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88 Cards in this Set
- Front
- Back
Black tarry stools
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Anemia - GI Bleed
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No leafy greens
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Anemia - low folate levels
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Paresthesias in hands and feet
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Anemia - Low B12
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Anemia in African descent?
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Sickle Cell or G6PD
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Anemia in Asian descent?
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Thalassemia
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What are the four initial labs for an anemia work-up?
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Complete Blood Count
Reticulocyte Count Mean Cell Volume Peripheral Blood Smear |
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How is an uncorr reticulocyte count corrected?
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(uncorr retic %) x (pt HCT / nml HCT)
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Are there arbitrary good and bad values for reticulocyte counts?
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No - reticulocyte counts must be interpreted in light of a patient's condition.
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What are the causes of a high reticulocyte count?
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1) Acute blood loss (last 5-10 days)
2) Chronic blood loss 3) Hemolysis |
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What are the causes of a normal MCV anemia?
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1) Renal Failure (low EPO)
2) Aplastic Anemia 3) Endocrine/Thyroid Disorders 4) Pure RBC Aplasia 5) Myelophthisic Process (CA mets to the marrow) |
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In general, what causes, high MCVs?
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A high MCV indicates a problem with RBC maturation, such as DNA synthesis
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Myelodysplasia
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Myelodysplasia is a diverse collection of neoplastic disorders of hematopoietic stem cells. Formerly called "pre-leukemia"
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What are some specific causes of high MCV anemia?
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B12 & Folate Deficiency
Chemotherapy Myelodysplasia |
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In addition to the standard, what additional labs should be added for a high MCV?
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1) B12 & Folate
2) Marrow Aspirate |
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Describe the work-up for normocytic, macrocytic and microcytic anemia
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What are three ways to evaluate an Iron deficiency?
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1) Marrow macrophages won't stain with Prussian blue.
2) Serum ferritin levels fall 3) Increases in Total Iron Binding Capacity |
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What heme precursor level may be evaluated to determine if iron deficiency is affecting red cell maturation?
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Protoporphyrin
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What are sideroblasts?
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Iron granules in the cytoplasm of bone marrow erythroid precursors.
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What are the two major characteristics of iron deficient anemia?
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1) Low reticulocyte index
2) Low MCV |
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What work-up should all anemic adult males and anemic post-menopausal females receive?
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Fecal hemoccult to exclude a malignant GI bleed.
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What are some of the signs and symptoms of iron deficient anemia?
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* Smooth, sore tongue
* Spooning of fingernails * "web" high in esophagus c/dysphagia * Pica (ice eating) |
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What is the usual treatment for iron deficiency?
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Ferrous sulfate tablets PO tid. Empty stomach.
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How do you measure improvement of iron stores after therapy?
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Ferritin levels will return to normal. This may take months, depending on the deficit.
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What is the major problem in Anemia of Chronic Inflammation?
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Iron sequestration. There's enough iron, but the marrow can't use it.
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What's the molecule thought to be behind ACI?
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Hepcidin production is thought to increase with inflammation, leading to iron sequestration
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What's the major issue with anemia and renal failure?
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EPO production falls of with advanced/chronic kidney disease.
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Are all blood counts affected in chronic kidney disease?
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No, platelets and WBCs are usually unaffected.
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The FDA has posted two black-box warnings for EPO. What are they?
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1) Risk of cancer progression
2) Risk of stroke |
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What are the two major causes of iron overload?
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1) Hereditary hemochromatosis
2) Transfusional Iron Overload |
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What screening tests are used for hemochromatosis?
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Transferrin saturation tests
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What major complication can arise from hemochromatosis?
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Hepatocellular carcinoma, especially in patients who develop a secondary cirrhosis.
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What is the therapy for hemochromatosis?
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Phlebotomy
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What patient populations are at risk for transfusion iron overload?
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Anemias associated with an underproduction of RBCs:
* Aplastic anemia * Myelodysplasia * Severe thalassemias |
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What pharmacotherapy is available for iron overload?
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Deferoxamine
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Name exemplary cells of the Myeloid Lineage:
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Neutrophils, Eosinophils, Monocytes, Basophils
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Name exemplary cells of the Erythroid Lineage:
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Reticulocytes, Erythrocytes
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What bone marrow cells give rise to platelets?
