Histopathology Notes

Cardiovascular Disease

BP = CO x SVR

CO = HR x SV

Infarction

·  Necrosis due to ischaemia

·  Arterial

o  MI

o  Stroke

o  Bowel infarction

o  Acute limb ischaemia

·  Venous

o  PE

o  Torsion of vascular pedicle

o  Sigmoid volvulus

o  Testicular torsion

Atherosclerosis

·  Arterial wall thickening and loss of elasticity

Stages

·  Endothelial cell injury

·  Inflammatory response in vessel wall

o  Oxidised LDL

·  Formation of stable atherosclerotic plaque

o  Vascular smooth muscle phenotype shift

·  Complication of stenosis or plaque rupture

Risk Factors

·  Non-Modifiable

o  Age

o  Sex

o  Family history

o  Others - Type A personality, oestrogen deficiency

·  Modifiable

o  Hypertension

o  Diabetes Mellitus

o  Smoking

o  Hypercholesterolaemia

o  Others - physical inactivity, lipoprotein Lp(a)

Complications

·  Plaque-related Stenosis (angina, intermittent claudication)

·  Plaque rupture

o  Thrombosis (MI, stroke, acute limb ischaemia)

o  Embolism (MI, stroke, acute limb ichaemia)

·  Weakening of vessel wall (AAA)

Acute Coronary Syndrome

·  Unstable angina

o  No cardiac damage (normal tropnin)

·  NSTEMI/STEMI

o  Cardiac damage (raised tropnin)

Acute Limb Ischaemia

·  Thrombosis (60%)

o  Hx of claudication/rest pain

o  Onset over hours

o  Signs of chronic vascularinsufficiency

o  Hard arteries

o  No bruits

·  Embolism (30%)

o  Recent MI, atrial fibrillation, aneurysm

o  Onset over seconds

o  No evidence of previous disease

o  Soft artery

o  Bruits

Hypertension

·  140/90 (based upon additional risk factors)

·  160/100 (absolute)

·  Primary (essential) - 95%

o  Idiopathic

·  Secondary

o  Renal disease

o  Endocrine disease

o  Pregnancy

o  Drugs

Sequalae

·  HTN retinopathy

·  CVA

·  ↑ Glucose levels

·  HTN encephalopathy

·  MI

·  HTN Cardiomyopathy

·  HTN Nephropathy

Cardiac Failure

A syndrome caused by any abnormality of the heart that may be characterised by a set of haemodynamic,

neural, and endocrine abnormalities

LVF

·  Causes:

o  IHD

o  Hypertension

o  Valve disease

o  Myocardial disease

·  Consequences:

o  Impaired pulmonary outflow

§  Congestion and oedema

o  Reduced renal perfusion

§  Salt and water retention + ATN

o  Reduced CNS perfusion (encephalopathy)

RVF

·  Causes:

o  Left-sided heart failure (Congestive)

o  Chronic Lung Pathology - Cor Pulmonale

·  Consequences:

o  Portal, systemic and peripheral congestion

o  Tricuspid regurgitation

o  Renal congestion (R > L)

Cardiomyopathy

Causes / Features
Hypertrophic / Inherited (50% AD)
Sporadic cases / Pathology - heavy muscular hypertrophy with poor compliance
Often asymmetrical septal hypertrophy
Sequelae:
- Arrhythmias - AF
- LV outflow obstruction
- CHF
- Sudden deathYoung man
Syncope, FH of sudden death, Jerky pulse and double apical impulse, Ejection systolic murmur (+/- mild mitral murmur)
Dilated / Idiopathic
Genetic
Infections e.g. viral myocarditis
Toxins - alcohol, chemotherapy / Four-chambered hypertrophy and dilatation
Poor prognosis
Progressive CCF
Progressive loss of mycoytes causing dilation + heart failure + arrhythmias
Restrictive / Pathology - restriction of ventricular filling with myocardial fibrosis
ARVD / Inflammation and thinning of the right ventricular wall
Usually due to mutation in cell adhesion genes / Key consequences:
- Arrhythmia
- CCF

Valve Disease

Congenital

·  Abnormalities e.g. Bicuspid valves

Acquired

·  Rheumatic fever/Endocarditis

·  Functional (e.g. Mitral/tricuspid valve disease)

·  Degeneration (e.g calcific aortic stenosis)

