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General Pathology

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

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General 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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