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Megakaryocytes
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What's the lifespan of a mature RBC? What about a myeloid cell?
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RBCs = 120 days
Myeloids last less than 24 hours |
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What are the major growth factors of the bone marrow?
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1) Erythropoietin
2) Thrombopoietin 3) GM-CSF 4) Stem Cell Factor |
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Where is DPG made? What biochemical pathway?
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2,3-DPG is made in the RBC through the Luebering-Rapaport Pathway.
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What are some of the major causes of sideroblastic anemia?
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1) Myelodysplastic syndrome
2) Drugs (Isoniazid et al.) 3) Toxins (lead, EtOH) 4) Hereditary |
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What is the fundamental problem in sideroblastic anemia?
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Inhibition of heme synthesis.
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Name the globin constituents of the following:
1) Hg-A 2) Hg-A2 3) Hg-F 4) Hg-H |
1) Hg-A alpha2-beta2
2) Hg-A2 alpha2-delta2 3) Hg-F alpha2-gamma2 4) Hg-H beta4 (only in alpha thal syndrome) |
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What are some of the symptoms of sideroblastic anemia?
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Microcytic, anisocytic RBCs. Normal WBCs and PLTs.
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What's the function of transferrin?
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Binds extracellular iron. Transferrin/Iron complex is then endocytosed by cells with a Transferrin receptor
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Proteins modulating intestinal iron importation include:
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* Iron Regulatory Proteins - block translation of ferritin, ferroportin when iron is low
* Hepcidin - decreases intestinal absorption of iron. Produced by liver in response to inflammation |
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What is the hematocrit cutoff for anemia (female and male)?
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Female < 36%, Male < 38%
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What are the two major questions to ask about an anemia?
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1) Are red cells being produced appropriately?
2) Do the red cells mature normally? |
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What are the three major causes of low reticulocyte counts?
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1) Marrow is missing something it needs
2) Something's keeping the marrow from doing what it's supposed to do 3) Primary stem cell defect |
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What are the two big cases were we see anemias with high reticulocyte counts?
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1) Blood Loss
2) Hemolysis (intrinsic, extrinsic) |
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What are the three major types of intrinsic hemolytic anemias?
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1) Membrane abnormalities (hereditary spherocytosis)
2) Enzyme abnormalities (G6PD...) 3) Hb abnormalities (thal, HbS, HbC...) |
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What are the extrinsic causes of hemolytic anemia?
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1) Fragmentation hemolysis (DIC, TTP, HUS)
2) Mechanical heart valve 3) Toxins 4) Immunohemolytic anemia |
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What are the three RBC membrane disorders causing hemolytic anemia?
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1) Paroxysmal Nocturnal Hemoglobinuria
2) Hereditary Spherocytosis 3) Hereditary Elliptocytosis |
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PNH
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Paroxysmal Nocturnal Hemoglobinurea: membrane disorder with PI membrane anchor. Sensitive to complement and hemolysis. Dx by flow cytometry.
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Hereditary Spherocytosis
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Defect in membrane proteins akyrin, spectrin, et al. Cells "sphere up" to minimize surface area.
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How is Hereditary Spherocytosis diagnosed?
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Morphology and osmotic fragility test.
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What does the Direct Coombs Test measure?
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Complement or antibody bound to RBCs
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What does the Indirect Coombs Test measure?
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Circulating antibodies specific to RBCs.
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Warm reactive antibodies do what?
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Warm antibodies (IgG) cause extravascular hemolysis. Cells in the spleen or liver pick out the tagged RBCs and destroy them.
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Autoimmune Hemolytic Anemia (AIHA)
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Destruction of RBCs caused by antibodies binding to the RBC surface.
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What are the causes of Warm Reactive AIHA?
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1) Idiopathic
2) Drug-induced 3) Chronic Lymphocytic Leukemia or Lymphoma 4) SLE |
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What are the three mechanisms of Drug-Induced AIHA?
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1) Hapten formation (i.e., PCN binding to RBC)
2) Immune Complex Formation (Quinine + AB) 3) Apparent Cross-Specificity (Methyldopa) |
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How is Warm-Reactive AIHA treated?
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1) Steroid therapy
2) Splenectomy |
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What are the causes of Cold-Reactive AIHA?
