Revision Flashcards
1
Q
Ach receptor blocked by autoantibodies resulting in weak , but not atrophied, muscles
A
Myaesthenia gravis
2
Q
Pernicious anaemia is lack of absorption of what? Why?
A
B12 due to lack of intrinsic factor
3
Q
what does ANCA stand for?
A
anti-neutrophil cytoplasmic antibodies
4
Q
3 forms ANCA vasculitis
A
Microscopy polynagitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis
5
Q
All forms of ANCA vasculitis have what? Some have what?
A
All- polyangitis
Some- granulomatosis
6
Q
ANA is positive in what type of raynauds?
A
Secondary
7
Q
Skin fibrosis and tightening in fingers and face, finger ulcers, lung fibrosis=? What antibody?
A
Scleroderma
ANA
8
Q
What is a positive predictive value?
A
Proportion of people with a positive test who have the target disorder (true positive/all positives)
9
Q
Type II hypersensitivity reaction called what? Which antibodies? Example?
A
Cytotoxic
IgM and IgG
Rh incompatibility
10
Q
Type III hypersensitivity reaction called what? Which antibodies? Example?
A
Immune complex
IgG/M (+ antigen = complex)
SLE
Serum sickness
11
Q
Type IV hypersensitivity reaction called? Mediated by what? Example?
A
Delayed
T cells
Contact dermatitis
12
Q
In a type I hypersensitivity reaction, Th2 cells secrete what, and what stimulates them to do so?
A
APCs stimulate them (using the allergen)
to secrete IL4 to B cells
and
IL-3,5,GM-CSF to eosinophils
13
Q
In a type I hypersensitivity reaction, B cells, having been activated by IL-4, do what?
A
Produce IgE which degranulates mast cells to stimulate the initial and late responses.
Also releases IL3 and IL5 to stimulate eosinophils
14
Q
In a type I hypersensitivity reaction, eosinophils, having been activated by IL3,5,GM-CSF, do what?
A
Degranulate and activate the late phase response.
15
Q
T cell deficient patients present with what? How to treat?
A
Opportunistic infection
Stem cell transplant, somatic gene therapy
16
Q
B cell deficient patients present with what? Treatment?
A
Recurrent pyogenic bacterial infection
Antibiotics then IV IgG
17
Q
X linked agammaglobulinaemia is a type of?
A
B cell deficiency
18
Q
Phagocyte deficient patients present with what?
A
Recurrent, prolonged infections that respond poorly to Abx.
Staphylococcal
Skin and mucous membranes
Supporative lymphadenopathy
19
Q
C1,2,4 deficient patients present with what?
A
Immune complex symptoms e.g malar rash, vasculitis
20
Q
C3 deficient patients present with what?
A
Increased susceptibility to bacterial infection- pneumonia, sepsis, meningitis
21
Q
C5-8 deficient patients present with what?
A
Recurrent neisserial infection (meningococcal meningitis)
22
Q
Immune stimulants other than immunisation?
A
alpha, beta, gamma interferons
IL-2
G/GM-CSF
23
Q
3 classes of immune suppressant
A
Corticosteroids
Lymphocyte targeting
DMARDs
24
Q
4 lymphocyte targeting therapies
A
Antimetabolites
Calcineurin inhibitors
M-TOR inhibitors
IL-2 receptor MABs
25
What are the classical HLA antigens? What region codes for them?
A, B, C
| Class I region
26
Class II region encodes which HLAs?
HLA-DR, DQ, DP
27
Class III region encodes which MHCs?
HSP70, TNF, C4a, C4b, C2, BF, CYP21
28
Where are all of the classical HLAs found?
All nucleated cells
29
What sort of expression do MHC genes have?
Codominant
30
3 types of recognition of the foreign body in transplantation?
Direct (T cells bind directly to graft)
Indirect (graft peptides)
Semi-direct (in lymph nodes)
31
What is cRF/sensitisation percentage?
percentage of deceased donors that will not be a suitable match
32
What is the minimum inhibitory concentration of an antibacterial agent?
