Disorders of Iron, Porphyrins and Hemoglobin MLAB 2401: Clinical Chemistry Keri Brophy-Martinez.
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Transcript of Disorders of Iron, Porphyrins and Hemoglobin MLAB 2401: Clinical Chemistry Keri Brophy-Martinez.
Iron DeficiencyIron depletion
Diminished total body iron storesFunctional iron not effected
Advanced iron deficiency = ANEMIAIron Deficiency Anemia(IDA)
absolute iron deficiencyAnemia of Chronic Disease(ACD)
functional iron deficiency
Iron Deficiency AnemiaDecreased hemoglobin production due to
decreased total body iron contentCauses of IDA:
Increased demandpregnancy
Increased lossMenstruationGI bleed
Nutritional deficiencyMalabsorption
Crohn’s or celiac diseaseIntestinal parasites
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
Iron Deficiency Anemia
Lab FeaturesMicrocytic,
hypochromic anemia
Anisocytosis, poikilocytosis
Total iron and ferritin decreased
TIBC increased
Anemia of Chronic DiseaseDecreased hemoglobin production due to
decreased amount of free ironAlso known as anemia of chronic
inflammationDecreased bone marrow erythropoiesis
Iron Overload Syndromes
Hemochromatosis HemosiderosisHereditary disorders
of iron metabolismHemosiderin 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 damageReversible
HemochromatosisCharacterized 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
Iron Status in Disease StatesCondition Serum
IronTransferrin
Ferritin % Saturation
IDA Decreased Increased Decreased Decreased
Iron Overdose Increased Decreased Increased Increased
Hematochromatosis
Increased Slight Decrease
Increased Increased
Malnutrition Decreased Decreased Decreased Variable
Chronic anemia Decreased Normal/decreased
Normal/increased
decreased
Acute liver disease
Increased Variable Increased Increased
Hemoglobin DisordersRefer to Hematology notes
HemoglobinopathiesThalassemia
Lab valuesIncreased ferritin
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.
PorphyriasClassification
Based onSpecific enzyme deficiencyHepatic vs erythropoieticCutaneous vs neurologicInherited or acquired
PorphyriasClinical symptoms
Port wine colored urineCutaneous photosensitivityItchy skin (photodermitidis)HyperpigmentationInflammatory reaction occurs on exposure to
ultraviolet lightNeurologic abnormalities
MyoglobinElevations
Acute myocardial infarctionRenal failureVigorous exerciseElectric shockIntramuscular injections
LEADClinical Features
ChildrenCNS symptoms: headache ,clumsiness, seizures,
behavioral changesGI symptoms: Abdominal pain, colic, constipation
AdultsPeripheral neuropathies, motor weakness, anemia
ReferencesArneson, 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].