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1) Idiopathic
2) Mycoplasma pneumonia (resolves with infection) 3) Infectious mononucleosis (resolves with infection) 4) Chronic Lymphocytic Leukemia/Lymphoma 5) Drug-induced |
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What are the symptoms of Cold-Reactive AIHA?
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Acrocyanosis - plugging of the vasculature of the extremities by agglutinated RBCs.
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What are the three microangiopathic hemolytic anemias?
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1) Disseminated Intravascular Coagulation
2) Thrombotic Thrombocytopenic Purpura 3) Hemolytic Uremic Syndrome |
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DIC
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Disseminated Intravascular Coagulopathy: loss of fibrinolysis and consequent hypercoagulation state consumes clotting factors and infarcts small vessels. Therapy is supportive (organ support, transfusions.)
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What are the general principles of managing hemolytic anemia?
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1) Folic acid supplements
2) Transfusion 3) Splenectomy = Infection, so ABs if febrile 4) Aplastic Crisis (e.g. parvo on SCA) will require short-term transfusion 5) Monitor reticulocyte counts for improvement |
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What are the compositions for the following hemoglobins: A, A2, F, H, Barts
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A SCA patient suddenly drops her hematocrit to 12% What's the differential dx?
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1) Sequestration Crisis - sudden, massive splenomegaly due to trapping of RBCs by splenic REs
2) Aplastic Crisis: temporary cessation of erythropoiesis (parvo or bacterial) |
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What neurotransmitter is depleted during a sickle cell crisis? How might this be treated?
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Nitric oxide is depleted from the vascular endothelium. Sildenafil is a possible treatment.
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What are the vaso-occlusive complications of SCA?
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1) Acute pain crisis
2) Dactylitis 3) Splenic sequestration 4) Osteonecrosis |
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What are the hemolytic complications of SCA?
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1) Pulmonary arterial hypertension
2) Priapism 3) Leg ulcers 4) Bile calculi |
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What are the systemic/long-term complications of SCA?
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1) Stroke
2) Acute Chest Syndrome 3) Renal Concentrating Problems 4) Retinopathy 5) Sickle Hepatopathy 6) Infection (asplenia, encapsulated organisms) 7) Iron Overload |
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What are the causes of RBC Macrocytosis?
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1) B-12 / Folate deficiency
2) Drugs (anti-metabolite) 3) Myelodysplasia 4) Liver disease 5) Brisk reticulcytosis |
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Hypersegmented Neutrophils
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Pathognomonic of megaloblastic anemia. Think B12/Folate, etc.
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What lab test helps differentiate Folate from B12 deficiency?
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Serum Methylmalonic Acid
* MMA is high in B12 deficiency * MMA is normal in Folate deficiency |
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Why would a Thalassemia Major (B0) patient show significant amounts of Hb-A on her Hb electrophoresis?
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The transfused blood contains a normal complement of Hb-A. Whole blood electrophoresis can't distinguish the two.
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Do Alpha Thalassemias come in major and minor?
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No, only Beta Thalassemias are rated major, intermedia, and minor. Alpha Thalassemia pts have either trait or disease.
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What is the differential diagnosis of pancytopenia?
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* Acute leukemia
* Aplastic anemia * marrow replacement with tumor, fibrosis, or granuloma * Drug side effect * Myelodysplasia * Occasionally deficiency of vitamin B12 or folate |
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How do you diagnose pancytopenia?
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Marrow aspirate and stain.
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What is the treatment of choice for aplastic anemia?
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Allogenic bone marrow transplant if a matched donor can be found.
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What is a shift cell?
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A young reticulocyte released early from the bone marrow which retains much of its RNA.
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What two disease classes would give a positive result to an osmotic fragility test?
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Hereditary Spherocytosis
Autoimmune Hemolytic Anemia |
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What is the cause of pernicious anemia?
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The majority of cases involve autoimmunity against gastric parietal cells, or antibodies to Intrinsic Factor iteself (or both.)
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How is Pernicious Anemia diagnosed?
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Shilling Test: Radio-labelled B-12 is given and absorption tested.
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What procedures/diseases are commonly associated with Pernicious Anemia?
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1) Auto-immune disorders (Graves, etc.)
2) Gastric bypass surgery 3) Crohn's |
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What are the causes of a warm autoimmune hemolytic anemia?
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* Idiopathic
* Drug-induced * CLL * Lymphoma * SLE |