Minimum concentration at which visible growth is inhibited
33
Aminoglycosides bind to what to inhibit protein synthesis?
30S
34
Macrolides etc. bind to what to inhibit protein synthesis?
50S
35
Tetracyclines bind to what to inhibit protein synthesis?
30S
36
Oxazolidinones bind to what to inhibit protein synthesis?
50S
37
Cephalosporins treat what?
Gram negative bacilli
38
Action of aciclovir? Used to treat?
Nucleoside analogue
| HSV, VZV
39
Rx CMV?
Ganciclovir
40
Rx influenza?
Oseltamivir and zanamavir
41
Rx Hep C and RSV
Ribavirin
42
Rx Hep C and B
Interferons
43
Ciprofloxacin is what type of antibiotic?
Quinolone
44
Co-amoxiclav is what type of antibiotic?
penicillin
45
Which antifungal is nephrotoxic?
Amphotericin B
46
Which antifungals are hepatotoxic?
Allylamines and azoles- ergosterol synthesis inhibitors
47
Ascariasis type of pathogen
Nematode (helminth)
48
Ascariasis where in body?
Digestive system, migrates to lung (Loeffler's syndrome)
49
Rx ascariasis?
Albendazole-benzimidazole
50
Schistosomiasis action in body?
Matures in GI blood vessels, exists in faeces after sexual reproduction
51
Definitive and intermediate hosts in schisto?
Def- human
| Int- snails
52
Definitive and intermediate hosts in hyatid?
Def- dogs
| Int- sheep
53
Type of pathogen in hyatid
Platyhelminth- tape worm
54
4 plasmodiums in malaria and which is most common
Ovale
Malariae
Vivax
Falciparium
55
Cryptosporidium spread
Faeco-oral human to human
56
Measles type of virus
Paramyxovirus
57
What involves cough, conjunctivitis, coryza?
Measles
58
What do you get prior to measles rash?
Koplik spots
59
Slapped cheek syndrome caused by which virus?
Parvovirus B19
60
'everything else' infant viral rash? Dx of exclusion
Enteroviral
61
Croup is caused by what?
Parainfluenza
62
Respiratory virus with conjunctivitis and diarrhoea is likely to be what?
Adenovirus
63
The frequency of reactions decreases with number of doses in live or inactivated vaccines?
Live
64
Difference between sterilisation and disinfection?
Sterilisation is killing all micro-organisms, disinfection is just killing sufficient numbers to make an item safe to use.
65
Disinfection applied to the skin is what?
Antisepsis
66
Decontamination method of surgical equipment
Moist heat
67
Decontamination method of syringe needle
gamma irradiation and disposal after use
68
Decontamination method of surgeons hands
surgial scrub
69
Decontamination method of flexible endoscope
Chemical
70
Decontamination method of catheter insertion site
Chemical
71
Pa O2 of respiratory failure
more than 8.0kpa
72
Type 1 resp failure pa O2 and what is driving it?
less than 6.3kpa, hypoxic
73
Type 2 resp failure pa O2 and what is driving it?
more than 6.3kpa, hypercapnic drived
74
4 types non-small cell lung carcinoma
Squamous
Adenocarcinoma
Undifferentiated large cell
Large cell neuroendocrine
75
What type of cancer can mimic pneumonia?
Bronchioloalveolar
76
CD56 detects what?
Neuroendocrine lung tumours
77
4 features typical carcinoid tumours? (lung cancer)
No necrosis, bland
Distant mets are rare
NOT assoc with smoking
May occlude bronchioles
78
TTF is expressed by which 3 lung cancers?
Adenocarcinoma
Undifferentiated large cell
Small cell
79
What colour asbestos is the worst?
Blue
80
Small plaques on parietal pleura + effusion =?
Mesothelioma
81
Which type of pneumonia in elderly and diabetic and alcoholic?
Klebsiella
82
3 distributions of emphysema
Centrilobar (smoking)
Panlobar (alpha-1-anti trypsin)
Paraseptal
83
Acute interstitial lung disease is?
Adult resp distress synd
84
What cellular change is seen in acute interstitial lung disease?
