Path revision Flashcards

1
Q

Increase in the number of cells =

A

Hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Increase in the size of cells =

A

hypertrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Cells that cant undergo hyperplasia - only hypertrophy

A

Cardiac muscle
Skeletal muscle
Nerve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Conditions that don’t have and increased risk of cancer despite hyperplasia =

A

Benigh prostatic hyperplasia
Apocrine metaplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Decrease in cell # occurs via

What changes occur in the cell during apoptosis

A

Apoptosis

decrease in size, red cytoplasm and condensed nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Decrease in cell size occurs via

A

Ubiquiten proteosome degradation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Causes of metaplasia

A

Stress, Vitamin A deficiency,

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Dysplasia

A

Disordered cell growth/proliferation of precancerous cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hallmark of reversible cell injury =
Hallmark of irreversible injury =
Hallmark of cell death =

A

cellular swelling

membrane damage

Loss of nucleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Necrosis =
what changes are seen in the cell?

Apoptosis =

causes of apoptosis - 2

A

Death of large groups of cells followed by ACUTE inflammation
cell swelling

Energy dependent (ATP) programmed cell death of single or small groups of cells - NO inflammation. - usually due to lack of oxygen or exposure to toxins/burns
Cell itself activates intrinsic enzymes that degrade the cells’ genomic DNA and nuclear and cytoplasmic proteins.
causes - 1 - normal physiological response 2- due to pathogenesis
eg cells with DNA damage
accumulation of misfolded proteins or due to infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Types of necrosis (6)

A

1 - Coagulative - cell shape & organ structure preserved - localised area of coagulative necrosis = infarct (doe not occur in brain)
2 - Liquefactive - brain infarct , abscess, pancreatitis
3- Gangrenous - coagulative necrosis that resembles mumified tissue
4 - Caseous - soft friable necrotic tissue - cheese like - combo of coagulative and liquefactive - TB fungal
5- Fat necrosis - pancreatitis +/- saponification
6 - Fibrinoid - necrotic damage to blood vessel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Apoptosis is mediated by ?
and activated by multiple pathways ?`

A

Caspases
Intrinsic mitochondrial pathway
Extrinisc receptor - ligand pathway
Cytotoxic CD8 T cell mediated pathway

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Free radicals cause damage via

Elimination is via

A

Peroxidation of lipids
Oxidation of DNA proteins

Antioxidants - glutathionine, Vit A, C and E

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Amyloid =

Can be systemic or local

A

misfolded protein which deposits in the extracellular space
Beta pleated sheet configuration
Congo red stain and green apple birefringence

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Primary amyloid =

Secondary amyloid =

A

AL amyloid - associated with MM derived from immunoglobulin light chain
AA amyloid - derived from serum amyloid protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Inflammation -
Acute or chronic

Acute inflammation mediators?

A

Acute - Oedema and neutrophils

Chronic - Lymphocytes and plasma cells

Toll like receptors
Arachidonic acid metabolites
Mast cells
complement
Hageman Factor (F12)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Neutrophil arrival and function

A

Margination
Rolling
Adhesion
Transmigration and chemotaxis
Phagocytosis
Destruction of phagocytosed material
Resolution

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Neutrophils are attracted by bacterial products - ?

A

IL-8
C5a
LTB4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Chronic granulomatous disease =

A

poor o2 dependent killing which leads to recurrent infection and granuloma formation with catalase positive organisms - KNOW
- staph aureus
- pseudomonas cepacia
- Serratia marcescens
- No cardia
- Aspergillus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What test is done to screen for CGD?

A

Nitroblue tetrazolium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

T lymphocytes

A

Produced in the BM as progenitor T cells and further develop in the thymus where they undergo rearrangement and become CD4+ helper T cells or CD8+ cytotoxic T cells
T cells use TCR complex for antigen surveillance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

T lymphocytes

A

Produced in the BM as progenitor T cells and further develop in the thymus where they undergo rearrangement and become CD4+ helper T cells or CD8+ cytotoxic T cells
T cells use TCR complex for antigen surveillance
CD8+ t cells - killing occurs via secretion or perforin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Granuloma =

Caseous granuloma

Non caseous granuloma

A

Collection of epitheliod histiocytes surrounded by giant cells and rim lymphocytes

Caseating - central necrosis - TB or fungal

Non-caseating - no central necrosis - FB, sarcoid, beryllium, Crohns, Cat scratch

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Primary immunodeficiencies (8) -

Di George

Severe combined immunodeficiency

x-linked agammaglobulinemia

Common variable immunodeficiency (CVID)

IgA deficiency - MOST COMMON

Hyper IgM syndrome

Wiskott-Aldrich syndrome

Complement deficiency

A

Di George = failure of the 3rd and 4th pharyngeal pouches to form normally. 22q11 microdeletion.
Tcell deficiency - lack of thymus and parathyroids

SCID - defective cell mediated and humoral immunity

x-linked agamma - complete lack of immunoglobulin - due to disordered B cell maturation. mutated BRUTON TYROSINE kinase

CVID - low immunoglobulin due to B cell or helper T cell defect

IgA def - low serum and mucosal IgA

Hyper IgM syndrome - Elevated IgM - mutated CD40 on T helper cells or CD40 receptor on B cells. Low IgA, IgG and IgE

Wiskott - Thrombocytopenia
Mutation in the WASP gener

Complement defi - C5-C9 deficiencies - Increased risk of Neisseria infection. C1 inhibitor deficiency - angiooedema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Autoimmune disorders (4)

A

SLE
Sjogrens
Scleroderma
Mixed connective tissue disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

SLE =

A

Antibodies against host damages multiple tissues via type 2 and 3 HS reaction.
LIBMAN-SACKS - endocarditis - small sterile deposits BOTH sides of the valve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

SLE =

Antihistone antibody is common in drug induced SLE

Association?

A

Systemic autoimmune disease
Antibodies against host damages multiple tissues via type 2 and 3 HS reaction.
LIBMAN-SACKS - endocarditis - small sterile deposits BOTH sides of the valve
Characterised by ANA and AntidsDNA antibodies

Associ - Antiphospholipid antibody syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Sjogrens syndrome

A

Autoimmune destruction of lacrimal and salivary glands
Lymphocyte mediated damage - Type IV HS with fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Sjogrens syndrome

Associated with ?

