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MLAB 2401: Clinical Chemistry Keri Brophy-Martinez

MLAB 2401: Clinical Chemistry Keri Brophy-Martinez. Disorders of Iron, Porphyrins and Hemoglobin. Iron Deficiency. Iron depletion Diminished total body iron stores Functional iron not effected Advanced iron deficiency = ANEMIA Iron Deficiency Anemia(IDA) absolute iron deficiency

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MLAB 2401: Clinical Chemistry Keri Brophy-Martinez

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  1. MLAB 2401: Clinical ChemistryKeri Brophy-Martinez Disorders of Iron, Porphyrins and Hemoglobin

  2. Iron Deficiency • Iron depletion • Diminished total body iron stores • Functional iron not effected • Advanced iron deficiency = ANEMIA • Iron Deficiency Anemia(IDA) • absolute iron deficiency • Anemia of Chronic Disease(ACD) • functional iron deficiency

  3. Iron Deficiency • Populations affected • Children • Young women/Expectant women • Elderly

  4. Iron Deficiency Anemia • Decreased hemoglobin production due to decreased total body iron content • Causes of IDA: • Increased demand • pregnancy • Increased loss • Menstruation • GI bleed • Nutritional deficiency • Malabsorption • Crohn’s or celiac disease • Intestinal parasites

  5. 3 Stages of IDA • Stage 1 : Iron DEPLETION • Decrease in storage iron (ferritin decrease) • No anemia • RBC morphology normal • Stage 2: Iron-deficient Erythropoiesis • Decrease in iron for erythropoiesis • Decrease in ferritin, serum iron • Increased TIBC • No anemia: RBC slightly microcytic, no hypochromia • Stage 3 : Iron DEFICIENCY • Decrease in hgb, ferritin, serum iron • Decrease in peripheral tissue oxygen delivery • Increased TIBC • All lab tests abnormal • Microcytic, hypochromic anemia

  6. Iron Deficiency Anemia • Lab Features • Microcytic, hypochromic anemia • Anisocytosis, poikilocytosis • Total iron and ferritin decreased • TIBC increased

  7. Anemia of Chronic Disease • Decreased hemoglobin production due to decreased amount of free iron • Also known as anemia of chronic inflammation • Decreased bone marrow erythropoiesis

  8. Anemia of Chronic Disease • Lab features • Iron, TIBC decreased • Ferritin increased

  9. Iron Overload Syndromes Hemochromatosis Hemosiderosis Hereditary disorders of iron metabolism Hemosiderin is deposited within cells and interstitial fluid Primarily effects liver, heart, endocrine glands Irreversible Secondary/acquired iron accumulation Hemosiderin is deposited in cells only No tissue damage Reversible

  10. Hemochromatosis • Characterized by an increased rate of absorption of iron • Excessive iron deposits in organs, tissues, and interstitial fluid • Patient develops bronze color in the tissues • Total iron, percent saturation increased • TIBC decreased

  11. Iron Status in Disease States

  12. Hemoglobin Disorders • Refer to Hematology notes • Hemoglobinopathies • Thalassemia • Lab values • Increased ferritin

  13. Porphyrin Disorders= Porphyrias • Enzyme deficiencies resulting in overproduction of heme precursors in bone marrow or liver • Disorders of porphyrin metabolism = porphyrias • Inherited • Acquired - lead, alcohol and other toxins, iron deficiency, renal or liver malfunctions.

  14. Porphyrias • Classification • Based on • Specific enzyme deficiency • Hepatic vs erythropoietic • Cutaneous vs neurologic • Inherited or acquired

  15. Porphyrias • Clinical symptoms • Port wine colored urine • Cutaneous photosensitivity • Itchy skin (photodermitidis) • Hyperpigmentation • Inflammatory reaction occurs on exposure to ultraviolet light • Neurologic abnormalities

  16. Myoglobin • Elevations • Acute myocardial infarction • Renal failure • Vigorous exercise • Electric shock • Intramuscular injections

  17. LEAD • Clinical Features • Children • CNS symptoms: headache ,clumsiness, seizures, behavioral changes • GI symptoms: Abdominal pain, colic, constipation • Adults • Peripheral neuropathies, motor weakness, anemia

  18. References • Arneson, W. (2007). Clinical Chemistry: A Laboratory Perspective. Philadelphia, PA: F.A. Davis Company. • Bishop, M., Fody, E., & Schoeff, l. (2010). Clinical Chemistry: Techniques, principles, Correlations. Baltimore: Wolters Kluwer Lippincott Williams & Wilkins. • http://phosphorus15.glogster.com/ • Sunheimer, R., & Graves, L. (2010). Clinical Laboratory Chemistry. Upper Saddle River: Pearson . • Wier, Dr. Edward. (2010, December). Principles and Pathology of Iron Metabolism [PowerPoint slides].

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