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61 Cards in this Set
- Front
- Back
Which type of anemia?
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Iron deficiency; microcytic, hypochromic anemia
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Causes of iron deficiency anemia
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Chronic bleeds, malnutrition/absorption disorders, increased demand (eg. pregnant)
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Iron deficiency findings
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Low iron, low ferritin, high TIBC
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Iron deficiency anemia - which syndrome association?
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Plummer-Wilson (esophageal webs, atrophic glossitis)
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Alpha-thalassemia etiology
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Alpha-globin gene mutation --> decreased alpha-globin synthesis; 3 and 4 gene deletions are severe
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Alpha-thalassemia 3 gene deletion
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HbH disease (excess beta-globulin forms HbH)
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Alpha-thalassemia 4 gene deletion
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No alpha-globin; excess gamma-globin forms Hb Barts; hydrops fetalis, incompatible with life
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Alpha-thalassemia common in which populations?
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Cis deletion: Asians
Trans deletion: Africans |
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Beta-thalassemia etiology
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Splice site point mutations, promotor region mutations --> decreased beta-globins
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Beta-thalassemia common in which population?
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Mediterranean
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B-thalassemia minor features
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Heterozygote, B-globin deficient; asymptomatic; Increased HbA2 for diagnosis
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B-thalassemia major featurse
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Homozygote; B-globin absent; marrow expansion - "crew cut" on skull XR, chipmunk facies; increased HbF
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Lead poisoning pathophys
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1) Ferrochelatase, ALA dehydratase inhibition --> decrease heme synthesis
2) rRNA degradation inhibition --> basophilic stippling |
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Lead poisoning clinical features ("LEAD")
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Lead lines (Burton's lines) on gingivae
Encephalopathy & Erythrocyte stippling Abdo colic & sideroblastic Anemia Drops (wrist and foot); Dimercaprol treatment |
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Treatment for lead poisoning
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Dimercaprol and EDTA; succimer for kids
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Sideroblastic anemia etiology
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ALA synthase deficiency, X-linked; reversible - alcohol, lead, isoniazid
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Thalassemia - type of anemia
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Microcytic, hypochromic
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Sideroblastic anemia - type of anemia
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Microcytic, hypochromic with ringed sideroblasts
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Sideroblastic anemia lab findings
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Increased iron, normal TIBC, increased ferritin
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Sideroblastic anemia treatment
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Pyridoxine (cofactor for ALA synthase)
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Folate deficiency etiology
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Malnutrition (alcohol), malabsorption, antifolates (MTX, TMP, phenytoin), increased requirements (pregnancy)
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Folate deficiency clinical features
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Hypersegmented PMNs, glossitis, increased homocysteine, NORMAL methylmalonic acid
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B12 deficiency etiology
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Poor nutrition, malabsorption (Crohn's), pernicious anemia, Diphyllobothrium lactum (fish tapeworm), proton pump inhibitors
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B12 deficiency clinical findings
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Hypersegmented PMNs, glossitis, increased homocysteine, INCREASED methylmalonic acid, neuro findings
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B12 deficiency neurological signs
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Bilateral; peripheral neuropathy, posterior column degeneration, lateral corticospinal degeneration, dementia
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Orotic aciduria - associated anemia
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Megaloblastic, not responsive to folate or B12 in children (UMP synthase deficiency)
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Nonmegaloblastic macrocytic anemia - causes
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Liver disease, alcoholism, reticulocytosis (Increased MCV), drugs (5-FU, AZT, hydroxyurea)
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Intravascular hemolysis clinical findings
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(RBC breakdown in vessels) Decreased haptoglobin, increased LDH, hemoglobin in urine
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Extravascular hemolysis clinical findings
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(RBC breakdown by spleen) Increased LDH, increased UCB, normal haptoglobin
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Anemia of chronic disease
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Increased hepcidin (inflammation) --> decreased Fe release and transport
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Anemia of chronic disease lab findings
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Decreased Fe, increased ferritin, decreased TIBC
