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What are the findings in orotic aciduria?
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Hypersegmented neutrophils
Glossitis Orotic acid in the urine |
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What's the treatment for orotic aciduria?
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Uridine monophasphate to bypass the mutated enzyme
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What is the pathophysiology of the nonmegaloblastic macrocytic anemias?
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Macrocytic anemia in which DNA synthesis is unimpaired
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What are the causes of nonmegaloblastic macrocytic anemias?
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Liver disease
Alcoholism Regiculocytosis Drugs |
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What are some drugs that can cause nonmegaloblastic macrocytic anemia?
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5-FU
AZT Hydroxyurea |
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What is the classification of the normocytic, normochromic anemias?
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Hemolytic vs. nonhemolytic
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How can you divide the hemolytic anemia/
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Cause of the hemolysis: intrinsic vs. extrisic
Location of the hemolysis: intravascular vs. extravascular |
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What are some findings in intravascular hemolysis?
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Decreased haptoglobin
Increased LDH Hb in the urine |
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What are some findings in extravascular hemolysis?
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Increased LDH
Increased UCB Jaundice |
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What are the causes of nonhemolytic, normocytic anemia?
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Anemia of chronic disease
Aplastic anemia Chronic kidney disease |
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What is the mechanism of anemia development secondary to chronic disease?
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1. Inflammation
2. Increased hepcidin release 3. Decreased release of iron from the macrophages 4. Anemia. |
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What is the action of hepcidin in the body?
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Binds ferroportin on the intestinal mucosa-->decreased uptake
Binds ferroportin on macrophages --> decreased release Overall, decreases body iron supply |
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Where is hepcidin released from?
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Liver
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What are the findings in anemia of chronic disease?
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Deceased iron
Decreed TIBC Increased ferritin |
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What are causes of aplastic anemia?
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Radiation and drugs
Viruses Fanconi's anemia Idiopathic |
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What are some drugs that can cause aplastic anemia?
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Benzen
Chloramphenicol Alkylating agents Antimetabolites |
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What are some viral agents that can cause aplastic anemia?
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Parvovirus B19
EBV HIV HCV |
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What is defective in Fanconi's anemia?
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DNA repair
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What type of cell is destroyed during aplastic anemia?
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Stem cells
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What are the findings in aplastic anemia?
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Pancytopenia
Hypocellular bone marrow wiith fatty infiltration |
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What are the symptoms of aplastic anemia?
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Fatigue
Malaise Pallor Purpura Mucosal bleeding Petechiae Infection |
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What's the treatment of aplastic anemia?
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Withdraw the offending agent
Immunosuppression Allogenic bone marrow transplation RBC/platelet transfucsion G-CSF GM-CSF |
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What is the cause of nonmegaloblastic macrocytic anemia secondary to chronic kidney disease?
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Decreased erythropoietin --> decreased hematopoiesis
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What are the intrinsic hemolytic normocytic anemias?
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Hereditary spherocytosis
G6PD deficiency Pyruvate kinase deficiency HbC sdefect Paroxysmal nocturnal hemoglobinuria Sickle cell anemia |
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Which of the intrinsic hemolytic normocytic anemias are intravascular?
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G6PD deficiency
Paroxysmal nocturnal hemoglobinuria |
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Which of the intrinsic hemolytic normocytic anemias are extravascular?
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Hereditary spherocytosis
G6PD deficiency Pyruvate kinase deficiency HbC defect Sickle cell anemia |
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What proteins are defective in hereditary spherocytosis?
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Defect in proteins interacting with the RBC membrane skeleton and the plasma membrane:
-Ankyrin -Band 3 -Protein 4.2 -Spectrin |
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What is the appearance of RBCs in hereditary spherocytosis?
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Small, round RBCs with no central pallor
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What occurs to spherocytes in the body?
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Premature removal by the spleen
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What are findings in hereditary spherocytosis?
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Splenomegaly
Aplastic criss |
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What are the lab findings in hereditary spherocytosis?
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Positive osmotic fragility tests
Normal to decreased MCV Increased MCHC |
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What's the treatment for hereditary spherocytosis?
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Splenectomy
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What's the inheritance of G6PD deficiency?
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X-linked
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How does RBC damage occur in G6PD deficiency?
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1. Defect in G6PD
2. Decreased glutathione 3. Increased RBC susceptibility to oxidant stress |
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What are some oxidant stresses that can exacerbate problems in G6PD deficiency?
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Sulfa drugs
Infections Fava beans |
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What are the labs in G6PD deficiency?
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Smear:
Heinz bodies Bite cells |
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What's the inhteritance of pyruvate kinase deficinecy
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Autosomal recessive
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What is the effect of a lack of pyruvate kinase on RBCs?