Stenosis

·  Pressure overload + hypertrophy

·  Develops slowly

Regurgitation

·  Volume overload

·  Develops quickly or slowly

Complications

·  Hypertrophy

o  LVF

o  Worsening of IHD, heart failure

o  Pro-arrhthymia

·  Dilation

o  Mitral and atria

·  Risk of Endocarditis

Types / Pathogenesis / Features
Rheumatic Fever / Acute inflammatory disorder of children (5-15)
Post-streptococcal (5 weeks) / Foci of fibrinoid necrosis → Aschoff Bodies, Fibrinous pericarditis, Valvulitis
Fribrous thickening + commissural fusion, MacCallum plaques
Infective Endocarditis / Acute / Usually stap. aureus
Very virilant, IV drug users, Very severe / Bulky vegitations with lots of erosion
May produce an abscess
Subacute / Usually strep. viridans
Less virulent, Prosthetic valves, Long course / Small vegetations with little erosion

Pathology of the Lung

Respiratory Failure

·  End-stage of all pulmonary disease

·  PaO2 < 8 kpa

Type I respiratory failure

·  Severe pneumonia, PE, asthma, fibrosis, LVF

·  V/Q Imbalance

o  CO2 - Compensation (pCO2 - normal/low)

o  O2 – No compensation

Type II respiratory failure

·  COPD, neuromuscular disease, severe acute asthma

·  Hypoventilation

o  Impaired transfer of Co2 and O2 (pCO2 elevated)

Pulmonary Embolus

·  95% of PE from deep vein thrombi in legs/pelvis

·  Large - Instant death (acute cor pulmonale)

·  Medium - Chest pain + pulmonary heamorrhage

·  Small - Clinically silent (multiple → Pulm. HTN)

·  Ix: ECG + D-Dimer

Primary Pulmonary Hypertension

·  Unknown cause

·  > 25mmHg pressure at rest

·  Young women

·  Complication → Right ventricular failure

·  Tx: vasodilators/lung transplantation

RVF

·  Causes:

o  Left-sided heart failure (congestive)

o  Chronic lung pathology → cor Pulmonale

·  Consequences:

o  Portal, systemic and peripheral congestion

o  Tricuspid regurgitation

o  Renal congestion (R > L)

Obstructive pulmonary disease

These are characterized by an increased resistance to airflow (low FEV1 and FEV1/FVC < 0.7)

Asthma

·  Kids > adults

·  Chronic airways inflammation that is usually reversible

·  Part of an atopic trait

·  Can be severe and life-threatening

·  Macroscopic

o  Overinflated, patchy atelectasis, mucus plugs

·  Microscopic

o  Oedema, pulmonary infiltrates (eosinophils), smooth muscle and mucoal gland hypertrophy

Chronic bronchitis

·  Chronic cough with production of sputum most days, for a least 3 months in 2 consecutive years

·  Usually smoking/old patients

Emphysema

·  Destruction/dilatation of the lung parenchyma distal to the terminal bronchioles

·  Can be young (congenital conditions) or old

·  Panacinar

o  Uniform destruction of acinus (lower basal zones)

o  A1AT deficiency

·  Centriacinar

o  Central/proximal respiratory tree (upper lobes/apices)

o  Smokers

Bronchiectasis

·  Permanent and abnormal dilatation of bronchi

·  Associated with inflammation

·  Congenital - Cystic fibrosis, severe immune deficiency

·  Post-infectious (severe viral, bacteria, fungal pneumonia)

·  Bronchial obstruction (tumour, foreign body)

·  Complications

o  Chronic infection with H. influenzae

o  Secondary infection with:

§  Staph. aureus

§  Moraxella catarrhalis

§  Pseudomonas

o  Right ventricular failure

o  Amyloidosis

Cystic Fibrosis

·  Autosomal recessive.

·  Mutation in CFTR gene → hyperviscous secretions

·  Complications:

o  Bronchiectasis (recurrent infections - staph, H. influenzae, P. Aeruginosa, B. Cepacia)

o  Pancreatic failure

o  Sperm maturation defects

Restrictive pulmonary disease

Pulmonary oedema

·  Causes:

o  IHD

o  Hypertension

o  Valve disease

o  Myocardial disease

·  Consequences:

o  Impaired pulmonary outflow

§  Congestion and oedema

o  Reduced renal perfusion

§  Salt and water retention + ATN

o  Reduced CNS perfusion (encephalopathy)

Pulmonary Fibrosis

·  Laying down of fibrotic tissue in the lung parenchyma

·  Most important restrictive pathology

o  FEV1/FVC > 0.7

·  Characteristic features:

o  Progressive shortness of breath

o  Cyanosis (+ clubbing)

o  Fine end inspiratory crackles

Extrinsic allergic alveolitis / Hypersensitivity Pneumonitis - Immune reaction from inhalation of antigens (fungal, bacterial, animal
protein, chemical)
Occupation-related
• Farmer’s lung (thermophilic actinomycetes)
• Bird fancier’s lung (avian proteins)
Pathology - interstitial pneumonitis + non-caseating granulomas
Pneumoconoses / Inflammatory lung conditions caused by inhalation of mineral dusts.
Lag Period of up to 30 years
Over-reaction to deeply seated dust particles with peristent inflammation
Types
• Coal dust (coal miners)
Lung nodules (coal macules) + massive fibrosis
• Silicosis (mining, quarrying, glass making)
Nodular fibrosis
• Asbestosis (ship building, construction)
•Diffuse fibrosis
Autoimmune / Sarcoidosis
Non-caseating granulomas + multisystem disease
Rheumatoid arthritis
Lupus
Systemic sclerosis
Ankylosing spondylitis
Drugs / Causes pneumonitis (may → fibrosis)
Reversible with early recognition
• Bleomycin
• Amiodarone
Radiation / Well-recognised complication of therapy for:
• Primary Lung Tumours
• Breast Carcinoma
1-6/12 after therapy

