General Pathology Basic Principles

of Cellular and Organ Pathology

Blood Derived Pigments

Jaroslava DuškováInst. Pathol. ,1st Med. Faculty, Charles Univ. Praguehttp://www1.lf1.cuni.cz/~jdusk/

PigmentsDefinition:

colored substances in the organism

or environment

PigmentsClassification: endogenous

– hemoproteins

derived– autogenous

exogenous

Hemoproteins Derived Pigments

- colour substances originating from hemoglobin modification or breakdown

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

Blood Pigment Breakdown

extravascular intravascular

ExtravascularBlood Pigment Breakdown

Hemoglobin biliverdin + globin + Fe

biliverdin bilirubin

ExtravascularBlood Pigment Breakdown

Globin aminoacids

Fe hemosiderin

Fe(OH)3 + protein carrier hematoidin = tissue bilirubin

(+ceroid)

Hemosiderin - Features

brown

with acid potassium

ferocyanide solution forms a deep

blue product Prussian blue

(Perls´ reaction)

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,

liverPHLEBOTOMY THERAPY

Blood Pigment Breakdown

extravascular intravascular

IntravascularBlood Pigment Breakdown

Hemoglobin bound to haptoglobin (2– globulin) + Fe

biliverdinbilirubinurobilinogen enterohepatalstercobilinogen circulationurobilinstercobilin

Bilirubin Processing transport to the liver

hepatocyte entry – hepatocyte blood pole

glucuronylation

output to bile - hepatocyte bile pole

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)

Bilirubin Processing transport to the liver

hepatocyte entry – hepatocyte blood pole

glucuronylation

output to bile - hepatocyte bile pole

Icterus – Jaundice generalized

– prehepatal flavin

– hepatal ruby

– posthepatal verdant icterus

melas

local– surrounding

hematoma

Icterus

hemolytical (dynamic)

hepatocellular (dissociated)

obstructive (resorptive)

mixed

Icterus

hemolytical (dynamic)– corpuscular

(hered. spherocytosis - def. G6P-dehydrogenase, thalassaemia)

– extracorpuscular

(icterus neonati simplex, icterus neonati gravis)

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

Ceroid prevalence

– erythrocytes breakdown places

– fatty tissue necroses

– avitaminosis E

– melanosis coli

– Dubin - Johnson syndrome

Icterus hepatocellular (dissociated)

– hepatotoxic – acquiredCCl4, amanitin,

hepatitis epidemica, febris flava,

leptospirosis, bact. sepsis...

– enzymopathies – inborn(Crigler–Najjar, Gilbert, -glucuronyltransferase defect Dubin–Johnson, Rotor)

Icterus

posthepatal – obstructive– acquired

obstruction– intraluminal– intramural,– extramural

– inborn(primary billiary atresia)

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

Icterus

hemolytical (dynamic)

hepatocellular (dissociated)

obstructive (resorptive)

mixed

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 +++