haematology/oncology Flashcards
1
Q
what is pernicious anaemia? (pathology)
A
B12 (from food) absorbed with intrinsic factor (from parietal cells in stomach) absorbed together in terminal ilium
In PA - IgA attacks parietal cells preventing secretion of intrinsic factor so no absorption of b12 - macrocytic anaemia
2
Q
how does Crohn’s disease cause macrocytic anaemia?
A
Crohn’s has a predilection for the terminal ilium
inflammation/resection of terminal ilium -> inability to absorb IF+B12
so get macrocytic (+megaloblastic) anaemia
3
Q
how does gastric bypass cause macrocytic anaemia?
A
bypass stomach where parietal cells which secrete IF are - so B12+IF cannot be absorbed by terminal ilium - macrocytic anaemia
4
Q
if impaired absorption of b12 - how should b12 be replaced?
A
IM
5
Q
apart from macrocytic anaemia, why is b12 deficiency a problem?
A
neuro sx - DCMLS (dorsal coloumn medial lemniscus system) - sensory pathway responsible for: - 2 pt discrimination - proprioception - vibratory sense IRREVERSIBLE
6
Q
what other disease causes similar symptoms (neuro) to b12 deficiency?
A
syphilis
7
Q
what does Schilling’s test determine?
A
whether b12 deficiency is nutritional or from impaired absorption (usually can get from Hx)
8
Q
how does Schilling’s test work?
A
give IM B12 - saturate B12 in liver, then give PO B12
- > check urine: if + for b12 they were able to absorb it so their previous problem was nutritional deficiency
- > if urine - for B12 - couldn’t absorb PO - absorption problem
9
Q
non-megaloblastic causes of macrocytic anaemia:
A
liver cirrhosis
ETOH
drugs - chemo (5FU)/Ara-C/HAART (AZT)
metabolic conditions
10
Q
how do you differentiate between the megaloblastic and non-megaloblastic macrocytic anaemias?
A
blood smear - megaloblastic if hypersegmented neutrophils - >5 nuclear segments
11
Q
how to differentiate between megaloblastic macrocytic anaemias if b12 and folate levels both on lower side of normal?
A
MMA (methylmalonic acid) test - raised if b12 deficiency, normal if folate deficiency
12
Q
what are the 4 types of microcytic anaemia?
A
IDA
ACID
Thalassaemia
Sideroblastic
13
Q
who gets IDA (generally which type of patients?
A
slow bleeds - subacute haemorrhages
male, >50, colon ca, +FOBC
female >20 menorrhagia
14
Q
what do iron studies show in IDA (remember the silo metaphor from Dustyn):
A
Fe low
Ferritin low
TIBC (transferrin) high
transferrin saturation level reduced
15
Q
what do iron studies show in ACID and why? (silo metaphor)
A
Fe low
Ferritin high
TIBC low
body’s cells can survive for some time without iron, but bacteria need iron to survive, so as an acute response, body cuts off Fe supply. In chronic inflammation, same response happens - pathology of ACID
16
Q
what type of pt gets ACID?
A
chronic inflammatory disease AI
eg lupus or RA
usually asx anaemia
17
Q
tx for ACID?
A
usually nothing
in severe cases EPO
but rather control disease
giving iron will do nothing
18
Q
pathology of thalassaemia?
A
globin problem
minor - asx
major - transfusion-dependent (monthly)
19
Q
what are iron studies like in thalassaemia?
A
normal - problem with globin, not haem
20
Q
if normal iron but macrocytic anaemia, which test do you do if suspect thalassaemia?
A
HgB electrophoresis will show b-thalassaemia
if normal - a-thalassaemia
also high bili
21
Q
due to blood transfusions containing a years worth of iron in them, what cx and f/u tx needed in transfusion dependent thalassaemia pts?