Lung cancer

·  Benign (rare)

o  Hamartomas,

o  Clear cell tumours

o  Papillomas

o  Fribromas

o  Asymptomatic

·  Malignant

o  Non-small cell - 80%

§  Squamous cell

§  Adenocarcinoma

§  Large cell undifferentiated/alveolar cell

o  Small cell (neuroendocrine) - 20%

·  Local effects

o  Bronchial obstruction → collapse

o  Impaired mucus clearance →infections

o  Invasion

o  SVC, brachial plexus, oesophagus

o  Extension through pleura/pericardium

o  Pleuritis and pericarditis

o  Lymphatic invasion → lymphangitis carcinomatosis

·  Distant effects

o  Peptide production

o  ACTH

o  ADH

o  PTH-like (particular to squamous)

o  Para-neoplastic syndromes

o  Lambert-Eaton myaesthenic syndrome, acanthosis nigrians, dermatomyositis

o  Metastases (common in bone, brain and liver)

Pneumothorax

·  Spontaneous

o  Primary - apical blebs

o  Secondary - COPD, asthma, pulmonary fibrosis, lung cancer

·  Acquired

o  Traumatic

o  Iatrogenic - central line insertion, pleural aspiration, barotraumas

Asbestos related lung-disease

·  Pleural plaques (marker of exposure)

·  Asbestosis (pulmonary fibrosis)

·  Adenocarcinoma (lung cancer - particularly in smokers)

·  Mesothelioma

o  Tumor of mesothelial lining (pleural, pericardial, peritoneal)

o  Epitheliod, sacromatoid, biphasic, desmoplastic

o  Estimated peak of disease around 2020

Hepato-biliary pathology

Hepatic Failure

Clinical syndrome occurring when > 90% functional capacity of liver is lost

Acute Liver Failure

·  Common causes

o  Acute Viral Hepatitis (AST > 1000)

§  Hep. A/B/C, CMV/EBV

o  Alcholic Hepatitis (AST < 300)

§  Raised GGT + MCV

o  Drug-related Hepatitis

§  Paracetamol, NSAIDS, antibiotics

·  Clinical Features

o  Hepatic encephalopathy

o  Irritability →sleep disturbance →disorientation → coma

o  Coagulopathy

o  Jaundice (conjugated)

o  Infection (sepsis + multi-organ failure)

o  Hepato-renal syndrome/hepato-pulmonary syndrome

Chronic Liver Disease

·  Persistent liver damage > 6 months without resolution

o  Progressive Fibrosis

o  Cirrhosis

Cirrhosis

Whole liver involved with triad of:

·  Fibrosis

·  Nodules of regenerating hepatocytes

·  Distortion of liver architecture

·  Complications

o  Hepatic failure (decompensation)

o  Hepatocellular carcinoma

o  Portal hypertension

o  Ascites

o  Porto-systemic shunts

o  Oesophageal varices, haemorrhoids, caput medusa

o  Splenomegaly (hypersplenism)

·  Causes

Cause / Deficit / Features
Alcohol / Alcohol byproducts activate Hepatic Stellate Cells → Fibrosis
3 distinct pathologies
- Alcoholic Hepatitis
- Alcoholic Fatty Liver
- Alcoholic Cirrhosis
Viral hepatitis
Steatohepatitis / Non-alcoholic liver disease
Similar appearance but associated with metabolic syndrome
? Insulin resistance (reduced peripheral lipolysis)
Continuum
- Non-alcoholic fatty liver disease
- Non-alcoholic steatohepatitis (may lead to cirrhosis)
Wilsonʼs disease / Disorder of ATPase Copper transport protein
Accumulation of copper in liver → hepatolenticular degeneration
AD transmission / Neurology + Kayser-Fleisher rings
Low caeruloplasmin + low copper
Haemochromatosis / HFE gene C282Y mutation → excessive GI absorption of iron
AR + manifests > 40 years old
Accumulation in multiple organs / Liver
– cirrhosis
– hepatocellular carcinoma
Heart
– Congestive cardiac failure
– Conducting defects and arrhythmia
– Constrictive pericarditis
Endocrine
– Diabetes
– Panhypopituitarism
– Hypogonadotropism
Joints
– Chondrocalcinosis
Loss of Libido
Alpha-1 anti-trypsin deficiency
Autoimmune / Middle aged women with co-existant autoimmune disease
High transaminases + raised IgG
ANA + anti-SM antibodies
Responds well to steroids