A

Autoimmune destruction of lacrimal and salivary glands
Lymphocyte mediated damage - Type IV HS with fibrosis
Characterised by ANA and antiribonucleoprotein antibodies

Assoc - rheumatoid arthritis
Increased risk for marginal zone B cell lymphoma (MALT)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Scleroderma

Diffuse or localised

Diffuse =

Localised =

A

Autoimmune tissue damage with activation of fibroblasts and dposition of collagen (fibrosis)

Diffuse - skin and early visceral involvement. Eosophagus common.
Characterised by ANA and AntiDNAtopoisomerase I antibody

Localised - skin + late visceral involvement
CREST syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Mixed connective tissue disorder.

A

Autoimmune mediated tissue damage with mixed features of SLE, systemic sclerosis and systemic sclerosis, polymyositis

Characterised by serum antibodies against U1 ribonucleoprotein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Granulation tissue is made up of -

What removes type III collagen?

What is required as a co factor?

A

Fibroblasts (deposits Type III collagen), capillaries and myofibroblasts.

Collegenase

Zinc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Cutaneous healing occurs via?

A

Primary or secondary intention

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Clonality of cells is determined by ?

Clonality of Blymphocytes is determined by

A

G6PD or
androgen receptor isoforms

Ig (light chains)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Most common adult ca by incidence?
Mortality?

A

incidence - 1 = brst/prostate
2 = lung 3 = colorectal
Mortality - 1 = lung 2 = brst/prostate 3 = colorectal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

DNA damage disrupts key regulatory systems
1) proto-oncogenes
2) Tumour suppresor genes
3) Regulators of apoptosis

A

Proto-oncogenes - GF, GFR, signal transducer, nuclear regulators, cell cycle regulators

Tumour supressor genes - P53 and Rb (retinoblastoma)
both regulate progression of cell cycle from G1 to S phase.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Li Fraumeni syndrome

A

Germline mutation of P53
leads to multiple types of carcinomas and sarcomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Sporadic mutation of RB =
Germline mutation of RB =

A

Unilateral retinoblastoma
Bilateral retinoblastoma and osteosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Bcl2 - normally stablisizes mitochondrial membrane blocking release of cytochrome C (activates apoptosis)

Bcl2 is over expressed in

A

FOLLICULAR LYMPHOMA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Lymphatic spread is characteristic of ?

Haematogenous spread is characteristic of ?

A

Carcinomas

Sarcomas and some carcinomas
- HCC
- RCC
- follicular ca of the thryoid
- choriocarcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Primary and secondary haemostasis
Primary =
Secondary =

A

P = weak platelet plug mediated by interaction of platelets with vessel wall
S = stablises platelet plug mediated by coagulation cascade.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

vWF is derived from ?

A

Weibel palade bodies of endothelial cells and alpha granules of platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Primary haemostasis disorders
2 Quantitative and 4 qualitative

A

ITP - immune thrombocytopenic purpura - MOST COMMON cause of thrombocytopenia in children and adults - autoimmune production of IgG against platelets

Microangiopathic haemolytic anaemia - pathologic microthrombi in small vessels - schistocytes. Seen in TTP and HUS
TTP - Decreased ADAMTS13 (cleaves vWF)
HUS - E.coli O157:H7 dysentry

  • Bernard-Soulier syndrome - GP1b deficiency
  • Glanzmann thrombasthenia - GPIIb/IIIa def
  • Aspirin
  • Uremia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Secondary haemostasis disorders (5)

Factors for coag cascade requires exposire to activating substances -
Tissue thromboplastin (activates F7) - ext
Subendothelial collagen (activates F12) - intrins

A

Haemophilia A (F8) deficiency

Haemphilia B (F9) deficiency

Coag factor inhibitor - acquired antibodies against coag factors - anti factor 8 most common

Von Willebrand disease - most common inherited disorder

Vit K deficiency - activates by epoxide reductase in the liver

Other = liver failure, large vol transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Other disorders of haemostasis

A

Heparin induced thrombocytopenia
DIC - pathologic activation of cascade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Disorders of fibrinolysis
Fibrinolysis normally removes thrombus after vessel has healed
usually due to overactivitiy of PLASMIN - enzyme that destroys blood clots by attacking fibrin.

A

Radical prostatectomy - released urokinase which activates PLASMIN
Cirrhosis - decreases production of alpha 2-antiplasmin
(normally inactivates plasmin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Thrombus is characterised by ? (2)

A

Lines of Zahn
Attachment to vessel wall.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Causes of endothelial damage = ?

What results in elevated homocysteine?
homocysteine = type of aa used to make proteins - normally vit B12, B6 and folic acide breaks it down and very little should be in blood stream.

A

atherosclerosis, vasculitis, high homocysteine levels

Vit B12 and folate deficiency
cystathionine beta synthase deficiency (SB)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Hypercoagulable state causes ?

A

Protein C or S deficiency
Factor V Leiden deficiency
Prothrombin 2010A
ATIII deficiency
OCP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Anaemia =

Based on MCV anaemia is classified as microcytic, normocytic or macrocytic

A

Decreased circulating RBC mass
Hb < 13.5 g/dl in males
Hb < 12.5 g/dl in females

MCV < 80 = microcytic
MVC 80 - 100 = normocytic
MVC > 100 = macrocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Microcytic anaemia - decreased production of Hb
Hb =

Types if microcytic anaemia ? (4)

A

Hb = heme + globulin
Heme = iron + protoporphyrin

Iron defic anaemia
Anaemia of chronic disease
Sideroblastic anaemia
Thalaseamia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Iron defic aneamia - iron is absorbed in the ?
What transports iron in blood?
How is iron stored?

What is Plummer vinson syndrome ?

A

Duodenum
via Transferritin
Bound to ferritin

iron def anaemia with eosophageal web, atrophic glossitis. Presents with anemia, dysphagia and beefy red tongue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Anaemia of chronic disease - chronic disease results in production of acute phase reactants from liver called ?

