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HEMATOLOGY for step 1

HEMATOLOGY for step 1. Lauren Meshkov, MS-3 lmeshkov@med.miami.edu. Tips. Memorize non-deducible info Predict board-style ?’s (2-3 step thinking) Make connections between subjects Who cares about other people!.

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HEMATOLOGY for step 1

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  1. HEMATOLOGY for step 1 Lauren Meshkov, MS-3 lmeshkov@med.miami.edu

  2. Tips Memorize non-deducible info Predict board-style ?’s (2-3 step thinking) Make connections between subjects Who cares about other people!

  3. Overview • Anemia • Platelets vs. Coagulation • Aspirin vs. Heparin vs. Warfarin vs…. • Lists to Memorize • Questions

  4. Anemia Mania! Alcoholism * Anemia of Chronic Dz * Autoimmune * AZT * B12 * DIC/TTP/HUS * Fe def * Folate def * G6PD * HbC * Lead * Liverdz * MAHA * Meds * Parvo B19 * PNH * Pyruvate kinase def * Reticulocytosis * Sickle Cell * Sideroblastic * Spherocytosis * Thalassemia *

  5. Anemia Break Down • Microcytic, Macrocytic, or Normocytic? • MCV is your best clue!!! Normal = 80-100 2. Intrinsic vs. Extrinsic • RBC naturally f’d up or being attacked (hemolysis) 3. Intravascular vs. Extravascular • Is RBC getting whacked in the blood or the spleen? 4. How does the retic count look? • Bone marrow problems  leukemia?

  6. Microcytic: Heme Synth (MCV < 80) Fe deficiency vs. Thalassemia Sxs – fatigue, pallor, pica Labs – everything normal but H&H Fe studies – low Fe, high TIBC, low ferritin, very low %sat Differential based on pt ID: Old folks – chronic GI bleed! Young person – malabsorb, IBD The Ladies – pregnancy, menses Plummer Vinson – esophageal web and glossitis Treat? Ferrous Sulfate (also for dx) Sxs – depends on type a: African/Asian missing 4 genes? Hb Barts (g4)  death (hydrops fetalis) missing 3 genes? HbH dz (b4)  symptoms missing 1-2 genes? Carrier  asymptomatic b: Mediterranean w/ pt mutations Labs – everything normal but H&H Fe studies – NORMAL Electrophoresis – btw a/b Major homozygote (HbF) Marrow expansion, skull crew cut xray, chipmunk Minor heterozygote Asymptomatic

  7. Macrocytic: DNA Synth (MCV > 100) Folate deficiency vs. B12 deficiency Sxs – Glossitis sxs from underlying condition Labs – folate low, high homocysteine Normal methylmalonic acid Differential based on pt ID: *Alcoholics – Malnutrition IBD pts - Malabsorption On bactrim or MTX The Ladies – pregnancy Hemolytic Anemias (demand) Smear – hypersegmented polys Sxs – Glossitis Neurologic, posterior columns (periph neuropathy, dementia) Labs – B12 low, high homocysteine high methylmalonic acid! Smear – hypersegmented polys *Years to lose B12 stores (vegans? Grandma “tea and toast”) (B12) DNA: Homocysteine  Methionine + THF (B12) FA’s: Methylmalonic acid  Succinyl CoA  TCA for energy

  8. Identifying other Anemias • G6PD – recent meds (sulfa, ASA, bactrim), x-linked! • Spherocytosis – smear, excess membrane • Sickle cell – osteomyelitis, pain crises (low O2 or dehydration), SHiNS, aplastic crisis. *Biochem: Valine put in place of glutamic acid • PNH – dark a.m. urine that fades through day, decay accelerating factor, complement mediated • MAHA – valve dz, DIC (all coags high), TTP, HUS • Autoimmune – other autoimmune dz • Parvo B19 – sickle cell aplastic anemia • Anemia of chronic disease – other dz! (CA), high ferritin • Lead poisoning – new home, colic, neuro, lead lines, stippling, *Biochem: blocks ferrochetalase and ALA dehydratase

  9. Platelets vs. Coagulation Factor II, V, X, VII, IX, XI, XII Factor VII • PT (9-13 sec) and PT (29-34 sec)? Normal! • Petechiae, Purpura, Epistaxis, Bleeding gums

  10. Plateletsvs. Coagulation Factor VII Factor II, V, X, VII, IX, XI, XII • PT (9-13 sec) and PT (29-34 sec)? Prolonged! • Easy bruising • D-dimer Fibrin being broken down in PE? • Not necessarily. Better negative predictive value. Vit K (epoxidereductase)  II, VII, IX, XX Antithrombin III def Protein C or S def Factor V Leiden Plasminogen--plasmin Hemophilia A or B Heparin (ATIII) Warfarin (epoxidereductase) tPA, streptokinase thrombolytics COAGULATION