Primary biliary cirrhosis

·  Intrahepatic bile ducts (cholangiocytes)

·  Pruritus

·  Raised ALP + GGT

·  Anti-mitochondrial/IgM

Primary sclerosing cholangitis

·  Sclerosis of Intra- and extrahepatic ducts

·  Association with UC (75%)

·  Isolated raised ALP

·  Risk of cholangiocarcinoma

Hepatic Neoplasia

Benign Tumours

·  Haemangioma

o  Most common - 3% population

o  Asymptomatic (incidental finding)

·  Liver cell adenoma

o  Young women on COC

Malignant Tumours

·  Hepatocellular Carcinoma

o  Develop on background of cirrhosis/chronic inflammation

o  1/3rd→ DNA mismatch repaid

·  Cholangiocarcinoma

o  Malignancy of intrahepatic bile ducts

o  Non-specific presentation

o  Jaundice, weight loss, pruritus, pain

o  Present late with poor prognosis

Gallstones

·  Types:

o  Cholesterol (most common)

o  Pigment (haemolytic states)

·  Risk factors

o  Female

o  Forty

o  Fat

o  Fertile

o  Fair

Annular Pancreas

·  Failure of migration of the ventral bud

·  Obstructs duodenum

·  Presents in infancy with a similar picture to pyloric stenosis

Pancreas Divisium

·  Incomplete fusion of the ventral and dorsal ducts

·  Predisposes to

o  Chronic Pancreatitis

o  Pancreas Ca

Acute Pancreatitis

·  Inflammatory condition of the pancreas

·  Causes

o  Gallstones (45%)

o  Ethanol (25%

o  Trauma

o  Steroids

o  Mumps/Infections

o  Autoimmune

o  Scorpion venom

o  Hyperlipidaemia, hypercalcaemia

o  ERCP

o  Drugs

·  Symptoms:

o  Epigastric pain radiating to back

o  Relieved by sitting forward

o  Associated with vomiting

·  Signs:

o  Unwell

o  Fever

o  Tachycardia/pnoeia

o  Jaundice

o  Local/general peritonitis +/- shock and ileus

o  Cullen’s Sign

o  Grey-Turner’s signS

·  Complications

o  Respiratory: ARDS, atelectasis, pleural effusion

o  Organ failure: myocardial depression

o  DIC

o  Metabolic: hypocalcaemia, hyperglycaemia, met acidosis

o  Necrosis, pseudocyst , abscess, infection

Chronic Pancreatitis

·  Repeated episodes of pancreatic inflammation leading to structural damage and fibrosis

·  Results in exocrine and endocrine dysfunction

·  Symptoms

o  Wt loss

o  Poor appetite

o  Pain

o  Exocrine dysfunction

o  Endocrine dysfunction

·  Signs

o  Epigastric tenderness

o  Erythema ab igne

Pancreatic Tumours

·  M>F

·  Elderly

·  Western Countries

·  Risk Factors

o  Lifestyle: smoking, alcohol

o  Toxin exposure: naphthylamine (dye industry), benzidine

o  Disease states: chronic pancreatitis, diabetes

o  Congenital: pancreas divisum

·  Symptoms

o  Anorexia

o  Malaise

o  Weight loss

o  Chronic epigastric pain radiating to back

o  Steatorrhoea

o  Diabetes symptoms

·  Signs

o  Cachexia

o  Lymphadenopathy

o  Signs of EtOH use

o  Painless obstructive jaundice

o  Palpable gallbladder

o  Hepatosplenomaegaly and ascites

o  Thrombophlebitis migrans

o  Marantic endocarditis

Pathology of the Gut

Achalasia

·  Dysmotility disorder of the oesophagus

·  Degeneration of Auerbach plexi

·  Sequelae:

o  Dysphagia (liquids and solids)

o  Hypertrophy

o  Squamous cell carcinoma

Hiatus Hernia

·  Impaired lower

·  oesophageal sphincter

·  Reflux of gastric contents + oesophagitis

Reflux Oesphagitis/GORD

·  Acidic gastric contents → lower oesophagus

·  Risk factors:

o  Increased IAP (obesity, posture, large meals, alcohol)

o  Hiatus hernia

·  Complications

o  Peptic Stricture

§  Scarring and deformation → narrowing

o  Barretts Oesophagitis (10%)

§  Columnar metaplasia

o  Adenocarcinoma

Barrett’s Oesophagus

·  Oesophageal columnar metaplasia