A
iron overload
de-furox-amine
22
Q
pathology of sideroblastic anaemia:
A
iron gets stick in mitochondria
sideroblastic RBCs are trying to make regular RBCs
bone marrow throws more iron at them
the sideroblastic cells themselves are loaded with iron but can’t use it
23
Q
what iron studies show in sideroblastic anaemia:
what test confirms
A
Fe high
others normal/irrelevant
biopsy shows a ring sideroblast
24
Q
reversible causes sideroblastic anaemia:
A
drugs
etoh
lead
25
irreversible causes sideroblastic anaemia:
```
B6 deficiency (cannot be corrected by giving B6)
Myelodysplastic syndromes
```
26
what is a sideroblast?
erythroblast with ferritin granules in cytoplasm
27
normal adult Hb contains:
2 a chains 2 b globin chains
28
HbA2 contains:
2 a chains 2 delta globin chains
29
what type of anaemia is hypothyroidism associated with?
macrocytic (non-megaloblastic)
30
folate also known as which b vitamin?
b9
31
a patients FBC shows normocytic anaemia, what other tests are you interested in to investigate if this is haemolytic?
```
raised reticulocyte countm
LDH
Haptoglobin
Bilirubin
blood smear
```
32
what will haemolysis bloods look like?
LDH raised
Hp down
Bili raised
smear - diff diseases have characteristic findings
33
why do you get a smear in haemolysis ix?
if LDH, Hp, bili all normal
| smear can show cancer - evidence of marrow malignancy
34
what does blood smear show in sickle cell anaemia?
sickled cells
35
what does smear show in G6PD?
bite cells
| heinz bodies
36
what haemolytic condition is likely if smear shows spherocytes? (2)
hereditary spherocytosis
| AI haemolytic anaemia
37
pathology of sickle cell?
cells sickle due to HbSS - AR
prediliction in african communitites
2 bad genes with sickling cells
38
how do sickle cell pts present?
generally a paeds condition
| in adults - vaso-occlusive crisis - pain!
39
pathophysiology of vaso occlusive crisis?
normally donut shaped RBC can squeeze through capillaries
under periods of acidosis, hypoxaemia, dehydration the cells sickle
cannot fit through capillary ->
1) back pressure causes haemolysis
2) capillary obstruction - ischemic tissue - pain
40
define haemolysis:
rupturing of RBC and the spilling of their cytoplasm into the surrounding blood plasma
lifespan of RBC <100 days (normally 120)
41
examples of vasooclusion manifesting as an emergency:
acute chest - MI, CHF exacerbation
acute brain - CVA
priapism (males) - painful and persistent erection
42
ix sickle cell crisis in adults:
1) smear - sickle cells
| best - Hb electrophoresis (once, probably done as child)
43
tx sickle cell crisis: (non-emergent)
chronic: Hydroxyuria - induces formation of HbF (if repeated attacks)
(regular transfusions sometimes required)
acute: IV fluids, o2, pain control
then treat what induced crisis eg infection
44
what makes up HbF?
a2Y2 (2 gamma chains)
45
tx sickle cell crisis emergency:
exchange transfusion
46
pathology of G6PD deficiency?
in cells rich with G6PD, oxidative stress comes on and is repelled.
if G6PD deficient, oxidant stress comes thru and destroys the RBC
47
who gets G6PD?
2 variants:
1) african male who is exposed to medication oxidant stress (Dapsone, TMP-SMX, nitrofurantoin)
2) fava beans Greek
X-linked AR
48
how does G6PD present in patients?
african male exposed to high risk medications and turns yellow, Hb low, bili up
49
G6PD deficiency ix:
1st - smear - heinz bodies, bite cells
| best - G6PD level 6-12/52 after attack
50
G6PD deficiency tx:
supportive during attack
| avoid the stress
51
Hereditary spherocytosis pathology:
```
RBC membrane protein problems:
spectrin
ankrin
pallidin
AD!
spherical RBCs cannot fit thru spleen, haemolysed by spleen
no spec hx of this condition - lab dx
```
52
HS ix:
1) smear - spherocytes
| best - EMA binding
53
HS tx:
splenectomy
| f/u folate, fe
54
AIHA ix:
Coomb's test
55
Coomb's test +ve for IgM: what does this indicate?