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Aplastic anemia
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Failure/destruction of myeloid stem cells; pancytopenia (hypocellular bone marrow with fatty infiltration)
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Aplastic anemia clinical symptoms
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Fatigue, malaise, pallor, purpura, mucosal bleeds, petechiae, infection
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Aplastic anemia causes
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Radiation, drugs, viruses (parvovirus B19, EBV, HIV, HCV), Fanconi's anemia (DNA repair defect), idiopathic
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Drugs causing aplastic anemia
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Benzene, chloramphenicol, alkylating agents, antimetabolites
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Aplastic anemia treatment
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Withdrawal of cause, immunosuppressants (cyclosporine, antithymocyte globulin), BM transplant, RBC/platelet transfusion, C-CSF, GM-CSF
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Conditions with non-hemolytic, normocytic anemia
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Anemia of chronic disease
Aplastic anemia Chronic kidney disease (decreased EPO) |
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Conditions with intrinsic hemolytic normocytic anemia
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Hereditary spherocytosis
G6PD deficiency (E/I) Pyruvate kinase deficiency HbC defect Paroxysmal nocturnal hemoglobinuria (I) Sickle cell anemia |
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Hereditary spherocytosis
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Defect in RBC membrane skeletal protein; small and round RBCs with no central pallor; premature RBC removal by spleen
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Hereditary spherocytosis clinical findings
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Labs: increased MCHC, RCDW, decreased MCV
Splenomegaly, aplastic crisis (parvovirus B19) |
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G6PD deficiency
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X-linked; decreased glutathione, RBC damage from oxidative stress; extravascular RBC destruction
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G6PD deficiency clinical findings
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Back pain, hemoglobinuria few days following stress (infection, fava beans, sulfa drugs)
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G6PD deficiency blood smear
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Heinz bodies and bite cells
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Heterozygous G6PD deficiency protective against
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P. falciparum
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Pyruvate kinase deficiency
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AR; decrease in ATP production --> rigid RBCs; hemolytic anemia in newborn
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HbC defect
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Beta-globin mutation: glutamic acid --> lysine mutation at residue 6; milder version of sickle cell
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Paroxysmal nocturnal hemoglobinuria
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Complement mediated RBC lysis; triad: hemolytic anemia, pancytopenia, venous thrombosis
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Paroxysmal nocturnal hemoglobinuria treatment
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Eculizumab (complement activation inhibitor)
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Sickle cell anemia
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Glutamic acid --> Valine at position 6, beta-globin; low O2, dehydration leads to cell sickling - anemia and vaso-occlusion
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"Crew cut" skull XR typical for
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Sickle cell anemia, thalassemias
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Sickle cell disease - complications in homozygotes
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Aplastic crisis (parvo B19)
Autosplenectomy (Howell-Jolly); risk of encapsulated bacterial infection Splenic sequestration crisis Salmonella osteomyelitis Painful vaso-occlusive crisis Renal papillary necrosis |
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Sickle cell treatment
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Hydroxyurea (increase fetal Hb), BM transplant
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Conditions with extrinsic hemolytic normocytic anemia
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Autoimmune
Microangiopathic Macroangiopathic Infections |
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Cold agglutinin disease
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Autoimmune hemolytic anemia, serum IgM antibodies bind to RBCs in cold weather, causing clumping
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Warm agglutinin disease
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Autoimmune hemolytic anemia, serum IgG bind to RBCs, then damaged by splenic macrophages (spherocytes), degraded in spleen
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Direct Coomb's test
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anti-Ig antibodies added to patient's serum; RBCs agglutinate if RBCs are coated with Ig
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Indirect Coomb's test
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normal RBCs added to patient's serum; serum agglutinates if serum has anti-RBC surface Ig
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Microangiopathic anemia
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RBCs damanged when passing through obstructed vessel lumen
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Micro/macro-angiopathic anemia - blood smear
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Schistocytes
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Conditions with microangiopathic anemia
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DIC, TTP, HUS, SLE, malignant hyperthermia
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Macroangiopathic anemia
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Hemolytic anemia caused by prosthetic heart valves, aortic stenosis --> mechanical destruction
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