1. Blood Derived Pigments
General Pathology
Basic Principles of Cellular and Organ Pathology
Blood Derived Pigments
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague

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

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

4. Hemoproteins Derived Pigments
- colour substances originating from hemoglobin modification or breakdown
daily iron need on average 1mg
– losses : bile, sweat
ferric form

5. Hemoproteins Derived Pigments
oxyhemoglobin
hematin (Fe3+)
methemoglobin (globin + hematin)
carboxyhemoglobin
ferric form of iron – trojmocné. Apoferitin up to 23% of iron.
Augmentation of iron absrorption: alcohol, vit. C., fructose
phytates, phophates, fats, calcium – impaired iron intake

6. Blood Pigment Breakdown
extravascular
intravascular

7. Extravascular Blood Pigment Breakdown
Hemoglobin 
 biliverdin + globin + Fe
biliverdin  bilirubin

8. Extravascular Blood 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. Intravascular Blood Pigment Breakdown
Hemoglobin bound to haptoglobin (2– globulin) + Fe
biliverdin
bilirubin
urobilinogen enterohepatal
stercobilinogen circulation
urobilin
stercobilin
unconjugated bilirubin is water insoluble, can be chemically measured only after alcohol (= lipid solvent miscible with water) is added.
Indirect van den Bergh test – diazotized sulfanilic acid

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)
visible amount – more than 10mg%

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

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

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

18. Icterus hemolytical (dynamic) corpuscular extracorpuscular
(hered. spherocytosis - def. G6P-dehydrogenase, thalassaemia)
extracorpuscular
(icterus neonati simplex, icterus neonati gravis)
both unconjugated and conj. bilirubin increased

19. Hemolytic Disease of the Newborn Morbus 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)
dissociated: unconjugated bilirubin increased, lack of bile acids due to hepatocyte destruction

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

23. 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

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

25. Icterus Cause Cholesterol Bile acids Hemolytic Hepatocellular
Obstructive
Cause
Bilirubin production
Input, conjug., output
Bile flow block
Serum bilirubin
Unconj.
Conj & unconj.
Conj.
Urine -urobilin, stercobilin +
+++
Urine - urobilinogen ++
- 0
Feces -stercobilinogen
+ - -
Cholesterol
Bile acids
Porucha intrahepat. žlučovodů zvýš. cholesterolu
Obstrukce extrahep. žlučovodů zvýš. žluč. kysel.