| include mx
```
COLD AIHA
associated with:
mycoplasma
mononucleosis (EBV)
(causes haemolytic anaemia in extremities when it's cold)
AVOID COLD
```
56
Coomb's test +ve for IgG: what does this indicate?
| include mx
```
WARM AIHA
associated with:
AI diseases
cancer - CLL
mx:
fix underlying, steroids, rituximab, splenectomy
```
57
what is paroxysmal nocturnal haemoglobinuria (PNH)?
deficiency of PIG-A gene
normally complement comes around tries to fix to a RBC, cells with PIG-A repel it no problem
in PNH: complement will fix, complex forms, cell lysed
58
who gets PNH:
paroxysmal - theoretical increased risk - threshold not always reached
nocturnal - breath less at night - increased risk of hypoxaemia, acidosis
Hburia - haemolysis + bili high so appears as tho passing blood as urine is dark
59
PNH dx:
flow cytometry - monoclonal expansion of CD55-ve cells
60
PNH tx:
usually supportive
| in refractory/severe cases: eculizumab
61
blood cells is classical of microangiopathic haemolytic anaemia on blood film?
schistocyte
62
what is Richter's transformation?
patient with CLL who's cancer transforms into high grade Non-Hodgkin's Lymphoma and makes the patient acutely unwell
63
You can use the mnemonic “ALL CeLLmates have CoMmon AMbitions” to remember the progressive ages of the different leukaemia from 45-75 in steps of 10 years. Remember that ALL (the first in the mnemonic) most commonly affects children under 5 years.:
Under 5 and over 45 – acute lymphoblastic leukaemia (ALL)
Over 55 – chronic lymphocytic leukaemia (CeLLmates)
Over 65 – chronic myeloid leukaemia (CoMmon)
Over 75 – acute myeloid leukaemia (AMbitions)
64
treatment for beta-thalassaemia patients?
life-long transfusions
65
which leukaemia is associated with Downs syndrome?
ALL
66
which leukaemia is associated with the Philadelphia chromosome (t9:22)?
CML
| has 3 phases including a 5 year asx chronic phase
67
which leukaemia associated with Auer rods?
AML
68
type of genetic transmission in thalassaemia?
alpha and beta both autosomal recessive
69
raised WCC, raised lymphocyte count, and the presence of smear cells and small/medium-sized lymphocytes on the blood film. indicates?
CLL
| smear/smudge cells
70
why do CLL patients get recurrent infetions?
due to the cx of hypogammaglobulinaemia
71
how far in advance of an operation should a woman stop taking COCP?
4 weeks before
72
Platelet transfusion for thrombocytopenia before surgery or an invasive procedure. Aim for plt levels of:
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site or CNS bleed
73
should compression stockings be offered to pts with confirmed DVT?
no
74
really high lymphocytosis in elderly patient suggests?
CLL
75
if young child comes in with features that concern you about leukaemia, when should FBC be done?
<48h
76
2 important side effects of tamoxifen?
VTE risk increased
| risk of endometrial cancer
77
man on warfarin in RTC comes to ED with reduced GCS, bloods ok but CT shows ICH. given vit K - what other blood products should he get?
prothrombin complex concentrate given for the emergency reversal of anticoagulation in patients with severe bleeding or a head injury
78
in Hodgkin Lymphoma, B symptoms indicate a poor prognosis, name 3 b symptoms:
night sweats
fever >38
weight loss >10% over last 6 months
79
which blood products is most likely to cause an iatrogenic septicaemia with a Gram-positive organism?
platelets - stored at room temperature
80
which blood products is most likely to cause an iatrogenic septicaemia with a Gram-negative organism?
packed red cells
81
myelodysplasia may progress to which condition?
acute myeloid leukaemia
82
Acute haemolytic transfusion reaction should be treated:
stop transfusion and generous fluid resus
83
pts over 60 presenting with IDA need which ix?
colonoscopy
84
New B-symptoms in CLL ->
richter's transformation
85
dx of richter's transformation confirmed by?
lymph node biopsy
86
sudden anaemia and low reticulocyte count in sickle cell pt indicates?
parvovirus
87
high reticulocyte count in sickle cell pt indicates?
