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PIGMENTS

COLOURED SUBSTANCES WHICH


MAY BE NORMAL CONSTITUENTS
OR ABNORMAL ACCUMULATIONS.

-EXOGENOUS
-ENDOGENOUS
EXOGENOUS PIGMENTS
- CARBON (COAL DUST)
(ANTHRACOSIS,
PNEUMONOCONIOSIS)
- TATTOO PIGMENT
ENDOGENOUS
- LIPOFUSCHIN (LIPOCHROME) (BROWN
ATROPHY IN HEART AND LIVER)
- MELANIN (MELAS-BLACK)
BROWN BLACK PIGMENT
FORMED WHEN TYROSINE IS
OXIDISED TO
DIHYDROXYPHENYLALAMINE
CATALYSED BY TYROSINASE
HOMOGENTISIC ACID
BLACK PIGMENT OCCURING IN
ALKAPTONURIA. DEPOSITED IN
CARTILAGE, CONNECTIVE
TISSUE AND SKIN.
(OCHRONOSIS)
HAEMOSIDERIN
YELLOW-GOLDEN BROWN PIGMENT
DERIVED FROM
HAEMOGLOBIN. FORMED FROM
FERRITIN WHEN THERE IS LOCAL
OR SYSTEMIC IRON EXCESS
BILIRUBIN
DERIVED FROM HAEMOGLOBIN
ACCUMULATIONS MOST EVIDENT
IN LIVER AND KIDNEYS. GREEN-
BROWN TO BLACK PIGMENT.
PATHOLOGIC
CALCIFICATION
ABNORMAL DEPOSITION OF
CALCIUM SALTS.
AND OTHER MINERAL SALTS).
DYSTROPHIC
TWO FORMS
METASTATIC
DYSTROPHIC
CALCIFICATION
NON VIABLE OR DYING TISSUES I.E.
NECROSIS.
- ATHEROMA, AGEING OR
DAMAGED CARDIAC VALVES
- TUMOURS (PSAMMOMA BODIES)
METASTATIC
CALCIFICATION
- OCCURS IN HYPERCALCAEMIA
E.G. HYPERPARATHYROIDISM,
IDIOPATHIC HYPERCALCAEMIA
OF INFANCY, MYELOMA
IMMOBILISATION, BONE
METASTASES
- AFFECTS KIDNEYS, BLOOD
VESSELS, LUNGS, GASTRIC
MUCOSA
- GENERALLY CLINICALLY SILENT
EXCEPT SEVERE NEPHROCALCINOSIS
AND LUNG INVOLVEMENT.
AMYLOIDOSIS
- PATHOLOGICAL PROTEINACEOUS
SUBSTANCE DEPOSITED BETWEEN
CELLS UB A WIDE VARIETY OF
CLINICAL SETTINGS.
- PINK, HYALINE, EOSINOPHILIC
- CONGO RED SALMON PINK
- GREEN BIREFRINGENCE WITH
POLARISED LIGHT
PHYSICAL NATURE OF
AMYLOID
- NON BRANCHING FIBRILS 7.5
10nm DIAMETER WITH B PLEATED
CONFIGURATION ON X-RAY
CRYSTALLOGRAPHY
- P COMPONENT (GLYCOPROTEIN)
CHEMICAL NATURE OF
AMYLOID
- AT LEAST 15 BIOCHEMICALLY
DISTINCT FORMS
- COMMONEST AL AND AA
(AMYLOID LIGHT CHAIN AND
AMYLOID ASSOCIATED)
- ATTR (TRANSTHYRETIN)
(PREALBUMIN)
- B2 MICROGLOBULIN
(HAEMODIALYSIS)
- B2 AMYLOID PROTEIN
(ALZEHEIMERS DISEASE)
- A CALCITONIN IN MEDULLARY
THYROID CARCINOMA
CLASSIFICATION OF
AMYLOIDOSIS
- SYSTEMIC OR LOCALISED

IRY (AL)
SYSTEMIC

IRY (AA)
HEREDITARY
PRIMARY (AL) IMMUNOCYTE
DYSCRASIA
- USUALLY SYSTEMIC
- COMMONEST IN US
- ASSOCIATED WITH PLASMA CELL
DYSCRASIA
REACTIVE SYSTEMIC
AMYLOIDOSIS
- MOSTLY AA TYPE (SECONDARY)
- ASSOCIATED WITH CHRONIC
INFLAMMATORY CONDITIONS RA,
Tb, TUMOURS, IBO)
- HEROINE ABUSERS
HAEMODIALYSIS
ASSOCIATED
- DERIVED FROM B2
MICROGLOBULIN
- LONG HAEMODIALYSIS
PATIENTS
HEREDOFAMILIAL
AMYLOIDOSIS
- FAMILIAL MEDITERRANEAN
FEVER (AA TYPE)
- FAMILIAL NEUROPATHIES
(ATTR)
LOCALISED AMYLOIDOSIS
- SINGLE ORGAN OR TISSUE
DEPOSITION
- LUNG, LARYNX, HEART, BLADDER
ETC.
- MAY BE AL OR AA
ENDOCRINE AMYLOID
- ISLET CELL TUMOURS OF
PANCREAS (TYPE II, D. MELLITUS)
- MEDULLARY THYROID
CARCINOMA
AMYLOID OF AGING
- SENILE CARDIAC, SPLENIC,
PANCREATIC, PULMONARY
- CEREBRAL AMYLOIDOSIS (A B2 IN
VESSELS AND ALZEHEIMERS
DISEASE)
MORPHOLOGY
AA TYPE KIDNEYS, LIVER,
SPLEEN, ADRENALS, LYMPH
NODES
AL TYPE GIT, PERIPHERAL
NERVES, HEART, SKIN,
RESPIRATORY TRACT, EYE
KIDNEY
- MAJOR CAUSE OF DEATH
- DEPOSITED IN GLOMERULI AND
VESSELS
SPLEEN:-
- SPLENOMEGALY
LIVER:-
- SPACE OF DISSE AND VESSELS
- HEPATOMEGALY
HEART:-
- AL (IN SYSTEMIC FORM)
- MAY BE LOCALISED (AGING)
- MYOCARDIAL PRESSURE
ATROPHY
- CONDUCTION SYSTEM IF
SUBENDOCARDIAL
OTHER ORGANS:-
- ADRENALS, GIT, INCLUDING
TONGUE (MACROGLOSSIA)
- LARYNX, CNS (ALZEHEIMERS
DISEASE, PERIPHERAL NERVES)
CLINICAL CORRELATION
- ASYMPTOMATIC
- RENAL INVOLVEMENT DOMINANT AND
MOST LIFE THREATENING
- CARDIAC INVOLVEMENT (HEART
FAILURE, ARRYTHMIAS, RESTRICTIVE
CARDIOMYOPATHY)
- GIT (MALABSORPTION,
MACROGLOSSIA)
DIAGNOSIS
CONGOPHILIA

BIOPSY IMMUNOPEROXIDASE
STUDIES

E. MICROSCOPY
PROGNOSIS
GENERALLY POOR IF SYSTEMIC
OR RELATED TO MULTIPLE
MYELOMA