A

HEPCIDIN - sequestres iron in storage sites by 1) limiting iron transfer from macrophages to erythroid precursors and suppressing EPO production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Sideroblastic anaemia

A

Due to defective protoporphyrin synthesis
iron remains trapped in mitochondria and form a ring around the nucleus or erythroid precursors aka ringed sideroblasts
Can be congenital - ALAS

or acquired - alcohol, lead poisoning, Vit B6 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Thalassemia =

Carriers are protected against?

A

Decreased synthesis of globin chains of Hb

Plasmodium falciparum malaria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Thalassemia is divided into alpha and Beta globin chains
Normal types of Hb =

Alpha thalassemia is due to ?

Beta thalassemia is due to ?
minor and major

Cis deletion =
trans deletion =

A

HbF
HbA
HbA2

gene deletion

gene mutation
Minor -> reduced HbA, increased HbF and HbA2
Major -> NO HbA, increased HbF and HbA2

cis = both deletions on the same chromosome
trans = one deletion on each chromosome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Erythroid hyperplasia results in
(beta thalassemia major)

A

expansion of haematopoeiesis into skull and facial bones
extrameduallry haematopoeisis + hepatosplenomegaly
Increased risk of aplastic crisis with parvovirus B19 infection of erythroid precursors
nucleated RBC + target cells

58
Q

Causes of macrocytic anaemia

Where is folate absorbed?
what do you see/get with folate deficiency ?

Where is Vit B12 absorbed ?
What do you see/get ?

Amylase (saliva) releases B12 which binds to R-binder and carried to stomach. Pacreatic proteases in duodenum detach Vit B12 from R-binder. Vit B12 bind to intrinsic factor (released by parietal cells - gastric)
intrinsic factor - Vit B12 is then absorbed in______

Causes of B12 deficiency ?

Disorders?

A

Folate or vitamin B12 deficiency

Jejunum
Large RBC and hypersegmented neutrophils
Reduced serum folate
Increased serum homocysteine
NORMAL methylmalonic acid

Terminal ileum
Pernicious anaemia - macrocytic RBC + hypersegmented neutrophils

Autoimmune destruction of parietal cells = intrinsic factor defi
Pancreatic insufficiency
Damage to terminal ileum (crohns, fish tapeworm)
Diet - rare

Subacute degeneration of the spinal cord
Low serum B12
High serum homocysteine
INCREASED METHYLMALONIC ACID

59
Q

Normocytic anaemia is due to ?
How do you distinguish it from eachother ?

A

increased peripheral destruction or underproduction
Reticulocyte count - normal 1-2 %
if bone marrow responds by increasing RC to > 3% then there is good response and means peripheral destruction
Corrected count < 3% means poor response and suggests underproduction

60
Q

Peripheral RBC destruction can be intravascular or extravascular
Extravascular causes ?

A

Hereditary spherocytosis - defect in the RBC cytoskeleton membrane
HOWELL-JOLLY bodies seen on bld smear

Sickle cell anaemia

61
Q

Sickle cell anaemia =

Results in ?

Sickle cell disease vs trait

A

Autosomal recessive mutation in the beta chain of Hb
Single AA change replaces normal glutamic acid with VALINE
Haemaglobin C - glutamic acid is replaced by LYSINE

extravascular and intravascular haemolysis
Expansion of haematopoeisis into skull
Extramedullary haematopoeisis + hepatomegaly
Risk of aplastic crisis with parvovirus B19
irreversible sickling –> dactylitis, autosplenectomy which increases risk of infection with encapsulated organisms such as strep pneum, H. influenzae
increases risk of salmonella paratyphi osteomyelitis
HOWELL JOLLY bodies

Sickle cell disease = 90% HbS, 8% HbF, 2% HbA2, NO HbA

Sickle trait = 55% HbA, 43% HbS, 2% HbA2

62
Q

Causes of predominant intravascular haemolysis ? (5)

PNH
G6PD defic
Immune haemolytic anaemia (IHA)
Microangiopathic haemolytic anaemia
Malaria

What test is used to screen for PNH?

Main cause of death?

Complications include? (2)

A

Paroxysmal nocturnal haemoglobinuria = defect in myeloid stem cells resulting in absent GLYCOSYLPHOSPHATIDYLINOSITOL (GPI) which renders cell susceptible to destruction by complement
Decay accelerating factor (DAF) - normally protects cell from this. GPI secures DAF to cell membrane
Absence of GPI = absence of DAP - cell susceptible to complement mediated damage

Sucrose test - detects lack of CD 55 (DAF) on cell membrane

Thrombosis of hepatic, portal, or cerebral veins
(destroyed platelets release cytoplasmic contents into circulation inducing thrombosis

iron def anaemia and AML

63
Q

Glucose 6 phosphate dehydrogenase deficiency =

What is used to screen for disease ?

A

xlinked recessive disorder resulting in reduced half life of G6PD - renders cells susceptible to oxidative stress
H2O2 (oxidative stress) neutralised by glutathionine (antioxidant) normally which becomes oxidized in the process
2 variants - African - mild
Mediteranean - marked reduced half life of G6PD
Oxidative stress precipitates Hb as HEINZ bodies HEINZ bodies are removed by splenic macrophages resulting in bite cells

Heinz preparation is used to screen for disease

64
Q

Immune haemolytic anaemia =

What test is used to screen for it ?

A

Antibody-mediated destruction of RBC’s (IgG or IgM)
IgG - usually extravascular haemolysis
IgM - usually intravascular haemolysis

IgG binds RBS in warm environment (warm aggluttinin)
Assoc with SLE, CLL and certain drugs (penicillin and cephalosporin)

IgM (cold aggluttinin)
Assoc with mycoplasma pneum and infectious mono

COOMBS test - direct or indirect
Directs confirms presence of antibody coated RBCs. Anti IgG is added to patients RBCs and aggluttination occurs if RBCs already coated with antibody

Indirect - Confirms presence of antibodies in patients serum.