acute sequestration or acute haemolysis
88
A grossly elevated APTT may be caused by (3):
heparin therapy
haemophillia
antiphospholipid syndrome
89
pathology of haemophillia B?
lack of factor IX
| 10% cases of haemophilia
90
pathology of haemophillia A?
lack of factor VIII
| 90% of haemophillia cases
91
which part of the skeleton is used to make the blood products in adults?
axial skeleton
92
difference between serum and plasma?
plasma contains plasma proteins as well
| serum purely contains electrolytes and nutrients
93
alcohol and liver disease commonly cause which type of anaemia?
macrocytic and thromboytopaenia
94
```
plts down
PT up
fibrinogen down
D-dimer up
dx?
```
DIC
95
dic mx:
major (unstable) haemorrhage protocol
stop bleeding
FFP/cryoprecipitate
platelet replacement
96
what could cause both IDA and b12 deficiency anaemia?
coeliac disease
97
definition cancer:
uncontrolled, abnormal cell proliferation
98
6 hallmarks of cancer:
```
uncontrolled proliferation
evade growth suppression
evade apoptosis
angiogenesis
activate invasion and metastasis
replicative immortality
```
99
what does the grade of a cancer refer to?
resemblance to original tissue
100
generally what is used to ix spread of Cancer?
CT CAP
101
define paraneoplastic syndrome:
non-metastatic manifestations of malignancy
| due to immune response, Ab cross-reaction, physiological reaction
102
what can tumour markers be used for:
screening, prognosis, tx response, recurrence
103
62M to ED with SOB. Anaemic, thrombocytosis. jugular vein distended, facial engorgement.
CXR: mediastinum widening, R hilar lesion. dx?
SVCO
| can also get blurred vision
104
3 causes of SVCO?
malignancy - 60-80% cases
thrombosis
infection
105
if malignant cause SVCO - which cancers?
LC - NSCLC
| No LC - lymphoma, germ cell, thymoma
106
why may the presentation be less severe if slow progression of SVCO?
slow progression enables time for collateral formation which decreases the venous pressure
107
what is the name of the clinical sign you may see in SVCO? describe
Pemberton's sign
cyanosis and facial oedema when elevation of both arms to above head
raised ICP
108
what is trosier's sign ?
aka Virchow's node
enlarged left subclavian LN
intra-abdominal ca
109
what is sister-mary-joseph's sign?
umbilical nodule
| sign of metastasis usually from a peritoneal adenocarcinoma spreading
110
Ix for ?SVCO?
initially CXR
| better ix - CT chest - dx, px
111
what supportive tx for SVCO?
raise head, corticosteroid (dex), diuretics, o2
112
life-threatening SVCO - tx?
urgent stent +/- thrombolysis
113
local cancer extension can be characterised by lumen or vessels changes -
lumen - GI obstr, biliary obstr, hydronephrosis, fistulae
| vessels - tumour associated thrombus, acute bleeding
114
71F ED via GP with worsening back pain, decreased balance 3/52. 10 years ago br ca mastectomy. OE: bilat crackles. CXR indicate mets bilat. reduced mobility. altered sensation T12. anaemic. WCC up. Plts down. AKI. egfr 22. raised K, lactate.
ideal initial investigation????
MRI whole spine <24h
MSCC is an emergency
115
where is MSCC commonly found?
Thoracic spine region (red flag la)
| then L, then C
116
how to differentiate where MSCC is likely to be based on clinical sx (3):
if SCC - UMN sx
If conus medulloris - mixed picture
if CES - LMN sx
117
5 non haematological cancers which commonly metastasise to bone:
prostate, lung, renal, thyroid, breast
118
commonest presenting complaint in MSCC?
back pain
119
name 4 manifestations other than MSCC of metastasis of cancer:
seizures - CNS
ascites - abdo
jaundice - liver
pain/# - bone
120
54M confused, SOB, CP over past few months. CXR total white out left side. Tracheal deviated L. ABG - met acidosis. lactate 3.1. bloods - anaemic, wcc+np up, plts up. Ca up, Mg down. dx?
hypercalcaemia of malignancy
121
what type of condition is hypercalcaemia of malignancy?
paraneoplastic
122
what is the mechanism of hypercalcaemia of malignancy?