Type II pneumocyte hyperplasia
85
Appearance of chronic interstitial lung disease?
Honeycomb lung
86
What 2 serum substrates are increased in sarcoidosis?
Calcium and ACE
87
Dx criteria for chronic bronchitis?
Productive cough on most days during at least 3 months of 2 successive years (with no alternative cause)
88
90% lobar pneumoniae is due to which pathogen?
Strep. pneumoniae
89
2 pathogens that cause hospital acquired pneumonia?
Enterobateraceae
| Pseudomonas ssp
90
2 pathogens that are spread from person to person causing community acquired pneumonia
S pneumoniae
| H influenzae
91
Pathogen from the environment that might cause community acquired pneumonia
L pneumophilia
92
Animal derived cause of community acquired pneumonia
C psittaci
93
4 atypical pneumonias
Mycoplasma pneumonia
Legionella pneumophilia
Chlamydophila pneumonia
Chlamydophila psittaci
94
Rare complications of Mycoplasma pneumonia?
Neuropathies, guilliane barre
95
Incidence of Chlamydophila pneumonia is highest in what group?
Elderly
96
What score is used to quantify the severity of pneumonia?
CURB65
97
Paul Bunnell Test is for what?
EBV
98
Rx malignant otitis externa
ceftazidime then ciprofloxacin
99
3 features mycobacteria
```
waxy cell wall (high in lipids)
slow growing
acid fast
intracellular
combination therapy required
latent phase
```
100
2 mycobacteria that cause TB
| BCG is against which?
m. tuberculosis and m.bovis
| Bovis is BCG
101
how does primary TB present?
influenza like
102
What is another name for disseminated TB?
Miliary
103
After the TB pathogen has multiplied, what are the 2 things that can happen?
Latency or dissemination
104
Rx TB?
Rifampicin, isoniazide (4 months), pyrazinamide, ethambutol or streptomycin (2 months)
If pericarditis or meningitis then add steroids
Sites other than meninges- 6 months
105
Tuberculoid leprosy is what cell type response? What symptoms?
Th1
| Skin plaques and nerve involvement
106
Lepromatous leprosy is what cell type response? Symptoms?
Th2
Subcut tissue accumulation
Leonine facies
Earlobes and face
107
Rx m. leprae?
Dapsone, rifampicin, clofazimine
108
2 types oesophageal carcinoma?
Squamous cell
| Adenocarcinoma
109
Barrett's oesophagus leads to which cancer?
Adenocarcinoma of the oesophagus
110
4 types gastric carcinoma, which is most common?
ADENOCARCINOMA
Endocrine
MALT
Stromal
111
CDH1/E-cadherin mutation = ?
Hereditary diffuse type gastric cancer
112
Pathogenesis of coeliac disease?
Gliadin in gluten induces epithelial cells to express IL-15
Causes actiation of CD8+ and IELs
These are cytotoxic
113
2 cancer types assoc with coeliac?
Enteropathy- associated T cell lymphoma
| Small intestinal adenocarcinoma
114
3 investigative tests for coeliac?
IgA antibodies to TTG
IgA or G antibodies to deamidated gluten
Biopsy- diagnostic
115
Where in the bowel are diverticula, commonly?
Sigmoid colon
116
Where on the circumference of the bowel are diverticula often positioned?
Between the mesenteric and antimesenteric taenia coli
117
Pathogenesis of diverticula?
Increased intra luminal pressure + points of relative weakness in the bowel wall
118
short bowel syndrome is a complication of what?
IBD
119
Apthoid and fissuring ulcers are in crohns or UC?
Crohns
120
Strictures are common in crohn's or UC?
Crohns
121
No spontaneous fistulae are present in crohns or UC?
UC
122
Crypt abscesses are common and crypt distortion is severe in crohn's or UC?
UC
123
Granulomas present in crohns or UC?
Crohns
124
7 extra-intestinal manifestations of IBD?
```
Hepatic
Skeletal
Mucocutaneous
Renal
Heamatological
Systemic
```
125
Which hyperplastic polyps may give rise to microsatellite unstable carcinoma?