65
Q

Microangiopathic haemolytic anaemia =

A

intravascular haemolysis due to vascular pathology. RBCs are destroyed as they pass through circulation.
Occur with microthrombi due to TTP-HUS, DIC and HELLP. , prosthetic heart valves and aortic stenosis
Produce schistocytes on blood smear

66
Q

Malaria =

P.Falciparum = daily fever
P vivax and P ovale = fever everyother day

A

Infection of RBC and liver with Plasomodium transmitted by female Anopheles mosquito
RBCs rupture as part of life cycle. resulting in intravascular hemolysis and cyclical fever
spleen also consumes some infected RBCs resulting in mile extravascular hemolysis with splenomegaly.

67
Q

Anaemia due to underproduction (3)

A

Parvovirus B19 - infects progenitor red cells and temporarily halts erythropoeisis. Results in significant anaemia in the setting pf pre-existing marrow stress (eg sickle ell)
Anaplastic anaemia - damage to haematopoeitic stem cells resulting in pancytopenia and low reticulocyte count . biospy shows empty fatty marrow
Myelophthisic process - pathologic process that replaces BM. Haematopoeisis is impaired resulting ni pancytopenia

68
Q

Infectious mononucleosis

A

EBV infection lymphocytic leukocytosis - reactive CD8+ T cells. CMV less common cause

69
Q

Acute leukaemia =

Can be further subdivided into ALL and AML.

A

Neoplastic proliferation of of blasts - >20% of blasts in BM which crowds out the normal haematopoeisis.
Blasts are large immature cells with punched out nuclei

70
Q

ALL =
MOST COMMON in children and assoc with Downs
Subclassified into B-ALL and T-ALL based on surface markers
B-ALL is most common type of ALL.

A

neoplstic accumulation of lymphoblasts (>20% in BM)
lymphoblasts are positive nuclear staining for TdT+ (usually absent in myeloid blasts and mature lymphocytes)

B-ALL is characterised by lymphoblasts that express CD10, CD19 and CD20.
Prognosis based on cytogenic abnormalities
t(12;21) good prognosis seen in children
t(9;22) poor prognosis seen in adults (philideplhia + ALL)

T-ALL is characterised by lymphoblasts postive for Tdt+ and express CD2-CD8 markers - NO CD10
usually presents in teenagers with mediastinal mass.

71
Q

AML =

Subclassified into APL (acute promyelocytic leukaemia)
Acute monocytic leukaemia - lack MPO
Acute megakeryoblastic leukaemia - lack MPO. Assoco with downs syndrome

A

Neoplastic accumulation of myeloblasts (>20% in BM)
Usually positive staining for myeloperoxidase (MPO)
seen in ilder adults (50-60) Crystal aggregate on MPO may be seen as AUER RODS

72
Q

Chronic leukaemia =

Subtypes -
CLL,
Hairy cell,
ATLL,
Mycosis fungoides

A

Neoplastic proliferation of mature circulating lymphocytes
Characterised by a high WBC count. Seen in older adults

CLL = neoplastic proliferation of Naive B cells that express CD5 and CD20.
MOST COMMON leukaemia overall

Hairy cell leukaemia = Neoplastic proliferation of MATURE B cells. Cells are + for TRAP.
splenomegaly - NO lymphadenopathy.

Adult T cell Leukaemia/lymphoma - Neoplastic proliferation of mature CD4+ T cells
Assoc with ATLV-1

Mycosis Fungoides = neoplastic proliferation of mature CD4+ T cells that infiltrate the skin, producing a rash, plaques and nodules.
Aggregates in epidermis = Pautrier Microabscesses
cells can spread to the blood causing Sezary syndrome

73
Q

Myeloproliferative disorders included
CML
PV
Essential thrombocytopenia
Myelofibrosis

CML =

Polycethemia Vera =

Essential thrombocytopenia =

Myelofibrosis =

A

Neoplastic proliferation of mature myeloid cells. Driven by the philidelphia chromosome t(9;22)

Neoplastic proliferation of mature myeloid cells - esp RBCs (granulocytes and platelets also increase)
Assoc with JAK2 kinase mutation.

Neoplastic proliferation of mature myeloid cells, esp platelets. (RBC and granulocytes also increase)
Assoc with JAK 2 kinase mutation

Neoplastic proliferation of mature myeloid cells - esop megakaryocytes. Causes marrow fibrosis. Splenomegaly due to extramedullary haematopoeisis
Assoc with JAK2 kinase mutation

74
Q

Lymphadenopathy
painful - acute infection
painless - chronic infection, mets, ca, lymphoma

In inflammation, lymph node enlargement is due to hyperplasia of particular regions of the lymph node
Follicular =
Paracortex =
Sinus histiocytes =

A

hyperplasia of B cell region - seen in Rheum arth and early HIV

hyperplasia of T cell region seen in viral infections

hyperplasia due to draining of tissue with cancer

75
Q

Lymphoma is neoplastic proliferation of lymphoid cells that form a “mass” may arise in a lymph node or in extranodal tissue.

Divided into
Hodgkin (40%) and Non-Hodgkin (60%)
What are the malignant cells for each?

NHL is further classified into B or T cell

Small B cells =
Intermediate B cells =
Large B cells =

A

NHL = lymhoid cells

HL = Reed -sternberg cells

Small B cells = follicular lymphoma, mantle cell lymphoma, marginal zone lymphoma and small lymphocytic lymphoma

Intermediate = Burkitts lymphpma

Large = Diffuse large B cell lymphpma

76
Q

NHL
Follicular lymphoma =

Mantle cell lymphoma =

Marginal zone =

A

Neoplastic proliferation of small B cells (CD20+) that form follicle like nodules. Overexpression in Bcl2

Neoplastic proliferation of small B cells (CD20+) expands the mantle zone. PResents in late adulthood with painless LAD. overexpression of cyclin D1

Neoplastic proliferation of small B cells that expands the marginal zone. Assoc with chronic inflammation states. MALToma = marginal zone lymphoma in mucosal sites

77
Q

Burkitt lymphoma

A

neoplastic proliferation of intermediate sized B cells
Assoc with EBV.
presents as extranodal mass in child or young adult
African form = Jaw
Sporadic form = Abdo
overexpression of c-myc oncogene

78
Q

Diffuse large B cell =

A

Neoplastic proliferation of large B cells (CD20+) that grow diffusely in sheets.
MOST COMMON form of NHL
Sporadic or transformation of low grade lymphoma (follicular)

79
Q

HODGKINS lymphoma =

Subtypes
Nodular sclerosis - MOST COMMON
Lymphocyte rich - best prognosis
Mixed cellularity - abundant eosinophils
Lymphocyte depleted - most aggressive

A

Neoplastic proliferation of REED-STERNBERG cells
which are large B cells with multilobed nuclei and prominent nucleoli - positive for CD15 and CD30

80
Q

Plasma cell disorders - Dyscrasias

Multiple myeloma =

MGUS =

Waldenstrom Macroglobulinemia =

A

MM = malignant proliferation of plasma cells in BM
MOST COMMON malignancy of bone, metastatic ca
High serum IL-6
features - bone pain, hypercalcaemia, M -spike on electrophoresis, increased risk of infection, primary AL amyloid. Bence jones proteins (free light chains in urine)

MGUS - High serum protein with M-spike but NO OTHER features of MM.
5% of 70 yr olds. 1% develop MM each year.