  11. Platelets vs. Coagulation(what’s messed up?) • Bernard Soulier: Gp1b • Glanzmann’s: GpIIb/IIIa • Abciximab: GpIIb/IIIa • von Willebrand:vWF • Clopidogrel: ADP rec • Ticlopidine: ADP rec • Aspirin: COX  TXA2 • ITP: autoimmune • TTP: • Hemophilia A: VIII • Hemophilia B: IX • Vitamin K def: epoxidereductase  II, VII, IX, X • Antithrombin III def:  can’t break down • Factor V Leiden:  mutated V can’t be broken down

  12. ASA vs. Heparin vs. Warfarin ASA MOA: Irreversibly inhibits COX-1 and COX-2. (Arachidonic acid  TXA2) Why take it? Fever, analgesic, anti-inflammatory, antiplatelet. *Kawasaki’s AEs: Gastric ulcer, bleeding, hyperventilation (acid-base), Reye’s, tinnitus Antidotes: NaHCO3, alkalinze the urine Heparin MOA: Activates Antithrombin, which breaks down thrombin and Xa. Why takes it? PE, DVT, stroke, ACS, MI. Bridge to Warfarin *Ok in pregnancy AEs: Bleeding (PTT), thrombocytopenia (*HIT), osteoporosis, drug-interactions Antidotes: 1. Stop the heparin 2. Protamine sulfate Warfarin MOA: Stopsepoxidereductase and d-carboxylation of Vit K (II, VII, IX, X) Why take it?Chronic anti-coagulation (heparin bridge), no good in preggo AEs: Bleeding (PT), teratogenic, *skin necrosis, *P-450 drug interactions (INR) Antidotes: Vit K, FFP

  13. Things to Memorize • Blood cell smears • Heme synthesis and the porphyrias

  14. Question 1 A 33 yo F comes to the office with fatigue and heavy menstrual cycle. Her H&H is 10.2/76.2 and RDW 18.5. A pregnancy test is negative and she is otherwise healthy. What do you recommend? • Colonoscopy • Blood Transfusion • Trial of ferrous sulfate • Schilling Test • Bone Marrow Biopsy

  15. Question 2 A Greek woman brings her 10 yo son to your office complaining that he tires too easily on the soccer field and the iron pills from a different doctor aren’t helping. On PE you note he has puffy cheeks. You do an electrophoresis of his hemoglobin and are most likely to see which of the following? • HbA2 • HbF • HbS • HbC • HbM • HbH

  16. Question 3 A 32 yo F has heavy menstrual bleeding, easy bruising, gingival bleeding when she brushes her teeth, and nose bleeds. She required a blood transfusion when she gave birth. Labs: Hb 10.8, MCV 78.1, WBC 4.3, Platelet 275 BT 14 (high), PT 11 (normal), PTT 40 (high) What is the most likely diagnosis? a) Hemophilia A b) Hemophilia B c) vonWillebrand’s disease d) Disseminated Intravascular Coagulation e) Hemolytic Uremic Syndrome

  17. Question 4 A 72 yo F comes has increasing memory problems and weakness over the last 7 months but can take care of her ADLs. She has a PMH of HTN. Labs: Hb 8.2 LDH 450 B12 324 (normal) Hct 22 Tbili 4.8 Folate 12 (normal) WBC 3.9 Dbili 0.3 Homocysteine 20 (high) MCV 110 Platelet 234 Methylmalo 400 (high) What is the most likely diagnosis? a) Autoimmune hemolytic anemia • Folate deficiency • Myelodysplasia • Vitamin B12 deficiency • Gilbert Syndrome

  18. Question 5 A family new to the area reports the strange behavior of their 2 yo girl and her constant belly aches. Her blood smear is shown. What is the best treatment? • Succimer and EDTA • N-acetylcysteine • Naloxone • Glucagon • Protamine sulfate • Atropine • Thiosulfate

  19. Question 6 A 59-yo who takes warfarin for a mechanical heart valve is brought to the hospital after a car accident. What would you give to help stop his bleeding? • Protamine sulfate • 2 units of packed RBCs • Fresh Frozen Plasma • Multiple doses of Vit K • Isolated factor VIII • DDVAP

  20. Question 7 A 31yo F comes in with blistering lesions all over the skin after a weekend escape to the beach. She also reports tea colored urine. What enzyme is likely defective? a) d-aminolevulinic acid synthase • d-aminolevulinic acid dehydratase c) Porphobilinogendeaminase • Uroporphyrinogendecarboxylase • Ferrochetalase

  21. Question 8 A previously healthy 9 yo M is currently being treated for a UTI that began 5 days ago. He returns with his Mom, who is scared that her son’s urine is now dark in color and he has excessive fatigue. What are you likely to see on his blood smear? • Bite cells • Schistocytes • Spur cells • Elliptocytes • Howell Jolly bodies • Target cells

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