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23 Cards in this Set
- Front
- Back
DDx for microcytic anemia.
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Mneumonic: TAILS
T - thalessemia A - anemia of chronic diseases I - iron deficiency L - lead poisoning S - sideroblastic anemia |
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What is the definition of thalessemia? Distinguish between ALPHA-thal and BETA-thal.
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= decrease or absence of normal GLOBIN chains of hemoglobin
ALPHA-thal = mut'n in ≥1 of the 4 genes coding for alpha-hemoglobin BETA-thal = mut'n in ≥1 of the 2 genes coding for beta-hemoglobin |
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Among which ethic groups is thalessemia more common?
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- African
- Middle Eastern - Asian |
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How is thalessemia diagnosed?
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- hemoglobin electrophoresis + DNA studies
Note - electrophoresis will be normal in alpha-thal |
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What are 3 root causes of iron deficiency anemia?
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1. Increased iron demand - e.g. pregnancy
2. Decreased supply - cow's milk diet among infants - tea 'n' toasters among elderly - malabsorption among IBD, celiacs - post-gastrectomy 3. Increased loss - hemorrhage - hemolysis |
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Symptoms and signs of iron-defiency anemia.
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Symptoms: fatigue, weakness, pica, h/a, irritable, exercise intolerant, syncope, dyspnea, cold, postural dizzy
Signs: brittle nails/hair, koilonychia (spoon-shaped nails), pallor, glossitis *can be asx is anemia develops slowly |
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What is Plummer-Vinson Syndrome?
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Triad of:
1. DYSPHAGIA (secondary to esophageal webs) 2. GLOSSITIS 3. IRON-DEF'Y ANEMIA *may see angular stomatitis (inflammation/fissures at corners of mouth) |
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What investigations should be done if one suspects a pt has iron-defiency anemia?
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1. Fe indices - ferritin <45 ug/L = Fe-def anemia*
2. Peripheral smear - hypochromic microcytosis, target cells 3. BM - gold standard (rarely done) would find dec'd iron in macrophages and erythroid precursors (Prussian blue stain for iron) *ferritin is an acute phase reactant, :. elevated in inflammatory conditions, liver disease |
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Treatment of Fe-def' anemia?
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1. Treat underlying cause.
2. Supplement. - PO: ferrous sulphate 325 mg TID ... - PO: ferrous gluconate 300 mg TID ... - PO: ferrous fumerate 300 mg TID ... until N ferritin - IV: iron sucrose or iron dextran ... anaphylaxis risk 3. Monitor response to Fe: - retic count should inc' w/n 1 wk - Hb should stabilize 10 g/L/wk |
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What is the pathophysiology of 'anemia of chronic disease'?
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Infection/malignancy/inflammation/autoimmune/renal disease/live disease/endocrine diseases cause an anemia of UNDERPRODUCTION secondary to impaired iron utilization.
Impaired iron utilization is due to either: - iron trapping within enterocyte - iron trapping within macrophage - BM is unrspnsive to N/elevated EPO |
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How is anemia of chronic disease diagnosed?
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= a diagnosis of exclusion
- ass'd w/ acute phase reactants (ESR, CRP, fibrinogen) - often co-exisits with Fe-def'y - peripheral smear is usu: - normocytic & normochromic if mild - microcytic & normochromic if moderate - microcytic & hypochromic if severe |
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What is sideroblastic anemia?
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Normally erythrocytes have iron-containing granules scattered through-out the cytoplasm.
In sideroblastic anemia a defect in the heme biosynthesis of erythroid precursors leads to iron deposition in the mitochondria. These are large, abn granules that can be seen as a 'ring' around the nucleus. |
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List 3 causes of sideroblastic anemia.
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1. Hereditary - X-linked, survival ~10 yr
2. Idiopathic Acquired - subtype of MDS 3. Reversible - drugs, etoh, lead, Cu def, hypothyroid |
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Symptoms and signs of sideroblastic anemia.
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Sx - typical of anemia
Signs - hepatosplenomegaly, Fe-overload syndrome |
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Diagnosis of sideroblastic anemia?
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1. Serum Fe indices:
- inc'd serum Fe - inc'd ferritin - inc'd sTfR 2. Peripheral blood film - ringed sideroblasts - hypochromic & micro/normo/macrocytic |
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What are 4 causes of NORMOcytic anemia?
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Mnemonic: ABCD
A - aplastic anemia B - blood loss C - chronic disease D - destruction (hemolysis) |
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Define aplastic anemia.
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= destruction of hematopoietic cells of the BM l/t hypocellular BM and pancytopenia
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List >5 causes of aplastic anemia.
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Congenital - Fanconi's, Shwachman-Diamond syndrome
Acquired - chemo, toxins (DDT), idiopathic T-cell med'd Ionizing radiation Post-viral infection - parvovirus B19, EBV, HIV, HHV6 Autoimmune - SLE |
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Sx and signs of aplastic anemia.
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Sx - typical of anemia/thrombocytopenia
Signs - NO hepatosplenomegaly |
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Diagnosis of aplastic anemia?
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1. CBC
- anemia, neutropenia, thrombocytopenia, +- pancytopenia, dec'd retics 2. Peri smear - dec'd number of N RBCs 3. BM: - aplasia or hypoplasia with FAT replacement - dec'd cellulariy |
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Treatment of aplastic anemia?
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1. Treat underlying cause
2. RBC, plt transfusions 3. Immunosuppression: - anti-thymocyte globulin or - cyclosporin 4. Allogenic BM transplant |
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How can the etiologies of normocytic anemia be subcategorized? What is the DDx of each category?
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1. INHERITED
(a) Hemoglobinopathy - sickle cell, thalessemia (b) Membrane - spherocytosis (c) Metabolic - glycolytic pathway 2. ACQUIRED (a) Immune (:. Coombs-postive) - cold-agglutin, drug-induced (b) Infection - malaria (c) Microangiopathic hemolytic anemias - DIC, HUS, TTP, HELLP (d) Oxidative/drug-related |
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How can the etiologies of macrocytic anemia be subcategorized? What is the DDx of each category?
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1. MEGALOBLASTIC (DNA-synthesis problem)
- B12 def'y - folate def'y - drugs that impair DNA synth. (MTX, sulfa, chemo) 2. NON-MEGALOBLASTIC (membrane abn d/t cholesterol metabolism problem) - liver disease - etoh-ism - hypothyroidism - myelodysplasia |