Large, right sided
126
What benign polyp is a CRC precursor?
Adenoma
127
2 genetic traits that increase susceptibility to colorectal cancer
FAP
| HNPCC
128
FAP is due to a mutation in what?
APC tumour supressor gene
129
HNPCC also increases risk of which cancers?
Endometrial, ovarian, gastric, small bowel, urinary tract, biliary tract
130
HNPCC what mutation?
DNA mismatch repair genes
131
Pathogens that cause intra abdominal infection
```
Pseudomonas ssp
Clostridium spp
Bacteriodes ssp
Enterococcus ssp
Enterobacteraceae
Prevotella ssp
```
132
Rx of intra abdominal infection of intestinal source
Cefuroxime and metronidazole if under 65
Piperacillin/tazobactam if over 65
Then depends on culture results
133
Pain in shoulder
Hiccoughs
Apparent hepatomegaly
Pleural effusion/lung collapse
Subphrenic abscess
134
Pathogens that cause gastroenteritis?
```
Bacterial:
Bacillus cereus
Vibrio cholerae
Salmonella
C diff
Staph aureus
E coli
Shigella
Campylobacter
```
Viral:
Norovirus and Rotavirus
Parasitic:
Entamoeba histolytica
Cryptosporidium
Giardia lamblia
Non-infective
135
How might you catch cryptosporidium gastroenteritis?
Waterborne- e.g. swimming pool or salad washed in infected water
136
Of the bacterial causes of gastroenteritis, which produce enterotoxins and which act by adherence?
```
Enterotoxins:
Bacillus cereus
Vibrio cholerae
C. diff and C. perfringens
Staph aureus
E. coli
```
```
Adherence:
Shigella sonnei/flexneri
E. Coli
Campylobacter jejuni
Salmonella
```
137
EHEC/VTEC form of E.coli =?
Enterohaemorrhagic
| Colitis/HUS
138
ETEC form of E.coli =?
Enterotoxigenic
| Traveller's diarrhoea
139
EAggEC
Enteroaggregative
| Traveller's diarrhoea
140
EIEC form of E.coli =?
Enteroinvasive dystentry like
141
EPEC form of E.coli =?
Enteropathogenic
| Childhood
142
E Coli 0157 releases what virulence factor?
Shiga toxin
143
Shiga toxin mechanism?
Like cholera
144
Salmonella mechanism?
It is the bacteria itself not the toxin.
145
Typhoidal strains of salmonella do what?
Affect cells outside of GI tract and spread
146
Rx gastroenteritis?
Supportive
Avoid abx unless very young/old, campylobacter infection or it has invaded (positive blood cultures)
Abd may make e coli hus worse or increase the duration of salmonella carriage
147
Type of colitis in abx associated diarrhoea?
Pseudomembranous
148
rx c diff
metronidazole or vancomycin
| faecal transplant
149
8 causes aseptic meningitis
```
Viruses
Partially treated bacterial
Listeria
TB
syphilis
Malignancy
autoimmune
drugs
```
150
2 main causes of viral meningitis?
```
Enteroviruses
Herpes virus (HSV2, VZV)
```
151
2 extra signs of viral meningitis seen in neonates/infants?
Nuchal rigidity
| Bluging anterior fontanelle
152
What is Kernig's sign?
Tight hamstrings
153
Brudzinski's sign?
Flexing neck causes hips and knees to flex
154
What investigation must you do ASAP in viral meningitis (and what would contraindicate it?) What is the gold standard test?
Lumbar puncture unless raised ICP (do a CT to check)
| Gold standard is viral PCR
155
difference in CSF between bacterial and viral meningitis?
White cell count- B very high, V high
Predominant WBC- B neutrophils, V lymphocytes
Protein- B high or very high, V normal or high
Glucose- B low, V normal
156
Rx viral meningitis
If risk of bacterial then abx
| Otherwise supportive
157
What is Mollaret's meningitis?
Recurrent aseptic, normally HSV2
158
Viral meningitis + fever, lymphadenopathy, pharyngitis, rash =?