PTHrP release (1)
osteolytic mets in breast ca
calcitriol secretion - HL
123
how to distinguish between hypercalcaemia of malignancy and hypercalcaemia due to primary hyperparathyroidism ?
HoM - PTH down. PHP - PTH up/normal
HOM - phosphate up/N/down. PHP - phosphate down.
ALP normal/up in both
124
tx hypercalcaemia of malignancy? (emergent)
IV NaCl aim for 125ml/h - 8h bags b2b
2. bisphosphonates - take 2-4/7 to act
address underlying malig
4. if resistant - denosumab
125
how is malignant hypercalcaemia classified?
mild <3 - PO fluids
mod 3-3.5
severe >3.5
mod, sev - IVI+/-bisphosphonates
126
common cancer that causes paraneoplastic syndromes:
SCLC
127
On examination, she has focal tenderness of the L1 spinal process. She has a normal neurological examination.
Ix?
MRI whole spine < 1 week
| looking for bony mets and pathological #
128
what form part of the diagnostic work-up in a woman found to have an abdominal malignancy of unknown primary?
ca 125 - ov ca
129
which chemo drugs can cause cardiomyopathy?
Anthracyclines (e.g. doxorubicin)
130
sfx cyclophosphamide?
Haemorrhagic cystitis, myelosuppression, transitional cell carcinoma
131
bleomycin sfx?
lung fibrosis
132
BRCA2 associated with which cancers in men?
breast and prostate
133
commonest lung ca in non-smokers?
adenocarcinoma
| peripheral lesion
134
A patient with lung cancer has a Positron Emission Tomography (PET) scan to evaluate possible metastatic disease. What does this type of scan demonstrate?
glucose uptake
135
recently been started on an immune checkpoint inhibitor to treat her non-small cell lung cancer. example of this class of cancer drug?
nivolumab
136
cisplatin sfx?
hypomagnesia
| Ototoxicity, peripheral neuropathy,
137
MTX sfx?
Myelosuppression, liver fibrosis and oral mucositis
138
5-FU sfx?
Myelosuppression, mucositis, dermatitis
139
hydroxyurea sfx:
Myelosuppression
140
spinal mets ix?
MRI spine
141
Calcitonin is a tumour marker in :
medullary thyroid ca
142
ca19-9:
pancreatic ca
143
ca15-3:
breast ca
144
AFP: marker for
Hepatocellular carcinoma, teratoma (testicular)
145
what is used to suppress nausea and vomiting with intracranial tumours?
dex
146
An 80-year-old man presents to the emergency department with back pain. He has no documented past medical history. An x-ray spine shows vertebral wedge compression fractures and focal sclerotic bony lesions.
dx?
metastatic prostate cancer
147
which HPV are non-carcinogenic and associated with genital warts?
6, 11
148
which HPV carcinogenic?
16, 18, 33
149
Iron defiency anaemia vs. anaemia of chronic disease: how to distinguish?
TIBC is high in IDA vs low/normal in ACID
150
A 48-year-old female who has just completed a course of chemotherapy complains of difficulty using her hands associated with 'pins and needles'. She has also experienced urinary hesitancy. Which cytotoxic drug is most likely to be responsible?
vincristine
periph neuropathy
bladder atony->hesitancy
151
Polycythaemia rubra vera - around 5-15% progress to?
myelofibrosis or AML
| jak2 mutation
152
Platelet transfusion is appropriate for patients with a platelet count???
under 30 x10>9 and clinically significant bleeding
153
35-F Ten days ago she had a tooth extraction but unfortunately bled profusely post-procedure, necessitating transfer to the local maxillofacial unit for suturing. She reports no history of bleeding previously and is otherwise well. What is the most likely diagnosis?
von-willibrand disease
commonest inherited clotting disorder
autosomal dominant
mild elevated aptt
154
how long DVT mx?
