SYSTEM, BLOOD VESSELS, HEART, LUNG, KI DNEY, GI T, LI VER, GALL BLADDER, PANCREAS Hematopoietic system 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 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 ASD VSD PDA etc RIGHT TO LEFT SHUNT early cyanosis is a feature Tetralogy of Fallot 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