Waldenstrom = B cell lymphoma with monoclonal IgM production. Generalised LAD, lytic bone lesions ABSENT
High serum protein with M-spike

81
Q

LCH - specialised dendritic cells found predominantly in the skin. Derived from BM monocytes

Subtypes
Lettere-Siwe disease
Eosinophilic granuloma
Hand Schuller-Christian disease

A

neoplastic proliferation of Langerhans cells
Characteristic Birbeck granules
cells are CD1a + and S100

Lettere-Siwe disease = malignant proliferation of Langerhans cells - skin rash, cystic skeletal defects in infant, may be fatal

Eosinophilic granuloma = Benign proliferation of Langerhans cells in bone. Can present as pathological fracture in adolecent

Hand-Schuller-Christian disease = Malignant proliferation of Langerhans cells. Scalp rash, lytic skull defects, Diabetes insipidus
Exopthalmos in children.

82
Q

Vasculitis
Large vessels =
Medium vessels =
Small vessels =

A

Large - aorta and branches
Temporal giant cell arteritis
Takayasu

Medium vessel - Muscular arteries supplying organs
Polyarteritis Nodosa
Kawasaki disease
Buerger disease

Small - arterioles, capillaries and venules
Wegners granulomatosis
Microscopic polyangitis
Churg Strauss
Henoch Schonlein Purpura

83
Q

Temporal arteritis =

Takayasu =

A

MOST COMMON form - adults > 50 F>M
Headache, visual dist, jaw claudication, polymyalgia rheum, high ESR

Granulomatous arteritis involving arch and branches - adults <50 yrs yng asian female
Visual and neurologic symptoms with weak absent pulses in upper extremity (pulseless disease)
ESR increased

84
Q

Polyarteritis Nodosa =

Kawasaki disease =

Buerger Disease =

A

Necrotising vasculitis - mutleiple organs - LUNGS SPARED
Yng adult with HTN
Abdo pain with melena
Neurologic dist and skin lesions
Assoc with serum HBsAg
STRING OF PEARLS sign - heals with fibrosis

Asian children < 4 yrs
fever, conjunctivitis, erythem rash of palms/soles, enlarged cervical lymphnodes
CORONARY art involvement
- thrombosis, MI, aneurysm/rupture

Necrotising vasculitis involving digits
Ulcertaion, gangrene, autoamput of fingers/toes
Highyl assoc with smoking

85
Q

Wegeners Granulomatosis =

Microscopic polyangitis =

Churg Strauss =

Henoch-Schonlein purpura =

A

necrotising granulomatous vasculitis involving nasopharynx, lungs, kidneys
Middle aged male with sinusitis or nasopharyngeal ulceration
Haemoptysis, bilateral nodular lung infiltrates, haematuria
C-ANCA positive

Necrotising vasculitis of multiple organs esp lung and kidney
P-ANCA positive
same presentation/treatement as wegners

Necrotising granulomatous inflamma with eosiniphils involving multiple organs, esp lung and heart
Asthma plus peripheral eosinophilia
P-ANCA positive

Vaculitis due to IgA immune complex deposition
MOST COMMON vasculitis in children
Palpable purpura on buttocks + legs
GI pain and bleeding and haematuria (IgA nephropathy)
Usually following upper resp infection
Self limited

86
Q

HTN - primary and secondary

Arteriosclerosis - 3 patterns
Atherosclerosis
Arteriolosclerosis - divided into ?
Monckeberg medial calcific sclerosis = ?

A

Hyaline and hyperplastic

Hyaline = proteins leak into vessel wall producing vascular thickening - cause = long term HTN or DM

Hyperplastic = hyperplasia of smooth muscle cause = malignant HTN

Monckeberg - calcification of media of muscular arteries - NON obstructive - not clinically significant

87
Q

Vascular tumours
Hemangioma - benign
Angiosarcoma - malignant proliferation of endothelial cells
Kaposi Sarcoma = ?

A

low grade malignanct proliferation of endothelial cells
Assoc with HHV-8
Purple patches, plaques and nodules on skin

88
Q

Stable angina = ?

Unstable angina = ?

Prinzmetal angina = ?

A

CP with exertion or emotional stress. Due to atherosclerosis of coronary artery >70% stenosis
Subendocardial ischaemia - ST depression

CP at rest - due to rupture of plaque and thrombosis/incomplete occlusion of vessel
Subendocardial ischaemia - ST depression

Episodic CP unrelated to exertion
Due to coronary art vasospasm
ST elevation due to transmural ischaemia

89
Q

LAD occlusion leads to infarct where?

RCA?

Left circumflex?

Troponin I for MI
CK MB used for ?

A

Ant wall and ant septum

Post wall and post septum

Lateral wall of LV

detecting reinfarction - normalised as 72 hrs

90
Q

Congenital heart defects
VSD
ASD
PDA
TOP
Transposition
Truncus arteriosus
Tricuspid atresia
Coarctation - adult and child forms

A

Large VSD can lead to Eisenmenger syndrome
ASD - normally ostium secundum (90%) - primum assoc with Downs
PDA assoc with congenital rubella
TOF - Stenosis of RV outflow tract, RV hypertrophy, VSD and aorta that overides VSD.
Transposition - assoc with maternal diabetes
Truncus arteriosus - single large vessel arising from both ventricles
Tricuspid atresia - RV hypoplastic
Coarctation - infant - assoc with PDA. Distal to arch but before PDA
Adult - distal to arch but no PDA

91
Q

Rheumatic fever = ?