DOAC - 3/12 if provoked
| 6/12 unprovoked
155
The transfusion threshold for patients with ACS?
80g/L
156
transfusion threshold for general patients?
70g/L
157
The isolated thrombocytopenia in a well patient points to a diagnosis of?
ITP
158
first line mx in ITP?
po pred
159
In a non-urgent scenario, a unit of RBC is usually transfused over ?
90-120mins
160
what is the most common inherited thrombophilia?
factor V leiden
| activated protein C resistance -> slow incactivation of Factor V
161
Hodgkin's lymphoma - best prognosis =
lymphocyte predominant
female
<45 at dx
162
reed sternberg cells (mirror image nuclei)?
Hodgkin's lymphoma
163
raised LDH makes you think of?
Non-Hodgkin's lymphoma
164
Cryoprecipitate contains?
factor VIII, fibrinogen, von willibrand factor, factor XIII
165
'rouleaux formation' is a stacking of red blood cells seen in a blood film. It is characteristic of ?
myeloma
166
The most important symptoms/complications of myeloma can be remembered by the acronym CRAB:
Calcium raised
Renal failure
Anaemia (pancytopaenia)
Bone pain
167
bence-jones protein in urine in which condition?
myeloma
168
If a 2-level DVT Wells score is ≥ 2 points then ?
arrange a proximal leg vein USS < 4h
| if scan will take >4h LMWH/doac cover
169
the recommended treatment for post-thrombotic syndrome?
graduated compression stockings
170
A decrease in haptoglobin levels can be seen in?
intravascular haemolysis
171
Low haptoglobin levels are seen in intravascular haemolysis, as well as raised urine haemoglobin and haemosiderin. Reticulocytes are raised causing slightly increased MCV. Examples of causes of intravascular haemolysis include?
G6PD
AIHA
HELLP syndrome
172
Tranexamic acid is given how?
IV bolus then slow infusion
173
mechanism of COPD -> polycythaemia?
Impaired oxygen exchange in the lungs can result in a low PaO2 which results in stimulation of EPO release from the kidneys. EPO stimulates erythropoiesis and increases red cell mass, thereby resulting in polycythaemia. The low SpO2 is highly suggestive of a hypoxic driven polycythaemia
174
Disproportionate microcytic anaemia -?
B-thalassaemia trait
| raised HbA2
175
Haemarthrosis without trauma is typically a feature of?
haemophilia A/B
176
differentiating between haemophilia and von willibrand disease off bloods?
same bloods - normal PT, raised APTT (tho grossly is Haemophilia). but VWD has longer bleeding time. bleeding time in haemophilia normal
177
Vitamin K deficiency bloods?
increased PT, APTT, normal bleeding time
178
If investigating a suspected DVT, and either the D-dimer or scan cannot be done within 4 hours?
start a doac
179
complicated revision of a total hip replacement, an 80-year-old lady receives two units of packed red cells. BG HF currently takes bisoprolol, ramipril and furosemide. Which should be prescribed between the units?
stat dose furosemide
180
dabigatran anticoagulation reversal?
idarucizumab
181
8/12girl ->ED. Her parents are concerned about her seemingly painful swollen hands and feet. Sudden onset three hours ago. Bilat swelling of the child's hands and feet and slight erythema of the overlying skin. There seemed to be marked tenderness over all extremities evidenced by her crying. Slight fever but nothing else remarkable. What is the most likely diagnosis?
sickle cell crisis
| can commonly present with hand-foot syndrome
182
c-myc gene translocation - which lymphoma?
burkitt's lymphoma
| 'starry sky' appearance microscopy
183
Thymomas are commonly associated with?
myasthenia gravis
184
A 4-year-old girl with sickle cell anaemia presents with abdominal pain. On examination, she is noted to have splenomegaly and is clinically anaemic. What is the most likely diagnosis?
sequestration crisis
185
what may cause neutropenia in a man with rigors who is 3 days post-op TURP for prostate ca?
sepsis la
186
pepperpot skull XRay?
primary hyperparathyroidism
187
raindrop skull xray?
myeloma
188
Hypercalcaemia, renal failure, high total protein =
myeloma
| high/normal phosphate, normal ALP
189
```
70-year-old coal miner presents with 3 weeks of haematuria and bruising. He is normally fit and well. He is on no medications. His results reveal:
Hb 9.0
WCC 11
Pl 255
PT 16 (normal)
APTT 58 (increased)
Thrombin time 20 (normal). dx?
