Small Group 2: Study Guide Kaplan videos: ANEMIAS Microcytic Anemia (MCV < 80) 1.

Iron Deficiency: microcytic, hypochromic RBCs in blood smear 2. -thalassemia: gene deletions in alpha-globin chains (prev in Asian/African pop) 1-2 del = asymptomatic/mild; 3 del = HbH disease 4 del = Hb Barts Hydrops Fetalis 3. -thalassemia: point mutations in splice sites/promoter seq (prev in Mediterranean pop) -thal minor: heterozyg; -chain production = usually asympt. Dx: increase HbA2 ( (22) -thal Major: homozyg; ABSENT -chain = severe anemia; req chronic blood transfusion. Dx: marrow expansion crew cut skull XR 4. Sideroblastic Anemia: Genetic = X-linked ALA synthase defect. Rx: B6 Reversible = alcohol or lead poisoning Lab Dx: Iron, Ferritin, normal TIBC; Ringed sideroblasts in blood smear 5. Lead Poisoning: inhibits heme synth enzymes Ferrochetolase & ALA dehydratase. Clinical Pres: Lead Lines gingivae & long bone epiphyses; Encephalopathy; Abd pain, periph neurop, wrist/foot drop Diagnosis: Basophilic stippling in RBCs blood smear Rx: EDTA, Succimer (children), Dimercaprol Normocytic Anemia (MCV is between 80 and 100) the following are Non-hemolytic: 1. Anemia of chronic disease Path: inflammation Hepcidin iron-release from M and Transferrin. Lab Dx: iron, TIBC, Ferritin 2. Aplastic Anemia Path: Pancytopenia Petachiae, bleeding, infection, neutropenia Causes: Radiation/chemo, viruses, Fanconi's anemia, idiopathic ( after acute hepatitis) Tx: eliminate causative agent, BMT, GM-CSF 3. Anemia of Chronic Kidney Disease Path: erythropoeitin production hematopoeisis Tx: EPO injections Macrocytic Anemias (MCV > 100): 1. Megaloblastic anemia Path: impaired DNA synthesis (2 causes) hypersegmented Neutrophils w cytoplasm Due to Folate deficiency: Folate, Homocysteine, OK methylmalonic acid Causes: alcoholism, malnutrition, etc. Due to B12 deficiency: B12, Homocysteine, methylmalonic acid Neuro Sx: subacute combined degeneration/dementia (reversible if caught) Causes: Pernicious anemia (AutoAb vs parietal cells) D. latum (fish tapeworm)

Crohn's disease Ileal resection 2. Nonmegaloblastic anemia Causes: Liver disease, alcoholism, reticulocytosis

Kaplan Vids: Hemolytic Anemia & Pathologic RBC Forms Hemolytic Anemia Characteristics: Instravascular hemolysis (inside vessels): haptoglobin, LDH, Hemoglobinuria Extravascular hemolysis (outside vessels): LDH, unconjugated bilirubin (blood/urine) Intrinsic hemolysis (inside RBC) Extrinsic hemolysis (outside RBC) Sickle Cell Anemia (HbS) Defect: Valine to Glutamic Acid substitution in chain HbS (2 normal , 2 abnormal ) Path: HbS precipitates during hypoxia RBC sickling vaso-occlusive crises infarction, pain Complications: Aplastic crises (Parvovirus B19), autosplenectomy, Salmonella, Osteomyelitis, painful crisis, renal papillary necrosis Tx: Hydroxyurea (to HbF) HbC Defect Defect: Lysine to Glutamic acid at position 6 of hemoglobin chain Path: less severe than HbS; Pt's can have both HbS/HbC Autoimmune Hemolytic Anemia 1. Complement Deficiencies: C3b deficiency: prone to encapsulated bacterial infection C3a & C5a deficiency: assoc w/ anaphylactic shock; C5a = neutrophil chemotaxis Hereditary angioedema: C1 esterase inhibitor deficiency; uncontrolled C' activation 2. Autoimmune Hemolytic Anemia: Warm agglutinin: IgG Abs; SLE, CLL, drugs (alpha methyldopa) Cold agglutinin: IgM Abs; CLL, M. pneumonia, infectious mono Erythroblastosis fetalis: Rh- mother w/ Abs vs Rh+ fetus hemolytic anemia & kernicterus in newborn Autoimmune Hemolytic Anemias are Coombs' test POSITIVE: DIRECT: anti-IgG Abs added to Pt's blood agglut if RBCs have IgG coating [test for hemolytic disease risk in newborns] INDIRECT: normal RBCs added to Pt's SERUM agglut if SERUM has IgG that binds RBCs [test for anti-Rh Abs in mothers] RBC Morphology: Acanthocytes (aka spur cell): assoc with LIVER disease Basophilic stippling: in Thalassemias, Anemia of chronic disease, iron deficiency, lead poisoning (T.A.I.L.) Bite Cells: in G6PD deficiency ( oxid stress of RBCs = HEINZ BODIES)

Elliptocyte: Ringed sideroblasts: (DIC, TTP, HUS) Schistocytes: (helmet) Sickle Cells: Spherocytes: Teardrop cells: bone marrow infiltration Target Cells: HbC, Asplenia, Liver disease, Thalassemia ("HALT said the hunter to his Target") Lab Values in anemia: Anemia Iron deficiency Anemia of chronic disease Hemochromatosis Serum Iron Can be Transferrin (TIBC) Ferritin (storage)