A

Systemic complication from pharyngitis due to group A B hemolytic strep
2-3 weeks after strep pharyngitis

Bacterial M protein mimics proteins in human tissue
Dx based on Jones criteria
Evidence of previous grp A B haemolytic strep infection (elevated ASO or anti DNAse B titres) + major and minor criteria

Minor = fever, elevated ESR - non specific
Major = Migratory polyarthritis
Pancarditis - infection of all three layers
Subacute nodules
Erythema maginatum

92
Q

Endocarditis -
bacteria and what they affect
Strep viridans
Staph aureus
Stap epidermidis
Strep bovis
HACKE

A

Strep viridans = low virulence MOST COMMON infects previously damaged valves . Small vegetations dont destroy valve

Stap aureus = high virulence - IV drug use - infects normal valves - TRICUSPID. Large vegetations destroy valve.

Stap epidermidis - assoc with prosthetic valves

Strep bovis - associated with underlying colorectal ca

HACEK group - negative blood cultures

93
Q

Types of cardiomyopathies
Dilated
Hypertrophic
Restrictive

A

Dilated - dilatation of all 4 chambers. MOST COMMON. Systolic dysfunction due to idopathic cause
Hypertrophic - Massive hypertrophy of left ventricle - due to sarcomere protein mutation
Restrictive - causes; amyloid, sarcoid, haemochromatosis, endocardial fibroelastosis, Loeffler syndrome

94
Q

Cardiac Tumours
Myxoma
Rhabdomyoma
Metastases

A

Myxoma - benign- MOST COMMON in ADULTS
pedunculated, left atrium

Rhabdomyoma - Benign - MOST COMMON in CHILDREN. Assoc with TS
Ventricle

Mets - breast, lung, melanoma, lymphoma
Pericardium –> effusion

95
Q

Rhinitis = ?
Most common cause ?

Subtype = allergic rhinitis

A

inflammation of the nasal mucosa
Adenovirus

Type 1 HS characterised by eosinophils, assoc with asthma and eczema

96
Q

Nasal polyp - secondary to recurrent bouts of rhinitis and also in CF and aspirin intolerant asthma

A

Aspirin intolerant asthma characterised by triad of asthma, aspirin induced bronchospasm and nasal polyps

97
Q

Juvenile nasopharyngeal angiofibroma

A

Benign tumour of nasal mucosa - locally aggressive
adolescent males
Vividly enhancing soft tissue mass centered on the sphenopalatine foramen
prominent flow voids with salt and pepper appearance

98
Q

Nasopharyngeal carcinoma = ?

A

Malignant tumour of nasopharyngeal epithelium
Assoc with EBV

99
Q

Epiglottis =

A

inflammation of the epiglottis - most common cause = H. influenzae type B.

100
Q

Croup =

A

inflammation of the upper airway - most common cause = parainfluenzae virus

101
Q

Laryngeal papilloma

A

benign papillary tumour of the vocal cord. Due to HPV 6 and 11.

102
Q

Laryngeal carcinoma

A

Squamous cell carcinoma arising from epithelial lining of the vocal cord. Risk factors are alcohol and tobacco.

103
Q

Lobar pneumonia

Classic phases of lobar pneumoniae?

A

Usually bacterial - most common causes are streptococcus pneumoniae (95%) and Klebsiella pneumoniae

Congestion - due to congested vessels and oedema
Red hepatization - due to exudate, neutrophils and haemorrhage
Gray hepatization - due to degradation of red cells in the exudate
Resolution

104
Q

Bronchopneumonia

A

Scattered patchy consolidation centered around the bronchioles; often multifocal and bilateral

105
Q

TB due to inhalation of Mycobacterium tuberculosis
Primary and secondary

primary =

secondary =

A

Primary arises with initial exposure
focal caseating necrosis in the lower lobe and hilar lymph nodes that undergo fibrosis and calcification forming Ghon complex

Secondary arises with reactivation of Mycobacterium tuberculosis.
reactivation is commonly due to AIDS or ageing
Occurs at the apex
Forms cavitary foci of caseous necrosis and may lead to miliary TB or bronchopneumonia
AFB stain - acid fast bacilli
Systemic spread often occurs and can involve any tissue.

106
Q

COPD

Chronic bronchitis =

Emphysema =

Asthma and bronchiectasis

A

Group of diseases characterised by airway obstruction; lung does not empty and air is trapped.

chronic productive cough lasting atleast 3 months over a minimum of 2 years - highly assoc with smoking
characterised by hypertrophy of bronchial mucinous glands - leads to increased thicknedd of mucus glands relative to overall bronchial wall thickness
(REID INDEX increases to > 50%; normal is <40%)

Destruction of alveolar air sacs
Due to imbalance between proteases and antiproteases
Smoking is most common cause
A1AT is rare cause - results in pancinar emphysema in the lower lobes

107
Q

Pulmonary hypertension =

Primary and secondary

A

high pressure in the pulmonary circuit (>25 mm Hg , normal <10 mm Hg.
Characterised by atherosclerosis of the pulmonary trunk, smooth muscle hypertrophy of pulmonary arteries and intimal fibrosis.

Primary - young adult females
cause unknown.

Secondary - due to hypoxemia or increased volume in the pulmonary circuit. May also arise due to recurrent PE’s.

108
Q

RDS =

Neonatal RDS =

whats used to test for lung maturity ?

neonatal RDS is associated with?

A

Acute RDS - diffuse damage to the alveolar-capillary interface. Leakage of protein rich fluid leads to oedema and formation of hyaline membranes in alveoli.

Due to inadequate surfactant levels. Made by type II pneumocytes. phosphatidylcholine (lecithin) is the major component. surfactant normally decreases surface tension in the lung, preventing collapse of alveolar air sacs after expiration. Lack of surfactant leads to collapse of air sacs and formation of hyaline membranes.

amniotic fluid lecithin to sphingomyelin ratio
lecithin levels increase if surfactant is produced.
ratio of >2 = adequate production

Prematurity, C-section, maternal diabetes

109
Q

Lung Cancer - key risk factors = smoke, radon and asbestos

divided into 2 categories - ?

