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REVISION PART2

PATHOLOGY OF THE HEMOPOEITIC &LYMPHOID SYSTEM, BLOOD VESSELS, HEART, LUNG, KIDNEY, GIT, LIVER, GALL BLADDER, PANCREAS

Hematopoietic system

Hematopoietic system

Diagnostic approach to

ANEMIA

Diagnostic approach to ANEMIA

First of all- Think of the

following

What are the symptoms of the patient ?

What is the level of Hemoglobin?

What does the MCV reveal ?

What does the peripheral blood smear reveal ?

What is the type of anemia ?

What is the cause of this anemia?

What is the pathogenesis ?

What are the investigations for this type of anemia ?

To make it as simple as possible ?

Is this an IRON DEFICIENCY anemia?

Is this ANEMIA OF CHRONIC DISEASE?

Is this HEMOLYTIC ANEMIA ? Is this MEGALOBLASTIC ANEMIA ?

A simple formula

Anemia + Microcytic hypochromic red cells = try to think of iron deficiency anemia first.

Anemia + Normocytic red cells + chronic disease = try to think of anemia of chronic disease.

Anemia + evidence of red cell destruction = try to think of haemolytic anemia

Anemia + macrocytes = try to think of Megaloblastic anemia

Note : The MCV value will give you an idea about the size of the RBC (

normocytic/ microcytic/ macrocytic )

Aplastic anemia

Caused by bone marrow failure.

Pancytopenia

Bone marrow- hypocellular

Polycythemia

Increase in red cells/unit volume of peripheral blood

Types : absolute, relative

Type of absolute polycythemia : Primary, secondary

Investigation: Serum erthropoietin

Neoplastic proliferation of

white cells

LYMPHOID NEOPLASMS : ALL, LYMPHOCYTIC leukemias , NHL, Hodgkin lymphoma, myeloma

MYELOID NEOPLASMS : AML, Myeloproliferative neoplasms (CML, polycythemia vera etc.), myelodysplastic syndrome.

HISTIOCYTIC NEOPLASMS: rare

How do we make a diagnosis of leukemia ?

The WBC count is markedly raised. Acute leukemias show many blasts.

Then we have to find out :

Is this AML

Is this ALL

Is this CML

Is this CLL

Bone marrow examination is absolutely essential for the diagnosis of leukemia. Blast count > 20% = acute leukemias Depending on the type of blast we classify it as myeloblastic/ lymphoblastic ALL- more common in children AML- more common in adults

Bone marrow

aspiration/biopsy

Bone marrow aspiration/biopsy Absolutely essential for the diagnosis of leukemia / aplastic anemia/ other neoplastic

Absolutely essential for the

diagnosis of leukemia / aplastic

anemia/ other neoplastic disorders of marrow etc.

Lymphoma

HODGKIN LYMPHOMA :

Malignant REED STERNBERG CELL.

surrounded by reactive lymphocytes, macrophages etc.

Orderly spread by contiguity

Morphological types : Five

subtypes

WHO classification: morphology+ immunophenotype

Clinical staging : ANN ARBOR

Classification

NON HODGKIN LYMPHOMA

Several types

Extranodal involvement common

Clinical staging : ANN ARBOR

WHO classification : morphology +

immunophenotype

Investigation for lymphoma

Histopathological study of the affected lymph node

Immunophenotype

Karyotype etc.

BLEEDING DISORDERS

Due to PLATELET abnormalities

THROMBOCYTOPENIA ( Example: ITP)

Due to CLOTTING FACTOR abnormalities

Hemophilia Von Willebrand disease

Due to vessel wall abnormalities : example: Scurvy

Tests for bleeding disorders (

basic)

Platelet count

Prothrombin time(PT)

APTT

DIC

Discussed in the previous revision session.

Recap- Thrombohemorrhagic disorder caused by the systemic

activation of coagulation resulting in systemic microthrombi & activation of fibrinolysis.

Pathogenesis

triggered by either releases of tissue factor/ thromboplastic substance or by

widespread endothelial damage Results in platelet aggregation & formation of widespread microvascular thrombosis This results in ischemic tissue damage

The depletion of platelets & clotting factors along with the activation of

plasmin results in an associated bleeding diathesis.