HIV meningitis
159
Viral encephalitis causes that are different to meningitis?
HSV1, rubella, rabies, acute disseminated encephalomyopathy
160
Rx viral encephalitis
High dose aciclovir
161
What is ADEM?
Immune mediated CNS demyelination
162
Difference between onset of encephalitis and brain abscess?
Encephalitis is acute
163
3 causes of bacterial meninigitis?
Neisseria meningitidis
Haemophilus influenza
Strep pneumoniae
164
Rx of bacterial meningitis?
Ceftraixone and cefotaxime
Rifampicin to contacts
+steroids for s. pneumoniae
165
3 causes neonatal meningitis
Group B strep
E coli
Listeria monocytogenes
166
Rx neonatal meningitis
Cefotaxime
| Ampicillin and gentamicin
167
Marfan syndrome is associated with what valvular heart disease?
Aortic regurg
168
Cyanosis in R to L or L to R shunts?
R to L
169
Downs syndrome is associated with which heart defect?
AVSD
170
Which 3 heart conditions require a shunt to survive?
Transposition great arteries
Tricuspid atresia
Total anomalous pulmonary venous connection
171
Coarctation of the aorta is associated with what genetic defect?
Turner's syndrome
172
Murmur in aortic regurg
Diastolic
173
Murmur in mitral stenosis
Opening snap and diastolic
174
Murmur in aortic stenosis
Ejection systolic
175
Murmur in mitral regurg
Pansystolic
176
3 types endocarditis
Infective
Non bacterial thrombotic
Endocarditis of SLE (Libman-Sacks Disease)
177
What pathogen found to be causing infective endocarditis should prompt investigation for bowel cancer?
Strep bovis
178
Signs of infective endocarditis
FROM JANE
| fever, roth spots, osler's nodes, murmur, janeway lesions, anaemia, nail haemorrhage, emboli
179
In whom does non-bacterial thrombotic endocarditis occur?
Debiliated patients in hypercoagulable state
180
Which valves affected in Libman-Sacks endocarditis?
Mitral and tricuspid (atrioventricular valves)
181
Aschoff bodies are found in what?
Rheumatic fever
182
3 pathogens that cause infective endocarditis
Strep viridans
Staph aureus
Strep bovis
183
5 types acute pericarditis and their causes
1. purulent (infection)
2. serous (adjacent inflammation)
3. Caseous (TB or fungal)
4. Fibrinous/serofibrinous (Dressler's syndrome post MI)
5. Haemorrhagic (trauma, neoplasm, surgery, infection)
184
3 types chronic pericarditis
- Adhesive
- Adhesive mediastino pericarditis (follows pericarditis caused by infection, surgery, radiation)
- Constrictive
185
4 classes of cardiomyopathies
- Dilated
- Restrictive
- Hypertrophic
- Arrythmogenic right ventricular
186
4 causes of dilated cardiomyopathy
Genetic, myocarditis, alcohol/toxin, autoimmune
187
Cause hypertrophic cardiomyopathy
Genetic
188
Sign of hypertrophic cardiomyopathy on examination?
Systolic ejection murmur
189
Restrictive cardiomyopathies are either idiopathic or secondary to which 5 things?
```
Amyloidosis
Fibrosis
Inborn errors of metabolism
Sarcoidosis
Tumours
```
190
Myocarditis is caused by which two infections?
Coxsackie virus A+B
| Chagas disease
191
Giant cell arteritis is a type of? It involves what sort of inflammation?
Vasculitis. Granulomatous.
192
What type of cells are seen in giant cell arteritis?
Multi-nucleated giant cells
193
Rx giant cell arteritis?
Corticosteroid, anti TNF
194
6 types aneurysm
```
Atherosclerotic
Dissecting
False
Berry
Micro and syphilitic
Mycotic
```
195
Which type of aneurysm is associated with subacute bacterial endocarditis?
Mycotic
196
3 signs nephrotic syndrome
Oedema
Proteinurea
Hypoalbuminaemia
197
5 signs acute nephritis
```
Oedema
Haematuria
Proteinuria
HTN
AKI
```
198
What is nephrotic syndrome?