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Decreased ATP-->rigid RBCs
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What is the presentation of pyruvate kinase deficiency?
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Hemolytic anemia in a newborn
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What is the cause of HbC defect?
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Glutamic acid-->lysine mutation at position 6 in beta globin
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What's the cause of paroxysmal nocturnal hemoglobinuria?
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incrased complement mediated RBC lysis due to impaired synthesis of a GPI anchor that protects the RBC membrane from complement
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What is the triad in PNH?
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Hemolytic anemia
Pancytopenia Venous thrombosis |
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What are the labs in paroxysmal nocturnal hemoglobinuria?
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CD55/59 negative labs on flow cytometry
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What is the treatment for paroxysmal nocturnal hemoglobinuria?
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Eculizumab
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What hemoglobin is mutated in sickle cell anemia?
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Hemoglobin beta
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What is the mutation that takes place in sickle cell anemia?
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Substitution of a glutamic acid with valine at position 6 in the beta globin gene
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How does pathology result from sickle cell disease?
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Low O2 or dehydration causes sickling of the hemoglobin within the cells, leading to anemia and vaso-occlusive disease
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What can cause HbS to sickle?
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Deoxygenation
Dehydration |
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When does sickle cell disease present?
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A couple of months after birth; due to the fact that there's still HbF around
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Why does HbS stick around in the population?
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Heterozygotes are resistant to malaria
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What ethnicity has the Hbs trait?
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African Americans: 8% carry the disease
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What is the appearance of sickle cells?
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Crescent shaped RBCs
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What is a cause of an Xray like this? Why?
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Beta-thalassemia major
Sickle cell disease Due to increased erythropoiesis |
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What are complication in sickle cell homozygotes?
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Aplastic crisis
Autosplenectomy Splenic sequestration crisis Salmonella osteomyelitis Pinful crisis! Renal papillary necrosis |
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What kinds of infections are people with sickle cell more vulnerable to?
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Encapsulated organisms:
S. pneumoniae H. influenzae type B N. meningitidis |
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What's the treatment for sickle-cell anemia?
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hydroxyurea
Bone marrow transplantation |
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What are the extrinsic hemolytic normocytic anemias?
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Autoimmune hemolytic anemia
Microangiopathic anemia Macroangiopathic anemia Infections |
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What are the different types of autoimmune hemolytic anemias?
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Warm agglutinin
Cold agglutinin |
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What type of immune globulin is implicated in warm agglutinin hemolytic anemia?
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IgG
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What type of immune globulin is implicated in cold agglutinin hemolytic anemia?
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igM
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What diseases are associated with warm agglutinin anemia?
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SLE
CLL Drugs |
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What are conditions associated with cold agglutinin anemia?
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CLL
Mycoplasma pneumonia Infectious mononucleosis |
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What are the different tests that you can run in autoimmune hemolytic anemias?
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Direct coombs test
Indirect Coombs test |
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What do you do in a direct coombs test?
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Anti-Ig antibody is added to the patient's serum
RBCs agglutinate if RBCs are coated with Ig |
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What is performed in an indirect Coombs test?
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Normal RBCs added to patient's serum agglutinate if serum has anti-RBC surface Ig
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What is the pathogenesis of microangiopathic anemia?
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RBCs are damaged when passing through obstructed or narrowed vessel lumina
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What conditions cause a microangiopathic anemia?
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DIC
TTP-HUS SLE Malignant hypertension |
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What are the findings in a microangiopathic anemia?
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Schistocytes (helmet cells)
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What are causes of macroangiopathic anemia?
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Prosthetic heart valves
Aortic stenosis |
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What are findings in macroangiopathic anemia?
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Schistocytes on a peripheral blood smear
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What types of infections can cause anemia?
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Malaria
Babesia |
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What are the the following lab findings in iron deficiency:
Serum iron? Transferrin? Ferritin? Transferrin saturation? |
Serum iron: decreased
Transferrin: increased Ferritin: decreased Transferrin saturation: markedly decreased |
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What are the the following lab findings in chronic disease:
Serum iron? Transferrin? Ferritin? Transferrin saturation? |
Serum iron: decreased
Transferrin: decreased Ferritin: increased Transferrin saturation: unchanged |
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What are the the following lab findings in hemochromatosis:
Serum iron? Transferrin? Ferritin? Transferrin saturation? |
Serum iron: increased
Transferrin: decreased Ferritin: increased Transferrin saturation: markedly increased |
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What are the the following lab findings in pregnancy/OCP use:
Serum iron? Transferrin? Ferritin? Transferrin saturation? |
Serum iron: no change
Transferrin: increased Ferritin: Transferrin saturation: decrased |
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What's the effect of pregnancy on transferrin production?
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Increased
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