Heart

CONGENITAL HEART DISEASE

ISCHEMIC HEART DISEASE

HYPERTENSIVE HEART DISEASE

VALVULAR HEART DISEASE

HEART FAILURE

Cardiomyopathy

pericarditis

Congenital heart disease

Left to right shunt

RIGHT TO LEFT SHUNT

ASD

early cyanosis is a feature

VSD

Tetralogy of Fallot

PDA etc

Transposition of the great

arteries etc.

Ischemic heart disease

Angina pectoris

Acute myocardial infarction

Chronic IHD Sudden cardiac death

MORPHOLOGIC CHANGES IN

MI

COAGULATION NECROSIS- begins at >4 hrs

12-24 HOURS- NEUTROPHILIC INFILTRATE APPEARS

7 10 days granulation tissue > 2 months- collagenous scar

Investigations for MI

ELECTROCARDIOGRAPH

TROPONIN/CK-MB

Consequences/ complications

of MI

VENTRICULAR RUPTURE

VENTRICULAR ANEURYSM

ARRHYTHMIA etc

ACUTE RHEUMATIC FEVER

Rheumatic fever is an acute immunologically mediated multisystem

inflammatory disease that occurs after group A beta haemolytic

streptococcal infection

Symptoms: Carditis, arthritis , fever etc

Jones criteria: Carditis, migratory polyarthritis, subcutaneous nodules ,

erythema marginatum along with raised ASLO.

Morphology : ASCHOFF BODIES , pancarditis

Pericarditis

Myocarditis

Valve involvement verrucae

hypertensive heart disease

Systemic ( left sided )

hypertensive heart disease.

Left ventricular hypertrophy

Complications- heart failure, IHD etc

Pulmonary hypertensive heart

disease

Right ventricular hypertrophy & dilation

Etiology : pulmonary

hypertension due to disorders of

lung parenchyma or pulmonary vasculature

Types : acute , chronic cor

pulmonale

Complication : right heart failure

LUNG

Pneumonia, lung abscess

Obstructive lung diseases

Restrictive lung diseases

Pulmonary embolism

Lung tumors

Pleural effusions

Acute lung injury

Atelectasis

Obstructive lung diseases

Bronchial asthma

Chronic bronchitis

Emphysema

Bronchiectasis

Bronchial asthma

A clinical syndrome characterized by episodic reversible airway

obstruction, bronchial hyperreactivity & airway inflammation.

Types of asthma : atopic , non atopic, drug induced, occupational

Pathogenesis : Type 1 hypersensitivity, acute & chronic airway inflammation, bronchial hyperesponsiveness.

Triggering of asthma ( sensitization) followed by immediate phase & the late phase

Morphology: Eosinophils ,Mucous plugs, Charcot Leyden crystals, Curschmann spirals

Clinical features : dyspnea + wheezing

Chronic bronchitis

Persistent production of cough for at least 3 consecutive months in

two consecutive years.

Risk factor_ cigarette smoking

Morphology- marked thickening of mucous gland layer

Emphysema

Chronic obstructive airway disease

Permanent enlargement of airspaces distal to terminal bronchiole

Types : Centriacinar smoking, Panacinar- alpha 1 antitrypsin deficiency

Bronchiectasis

permanent dilation of bronchi & bronchioles caused by destruction of

the muscle & elastic tissue , resulting from or associated chronic

necrotizing infection.

Pathogenesis : persistent infection + obstruction

Restrictive lung diseases (

some examples)

Pneumoconiosis

Sarcoidosis

Hypersensitivity pneumonitis

Pneumonia

Infection of lung

Types :

Community acquired pneumonia : Types - Acute , atypical

Hospital acquired pneumonia

Morphology: congestion, red hepatisation, grey hepatisation,

resolution

Complications: lung abscess, empyema, meningitis, arthritis, infective endocarditis

Pulmonary tuberculosis

Chronic granulomatous diseases caused by Mycobacterium

tuberculosis.

