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Understanding Blood Pigments in Pathology

Explore the classification and breakdown of hemoproteins derived pigments in cellular pathology. Learn about hemochromatosis, jaundice, and different types of icterus. Discover causes and treatments for disorders related to blood pigment breakdown.

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Understanding Blood Pigments in Pathology

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  1. General Pathology Basic Principles of Cellular and Organ Pathology Blood Derived Pigments Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague http://www1.lf1.cuni.cz/~jdusk/

  2. Pigments Definition: colored substances in the organism or environment

  3. Pigments Classification: • endogenous • hemoproteins derived • autogenous • exogenous

  4. Hemoproteins Derived Pigments -colour substances originating from hemoglobin modification or breakdown

  5. oxyhemoglobin hematin (Fe3+) methemoglobin (globin + hematin) carboxyhemoglobin Hemoproteins Derived Pigments

  6. Blood Pigment Breakdown • extravascular • intravascular

  7. ExtravascularBlood Pigment Breakdown Hemoglobin biliverdin + globin + Fe biliverdin  bilirubin

  8. ExtravascularBlood Pigment Breakdown Globin  aminoacids Fe hemosiderin Fe(OH)3 + protein carrier hematoidin= tissue bilirubin (+ceroid)

  9. Hemosiderin - Features • brown • with acid potassium ferocyanide solution forms a deep blue product Prussian blue (Perls´ reaction)

  10. Hemochromatosis • autosom. recessive 6th chromosome – mutation of transferin. receptor ass. protein prevalence 0,3–0,8 % (!!!) • deblocation of Iron intake (Iron need = cca 1–2mg/day) • deposits of HEMOSIDERIN: pancreas (diabetes bronze), myocardium, pituitary, joints, liver PHLEBOTOMY THERAPY

  11. Blood Pigment Breakdown • extravascular • intravascular

  12. IntravascularBlood Pigment Breakdown Hemoglobinbound to haptoglobin (2– globulin) + Fe biliverdin bilirubin urobilinogen enterohepatal stercobilinogen circulation urobilin stercobilin

  13. Bilirubin Processing • transport to the liver • hepatocyte entry – hepatocyte blood pole • glucuronylation • output to bile - hepatocyte bile pole

  14. Icterus –Jaundice Definition: a condition in which the tissues are yellowish due to the increase of bilirubin concentration (normal plasma: 1mg% unconj. bilirubin bound to albumin)

  15. Bilirubin Processing • transport to the liver • hepatocyte entry – hepatocyte blood pole • glucuronylation • output to bile - hepatocyte bile pole

  16. generalized prehepatal flavin hepatal ruby posthepatal verdant icterus melas local surrounding hematoma Icterus – Jaundice

  17. Icterus • hemolytical (dynamic) • hepatocellular (dissociated) • obstructive (resorptive) • mixed

  18. Icterus • hemolytical (dynamic) • corpuscular (hered. spherocytosis - def. G6P-dehydrogenase, thalassaemia) • extracorpuscular (icterus neonati simplex, icterus neonati gravis)

  19. Hemolytic Disease of the NewbornMorbus hemolyticus neonati – erythroblastosis fetalis • Rh- mother with an Rh+ fetus • anti Rh IgG crossing the placenta barrier • anaemia neonati • icterus neonati gravis • hydrops fetus universalis • abortus

  20. Ceroid • prevalence • erythrocytes breakdown places • fatty tissue necroses • avitaminosis E • melanosis coli • Dubin - Johnson syndrome

  21. Icterus • hepatocellular (dissociated) • hepatotoxic – acquired CCl4, amanitin, hepatitis epidemica, febris flava, leptospirosis, bact. sepsis... • enzymopathies – inborn (Crigler–Najjar, Gilbert, -glucuronyltransferase defect Dubin–Johnson, Rotor)

  22. Icterus • posthepatal – obstructive • acquired • obstruction • intraluminal • intramural, • extramural • inborn (primary billiary atresia)

  23. multiple intrahepatal both right and left hepatic ducts common hepatic duct choledochus papilla Vateri intraluminal - stone, ascaris… intramural - non-neoplastic (inflamm.), neoplastic… extramural – non-neoplastic,neoplastic combined Blockage of the Biliary Ways

  24. Icterus • hemolytical (dynamic) • hepatocellular (dissociated) • obstructive (resorptive) • mixed

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