A

Small cell ca (15%) - NOT amenable to surgery - treated with chemo

Non small cell ca (85%) - surgical resection, does not respond well to chemo.
subtypes include;
- adenocarcinoma (40%)
- squamous cell carcinoma (30%)
- large cell carcinoma (10%)
- carcinoid tumour (5%)

110
Q

Staging of lung ca is based on ?

A

Tumour size and local extension
pleural involvement classically seen with adenocarcinoma
Obstruction of SVC
involvement of recurrent laryngeal or phrenic nerve
Compression of sympathetic chain leads to Horner syndrome
Spread to regional lymph nodes
unique site of distal mets = ADRENAL gland

111
Q

Cancer histology association location comment
Small cell carcinoma

Squamous cell

Adenocarcinoma

Large cell

Bronchoalveolar

Carcinoid

Metastasis

A

neuroendocrine cells, male smokers, central, rapid growth and early mets. may produce ADH or ACTH or cause Eaton-Lambert syndrome (paraneoplastic syndrome)

keratin pearls or intracellular bridges, male smokers, central, may produce PTHrP

Glands or mucin, non smokers, female, central or peripheral

poorly differentiated large cells, smoking, central or peripheral, poor prognosis

Columnar cells that grow along pre existing brocnhioles and alveoli, Not smoking related, peripheral, pneumonia like consolidation, excellent prognosis

Neuroendocrine cells, chromagranin positive, not smoking related, central or peripheral, polyp like mass in bronchus. Low grade malignancy, rarely can cause carcinoid syndrome

most common source is breast and colon carcinoma
multiple cannon ball like nodules on imaging. more common than primary tumours.

112
Q

Pleural lesions

A

Pneumothorax - accumulation of air in the pleural space.
Mesothelioma - malignant neoplasm of mesothelial cells

113
Q

Aphthous ulcer =

Bechet syndrome =

A

Painful superficial ulceration of the oral mucosa
arises in relation to stress and resolves spontaneously

Recurrent aphthous ulcers, genital ulcers and uveitis. Due to immune complex vasculitis involving small vessels
cause unknown. Can be seen in viral infections

114
Q

Oral herpes =

Squamous cell ca =

A

HSV 1

tobacco and alcohol is major risk factors
floor of mouth most common location
oral leukoplakia and erythroplakia are precursor lesions

115
Q

Salivary gland lesions
Mumps=

Sialadenitis =

Pleomorphic adenoma=

warthin tumour =

mucoepidermoid carcinoma =

A

mumps virus - bilateral inflammed parotid glands
orchitis, pancreatiis and aseptic meningitis may also be present

inflammation of the salivary gland. due to obstructing stone leading to staph aureus infection usuallu unilateral

benign tumour composed of stromal and epithelial tissue - MOST common tumour of the salivary gland
Arises in parotid - mobile, painless, circumscribed mass at the angle of the jaw. high rate of recurence
rarely transforms into ca

benign cystic tumour with abundant lymphocytes and germinal centres (lymph node like stroma) 2nd most COMMON tumour of salivary glands almost always in parotid

Malignant tumour composed of mucinous and squamous cells; most common MALIGNANT tumour of the salivary gland. arises in parotid and commonly involves the facial nerve

116
Q

Two types of chronic gastritis =

A

Chronic autoimmune gastritis - autoimmune destruction of gastric parietal cells which are located in the stomach BODY and FUNDUS.
Associated with antibodies against parietal cells and/or intrinsic factor.

H.pylori gastritis - MOST COMMON FORM (90%) H.pylori induced acute and chronic inflammation.
most common in the ANTRUM

117
Q

Peptic ulcer disease =

A

solitary mucosal ulcer involving the proximal duodenum (90) and distal stomach (10%)
Duodenal ulcer is almost always due to H. pylori. rarely may be due to ZE syndrome
biopsy shows hypertrophy of BRUNNER glands. Pain improves with meals

Gastric ulcer is usually due to H. pylori (75%) other causes - NSAIDS and bile reflux. Pain worsens with meals
ulcers usually at lesser curve of antrum.

Duodenal ulcers are almost never malignant

Gastric ulcers can be caused by gastric carcinoma
benign = < 3 cm sharply demarcated surrounded by radiating folds
malignant = larger, irregular with heaped margins

118
Q

Gastric carcinoma

subtypes
- intestinal
- diffuse

A

malignant proliferation of surface epithelial cells

Intestinal type(more common) presents as a large, irregular ulcer with heaped up margins; most commonly involves the lesser curve of the antrum.
Periumbilical region (sister mary Joseph)

Diffuse type characterised by signet ring cells that diffusely infiltrate the gastric wall.
spread can involve left supraclavicular (Virchows node)
Distant mets most commonly to liver.
Krukenberg tumour

119
Q

lactose intolerance =

celiac disease =

Tropical sprue =

A

Decreased function of the lactase enzyme found in the brush border of enterocytes

immune -mediated damage of small bowel villi due to gluten exposure; associated with HLA-DQ2 and DQ8

damage to small bowel villi due to unknown organism resulting in malabsorption.
Similar to coeliacs but occurs in tropical regions

120
Q

Whipple disease =

Abetalipoproteinemia =

Carcinoid tumor =

Carcinoid syndrome =

Carcinoid heart disease = ?`

A

Systematic tissue damage characterised by macrophages loaded with tropheryma whippelii organisms. Classic site of involvement is the small bowel lamina propria.

Autosomal recessive deficiency of apolipoprotein B-48 and B-100.

Malignant proliferation of neuroendocrine cells, low-grade malignancy
positive for chromagrannin
Can arise anywhere along the gut - small bowel is most common site’
Often secretes serotonin. Serotonin is released into the portal circulation and metablosed by the liver into 5-HIAA
Mets of carcinoid to the liver allows serotonin to bypass the liver metablism
Serotonin is released into hepatic veins and leaks into systemic circulation via hepato-systemic shunts, resulting in carcinoid syndrome and carcinoid heart disease.