Pathogenesis :

Unexposed immunocompetent host : Primary pulmonary TB ( Ghon complex)

Secondary tuberculosis : arises in a previously sensitized host- upper parts of lung, caseation, cavitation

Atelectasis

Lung collapse

Types :

Resorption atelectasis Compression atelectasis Contraction atelectasis

Lung tumors

Risk factor for carcinomas- smoking

Types of lung tumors : adenocarcinoma, squamous cell carcinoma,

large cell carcinoma, small cell carcinoma, carcinoids.

Small cell carcinomas- best treated by chemotherapy.

Carcinoid- malignant tumors

Oral cavity

Oral inflammatory lesions

Proliferative & neoplastic lesions

Esophagus

Obstructive diseases

Reflux esophagitis

Esophageal tumors

Stomach

Inflammatory diseases : Gastritis

Neoplastic diseases :

polyps Adenocarcinoma:

INTESTINAL TYPE OF GASTRIC ADENOCARCINOMA ( PRESENTS AS AN EXOPHYTIC MASS)

DIFFUSE TYPE OF GASTRIC ADENOCARCINOMA ( LINITIS PLASTICA)

Small & large intestines

Intestinal obstruction

diarrheal disease

Inflammatory bowel disease

Colonic polyps

Adenocarcinoma

Liver, gall bladder, biliary tract

Jaundice

Hepatic failure

Cirrhosis

Porta hypertension

Fatty liver

Tumors

Cholelithiasis

Cholecystitis

Tumors

Patterns of hepatic injury

Hepatic degeneration

Intracellular accumulation

Hepatic necrosis & apoptosis

Inflammation

Regeneration

Fibrosis

Pancreas

Congenital anomalies

Acute & chronic pancreatitis

Pancreatic neoplasma

Etiologic factors in acute

pancreatitis

Metabolic example: alcoholism

Genetic

Mechanical- gallstones, trauma

Vascular- shock

Infection- mumps

Pathogenesis of acute

pancreatitis

Inappropriate activation of trypsin is the key triggering event

Net result: proteolysis, lipolysis, hemorrhage

Chronic pancreatitis

Inflammation with irreversible parenchymal destruction & fibrosis.

Etiology: long term alcohol abuse

Pathogenesis : recurring bouts of acute pancreatitis. Events include ductal obstruction, toxic effects, oxidative stress

Morphology: replacement of pancreatic acinar tissue by dense fibrous

connective tissue,

Kidney

Glomerular diseases

Nephrotic syndrome

Nephritic syndrome

RPGN

Tubulointerstitial diseases

Tubulointerstitial nephritis

Acute tubular necrosis

Cystic diseases of kidney : Simple cysts, autosomal dominant & recessive polycystic kidney disease

Obstructive diseases : renal stones, hydronephrosis

Tumors : renal cell carcinoma

Pathology of BLOOD VESSELS

ARTERIOSCLEROSIS

ATHEROSCLEROSIS : EPIDEMIOLOGY, RISK FACTORS, PATHOGENESIS ,

MORPHOLOGY, CONSEQUENCES

HYPERTENSIVE VASCULAR DISEASE

ANEURYSMS AND DISSECTIONS

VASCULITIS

TUMORS

Types & causes of

hypertension

Essential hypertension

Secondary hypertension

Renal example acute glomerulonephritis

Endocrine- example Cushing syndrome

Cardiovascular- example- coarctation of aorta

Neurologic

Vascular pathology in

hypertension

Hyaline arteriosclerosis

Hyperplastic arteriosclerosis

Atherosclerosis

Risk factors: Modifiable, non modifiable

Slowly progressive disease

affects large to medium sized muscular & elastic arteries

Pathogenesis : endothelial injury ,Smooth muscle proliferation

Morphology: atheromatous plaque

Atheromatous plaque = necrotic core, superficial fibrous cap composed of smooth muscle cells, inflammatory cells

Consequences of atherosclerosis

Stenosis Thrombosis

Aneurysm

Abnormal vascular dilation

Types of aneurysms: Abdominal aortic aneurysm, thoracic aortic aneurysm

Thoracic aortic aneurysm

Most common etiology- hypertension. Others Marfan syndrome, syphilis

Abdominal aortic aneurysm

Etiology: Most common cause of abdominal aortic aneurysm is atherosclerosis . Other causes include vasculitis, trauma ,mycotic aneurysms, inflammatory

Complication: rupture, compression of adjacent structures etc