Glomerular damage
199
5 causes nephrotic syndrome
```
Membranous nephropathy
Diabetes
FSGS
minimal change disease
SLE
```
200
6 causes acute nephritis?
```
IgA nephropathy
Post infective
HUS
Vasculitis
Henoch schonlein purpura
SLE
```
201
Myeloma can have what effect on the kidneys?
Immunoglobulins in the tubules
202
Major cause of CKI and 3 other causes
DIABETES
Malformation
Reflux nephropathy
Glomerulonephritis
203
Haematuria + ear and eye problems = ?
Alport type hereditary nephrophathy
204
4 caues proteinuria
SLE
FSGS
Diabetes
Henoch schonlein purpura
205
4 possible composition of renal stones?
Calcium
Struvite (Mg NH4+ P)
Urate
Cystine
206
Cause of struvite stones
Urease producing bacterial infection (urease converts urea to ammonia)
207
Staghorn caliculi are a sign of which type of stone?
Struvite
208
What is the gold standard investigation for renal stones?
Non-contrast CT
209
Majority of renal cancers are what tyoe?
Clear cell
210
Renal cancer is associated with which genetic disease?
Von Hippel-Lindau syndrome
211
Foamy cell renal tumours are likely to be
Papillary tumours
212
True or false renal cell carcinoma responds well to chemo?
False
213
Urothelial cell carcinoma is found in the?
Bladder
214
Prostate carcinomas normally arise from which zone of the prostate?
Peripheral
215
Seminoma or teratoma is after puberty?
Teratoma
216
What serum substrates are increased in seminoma?
PLAP and hCG
217
True or false teratomas do not secrete tumour markers
True
218
Pain and swelling in the epididymis following a vasectomy
Sperm granuloma
219
Inflammatory and myofibroblastic mass in the testis of unknown aetiology
Myofibroplastic pseudotumour of testis
220
Swollen, tender testis with flu-like illness and a granulomatous mass
Idiopathic granulomatous orchitis
221
Painless scrotal swelling
| Caseating, granulomatous inflammation, fibrosis
Tuberculous orchitis
222
Infarcted seminiferous tubules
Epididymoorchitis
223
Yellow/brown nodules and histiocytes in testis
Malakoplakia
224
Rx pyelonephritis
Cefuroxime, ciprofloxacin (or pip and tazo if over 65)
225
3 common pathogens that cause UTIs
```
E coli
Staph saprophyticus (GP)
Enterococcus ssp (hospital)
```
226
Rx cystitis
Nitrofurantoin
Pivmecillinam
Trimethoprim
Fosfomycin
227
What is urethral syndrome?
Symptoms UTI w/o infection
228
When would you treat asymptomatic bacteriuria
Pregnancy
Infant
Prior to urological procedures
229
Thalassaemia leads to what type of anaemia?
Microcytic hypochromic
230
Megaloblastic macrocytic anaemia can be caused by what?
B12 or folate deficiency
231
Pathogenesis of acute haemolytic reaction (ABO incompatibility)
Release of free Hb
232
Fatigue, jaundice and fever 3-14 days post blood transfusion =? Due to what?
Delayed haemolytic reaction
| Due to RBC antigens other than ABO
233
What does coomb's test detect?
IgG antibodies
234
True or false febrile non haemolytic transfusion reaction is life threatening?
False
235
Prothrombin time measures which pathway?
Extrinsic
236
Time from thrombin to fibrin is measured by what test?
Thrombin clotting time
237
4 in vivo anticoagulants
protein c
protein s
anti-thrombin III
fibrinolytic system
238
What does the fibrinolytic system involve?
Plasmin an ezyme that causes fibrin breakdown
Plasminogen cleaved by plasminogen tissue activator to plasmin
Alpha 2 anti plasmin mops up excess plasmin in plasma
239
Coagulation problems that are platelet/vessel wall defects give rise to what sort of symptoms?
Mucosal and skin bleeding e.g. heavy periods, nose bleeds
240
Coagulation problems that are coagulation defects give rise to what sort of symptoms?
