General%20Pathology

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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/
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Pigments

• Definition
• colored substances
• in the organism
• or environment

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Pigments

• Classification
• endogenous
• hemoproteins derived
• autogenous
• exogenous

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Hemoproteins Derived Pigments

• - colour substances originating from hemoglobin
  modification or breakdown

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Hemoproteins Derived Pigments

• oxyhemoglobin
• hematin (Fe3)
• methemoglobin (globin hematin)
• carboxyhemoglobin

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Blood Pigment Breakdown

• extravascular
• intravascular

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ExtravascularBlood Pigment Breakdown

• Hemoglobin ?
• ? biliverdin globin Fe
• biliverdin ? bilirubin

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ExtravascularBlood Pigment Breakdown

• Globin ? aminoacids
• Fe ? hemosiderin
• Fe(OH)3 protein carrier
• hematoidin tissue bilirubin
• (ceroid)

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Hemosiderin - Features

• brown
• with acid potassium ferocyanide solution
  forms a deep blue product Prussian
  blue (Perls reaction)

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Hemochromatosis

• autosom. recessive
• 6th chromosome mutation of
  transferin. receptor ass. protein
• prevalence 0,30,8 (!!!)
• deblocation of Iron intake (Iron need cca
  12mg/day)
• deposits of HEMOSIDERIN
• pancreas (diabetes bronze), myocardium,
  pituitary, joints, liver
• PHLEBOTOMY THERAPY

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Blood Pigment Breakdown

• extravascular
• intravascular

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IntravascularBlood Pigment Breakdown

• Hemoglobin ?bound to haptoglobin (?2 globulin)
  Fe
• biliverdin
• bilirubin
• urobilinogen enterohepatal
• stercobilinogen circulation
• urobilin
• stercobilin

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Bilirubin Processing

• transport to the liver
• hepatocyte entry hepatocyte blood pole
• glucuronylation
• output to bile - hepatocyte bile pole

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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)

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Bilirubin Processing

• transport to the liver
• hepatocyte entry hepatocyte blood pole
• glucuronylation
• output to bile - hepatocyte bile pole

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Icterus Jaundice

• generalized
• prehepatal
• flavin
• hepatal
• ruby
• posthepatal
• verdant
• icterus melas

• local
• surrounding hematoma

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Icterus

• hemolytical (dynamic)
• hepatocellular (dissociated)
• obstructive (resorptive)
• mixed

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Icterus

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

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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

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Ceroid

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

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Icterus

• hepatocellular (dissociated)
• hepatotoxic acquired
• CCl4, amanitin,
• hepatitis epidemica, febris flava,
• leptospirosis, bact. sepsis...
• enzymopathies inborn
• (CriglerNajjar, Gilbert, -glucuronyltransferas
  e defect DubinJohnson, Rotor)

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Icterus

• posthepatal obstructive
• acquired
• obstruction
• intraluminal
• intramural,
• extramural
• inborn
• (primary billiary atresia)

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Blockage of the Biliary Ways

• 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

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Icterus

• hemolytical (dynamic)
• hepatocellular (dissociated)
• obstructive (resorptive)
• mixed

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Icterus Hemolytic Hepatocellular Obstructive
Cause Bilirubin production Input, conjug., output Bile flow block
Serum bilirubin Unconj. Conj unconj. Conj.
Urine -urobilin, stercobilin 0
Urine - urobilinogen - 0
Feces -stercobilinogen - -
Cholesterol
Bile acids