Characterised by bronchospasm, diarrhea and flushing skin; symptoms can be triggered by alcohol or emotional stress
Charactersied by right sided valvular fibrosis leading to tricuspid regurg and pulmonary valve stenosis
LEFT sided lesions are NOT seen because of MONOAMINE OXIDASE (metabolises serotonin) in the lung.

121
Q

Hirschsprungs disease =

A

Defective relaxation and peristalsis of rectum and distal sigmoid colon - assoc with Downs syndrome.
Due to congenital failure of ganglion cells to descend into myenteric and submucosal plexus.
RECTAL suction biopsy reveals lack of ganglion cells.

122
Q

Colonic Polyps - raised protrusions of colonic mucosa

Hyperplastic polyps = ?

Adenomatous polyp = ?

A

hyperplasia of the glands - MOST COMMON
left colon (rectosigmoid). Benign with no maligant potential

Neoplastic proliferation of glands- 2nd most common type of colonic polyp.
Benign but pre- malignant - may progress to adenocarcinoma via the adenoma-carcinoma sequence

123
Q

Adenoma-carcinoma sequence =

A

molecular progression from normal colonic mucosa to adenomatous polyp to carcinoma
APC mutations increase risk for formation of polyp
K-ras mutation leads to formation of polyp
p53 mutation and increased expression of COX allow for progression to carcinoma; aspirin impedes progression from adenoma to carcinoma

124
Q

FAP =

Gardner syndrome =

Turcot syndrome =

A

Autosomal dominant disorder charactersied by 100s to 1000s of adenomatous polyps
Due to inhereted APC mutation (chromosome 5)

FAP with fibromatosis and osteomas

FAT with CNS tumors

125
Q

Juvenile polyp =

A

sporadic hamartomatous (benign) polyp that arises in children (<5yrs)
usually solitary rectal polyp that prolapses and bleeds
Juvenile polyposis = multiple juvenile polyps in stomach and colon. large numbers increase risk of ca

126
Q

Peutz Jeghers syndrome

A

Hamartomatous (benign) polyps throughout the GI tract and mucocutaneous hyperpigmentation on lips, oral mucosa and genital skin
Increased risk for colorectla, breast and gynaecological cancer.

127
Q

Colorectal carcinoma

pathaways ? =

HNPCC is due to ?

COLONIC carcinoma is associated with increased risk of what infection ?

What marker is used for assessing treatment response and detecting recurrence (nor for screening)

A

Carcinoma arising from colonic or rectal mucosa
most commonly from

adenoma-carcinoma sequence
Microsatelite instability

inherited mutation in DNA mismatch repair gene (microsatellite instability).
increased risk for colorectal, ovarian and endometrial cancer. arises de novo (not from adenomatous polyp) at early age and usually right sided

Strep Bovis endocarditis

CEA

128
Q

Pancreatic ca

tumour marker?

A

Adenocarcinoma arising from the pancreatic ducts.
risk factor = smoking and chronic pancreatitis.

Ca19-9

129
Q

gallstones - cholesterol or billirubin
MOST common is cholesterol stones
usually radiolucent

bilirubin stones - usually radiopaque
risk factors?

A

extravascular haemolysis
biliary tract infections -
Ascaris lumbricoides - round worm
Clonorchis senesis

130
Q

Jaundice - causes?
RBCs are consumed by macrophages
Protoporphyrin (from heme) is converted to unconjugated bilirubin (UCB)
Albumin carries UBC to liver
uridine glucuronyl transferase (UGT) in hepatocytes conjugates bilirubin

A

Extravascular haemolysis or ineffective erythropoesis (high levels of UCB overwhelms liver)
Physiologic in the newborn (low UGT activity)
Gilbert syndrome - low UGT activity
Crigler-Najjar syndrome - absence of UGT
Dubin-Johnson syndrome -
Biliary tract obstruction
Viral hepatitis

131
Q

Types of hepatitis ?

A

Hep A and Hep E - fecal oral. A = travelers, E - contaminated water or undercooked seafood. Only acute - no chronic

Hep B - parenteral transmission - acute or chronic

Hep C - parentral transmision - acute and chronic

Hep D -

132
Q

What is the 1st serologic marker to rise in acute hepatitis?

What marker shows immunization ?

A

HBsAg - if present >6 months = chronic state

HBsAB

133
Q

Viral hepatitis -

A

inflammation of liver parenchyma due to hepatitis virus
other causes - EBV and CMV

134
Q

Cirrhosis -

A

End stage liver damage - disruption of normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes. Mediated by TGF-B from stellate cells

135
Q

Haemachromatosis

A

excess body iron leading to deposition in tissues and organ damage due to free radicals
Due to autosomal recessive defect in iron absorption (primary) or chronic transfusions (secondary).

136
Q

primary haemochromatosis =

A

due to mutation in HFE gene usually C282Y
increased risk of HCC

137
Q

Wilsons disease =

A

Autosomal recessive defect in (ATP7B gene) in ATP mediated hepatocytes copper transport
Kayser Fleisher rings in the cornea
increased risk of HCC

138
Q

Primary biliary cirrhosis =

Primary sclerosing cholangitis =

A

autoimmune granulomatous destruction of intrahepatic bile ducts

inflammations and fibrosis of intrahepatic and extrahepatic bile ducts
periductal fibrosis with onion skin appearance
cause unknown but assoc with UC and P-ANCA +
increased risk of cholangiocarcinoma

139
Q

Reye syndrome -

A

Liver failure and encephalopathy in children with viral illness who take aspirin

140
Q

Hepatic adenoma

A

benign tumor of hepatocytes
assoc with OCP - regress if stopped
risk of rupture and intraperitoneal bleeding esp during pregnancy
tumours are subcapsular and grow with exposure to estrogen

141
Q

HCC

serum marker?

A

Malignant tumour of hepatocytes
Risk factors - HBV and HCV
Cirrhosis
Alfatoxins derived from aspergillus
increased risk for Budd-Chiari syndrome

AFP

142
Q

Normocytic anaemia with predominant intravascular haemolysis?

A

1- Paroxysmal nocturnal haemaglobinuria
2- G6PD deficiency
3- immune haemolytic anaemia
4- microangiopathic harmolytic anaemia
5- malaria