Deep muscular and joint beds
241
VW disease affects what part of clotting?
Aggregation
242
True or false haemophilias VIII and IX have different presentations to each other
False
243
Which clotting test is abnormal in VWD and haemophilia?
APTT
244
What is a treatment of VWD that stimulates VWF release?
DDAVP a vasopressin/ADH analogue
245
Patients with liver disease invariably have what blood problem?
Impaired haemostasis
246
4 features of impaired haemostasis in liver disease?
Low and dysfunctional platelets
Reduced synthesis of all coagulation factors except VIII
Fibrin polymerisation is delayed
Excessive plasmin activity
247
Massive transfusion can have what 2 problems?
DIC
| Dilutional depletion of platelets and coagulation factors
248
What chromosomes are the different Hbs found on?
Alpha- 16
| Gamma, delta and beta- 11
249
4 acute complications of sickle cell disease
Vaso-occulsive crisis
Septicaemia
Aplastic crisis
Sequestration crisis
250
Chronic complications of sickle cell disease
Hyposplenism
Renal disease
Avascular necrosis
Leg ulcers, osteomyelitis, gall stones, retinopathy, cardiac, respiratory
251
True or false sickle cell is an inflammatory condition in some ways
true
252
Alpha thalassaemia results in which 2 types of Hb?
Hb Barts and HbH
253
True or false alpha thalassaemia trait is clinically significant
False
254
Thalassaemia beta major has what 6 features?
Severe aneamia
Skeletal abnormalities and stunted growth
Increased BM activity so increased iron absorption
Splenomegaly (RBC pooling)
Abnormal dentition
Frontal bossing
255
Rx thalassaemia beta major?
Transfusion to maintain Hb and suppress rbc production
| And chelation with desferrioxamine to get rid of excess iron.
256
What do most thalassaemia maj beta patients die of?
Heart disease
257
Blood differences in pregnancy?
```
MCV +
Prothrombotic
Fe and folic acid used
Neutrophilia
Haemodilution
Reduced platelets
```
258
3 main areas of chronic myeloproliferative disorders
Polycythaemia vera
Essential thrombocytosis
Idiopathic myelofibrosis
259
Symptoms polycythaemia vera
Redness, itching, gout, splenomegaly
260
Some causes of secondary polycythaemia?
```
Chronic lung disease
R-L shunts
Smoking
altitude
renal disease
EPO producing tumours
drugs
congenital
```
261
Rx polycythaemia
Aspirin
| Venesection
262
What mutations are associated with thrombocytosis and myelofibrosis?
JAK2 and CALR
263
Thrombocytosis is increase in what?
Megakaryocytes and platelets
264
What are the two types of thrombocytosis?
Primary essential
| Reactive
265
What is myelofibrosis?
BM fibrosis
266
What are the symptoms of myelofibrosis?
Pancytopenia
Fever, weight loss, night sweats
Splenomegaly
267
Chronic myeloid leukaemia symptoms
```
Leucocytosis ++
Leukoerythroblastic blood picture
Anaemia
Splenomegaly
Venous occulsion
Gout
```
268
What chromosome is associated with CML?
Philadelphia (9 and 22 swap= cell proliferation = active tyrosine kinase. Inhibited by imatinib)
269
Name the two light chains of Igs
Kappa and lambda
270
What 2 states precede myeloma?
MGUS, asymptomatic myeloma
271
What are the diagnosis criteria for myeloma?
more than 10% BM plasma cells neoplastic
OR
Lump of plasma cells found
AND
One of more of either CRAB or MDEs
hyperCalcaemia, Renal insufficiency, Anaemia, Bone lesoins
272
What can myeloma go on to become?
Plasma cell leukaemia
273
AKI and myeloma is what?
A medical emergency! Give steroids
274
Non hodgkins lymphoma is what?
Too many follicles in lymph nodes, lump on neck
275
Hodgkins lymphoma has what cells? Linked to what virus?
Hodgkin Reed-Sternberg cells with infiltrate of non-malignant inflammatory cells.
EBV