```
acquired haemophilia
190
A 28-year-old female is attends the gynaecology unit for a D+C following an incomplete miscarriage. She has previously had recurrent pulmonary embolic events. After the procedure she is persistently bleeding. Her APTT is 52 (increased). dx?
antiphospholipid syndrome
191
A painful maculopapular rash is a common feature of which disease?
graft vs host disease
192
spinal mets mx:
anaglesia
radiotherapy
spinal surgery
bisphosophonates
193
?CES mx:
MRI whole spine
| IV dexamethasone
194
A 54-year-old female is receiving a course of chemotherapy for breast cancer. She is experiencing troublesome vomiting which has not been helped by domperidone. What is the most appropriate next management step?
5HT3 antagonist (ondansetron)
195
commonest site of bone metastasis?
spine
| then pelvis, ribs, skull, long bones
196
If neoplastic spinal cord compression is suspected, give what while awaiting ix?
high dose oral dexamethasone
197
5M recurrent episodes of sinusitis. The casualty staff are surprised to find his liver lying in the left upper quadrant of the abdomen
what is the dx?
kartagener's syndrome
| situs invertus
198
A 22-year-old man presents with carcinoma of the caecum. His brother died from colorectal cancer aged 25 and his father died from the disease aged 30. dx?
lynch syndrome
| HNPCC (AD)
199
A tall 32-year-old lady presents with a diffuse neck swelling a carcinoma of the thyroid medullary type is diagnosed. genetic cause?
MEN IIb
| AD
200
lung ca commonest in smokers??
squamous cell ca
201
Autosomal dominant familial colorectal polyposis
Multiple colonic polyps
Extra colonic diseases include: skull osteoma, thyroid cancer and epidermoid cysts
Desmoid tumours are seen in 15%
Mutation of APC gene located on chromosome 5
Due to colonic polyps most patients will undergo colectomy to reduce risk of colorectal cancer
name?
gardener's syndrome
202
unexplained petechiae or hepatosplenomegaly in 0-24yo - mx?
immediate referral for assessment
203
white cell differential in this case demonstrates granulocytes at different stages of maturation (immature band forms and mature neutrophils) which is suggestive of?
CML
also low lymphocytes, high plts high band %
also +/- thrombocytosis
204
The universal donor of fresh frozen plasma is?
AB RhD negative blood
205
80M-> GP complaining of persistent mid-back pain for past 6/12. 7/10 in severity and preventing him from sleep. PMH stable angina and hypertension. Bony tenderness particularly in the thoracic spine and restricted range of movement. Bloods: raised corrected calcium level as well as a leucopenia. appropriate action?
urgent (<48h) bence jones, protein electrophoresis
206
high risk pts can get G-CSF (filgrastim). Examples of patients at high risk of febrile neutropenia include:
elderly
specific malignancies (e.g. non-Hodgkin's lymphoma, acute lymphoblastic leukaemia)
Previous neutropenic episodes
Those receiving combination chemotherapy and radiation therapy
207
how to decide whether question is getting you to tx neutropenic sepsis with Taz or with G-CSF?
in G-CSF no fever, pre-emptive
208
35M Hodgkin's lymphoma. He started the first day of his chemotherapy yesterday, but since then has not been feeling well. U&E and found some abnormalities including hyperkalaemia, hyperphosphataemia and hypocalcaemia. also raised creat. Cx, prophylaxis tx?
Tumour lysis syndrone with these electrolyte abnormalities
| allopurinol prophylaxis
209
which patients are more at risk for tumour lysis syndrone?
high-grade lymphomas and leukaemias - high turnover of cells
210
52F abdominal pain and constipation. Blue lines on the gum margin. She mentions that her legs have become weak in the past few days. dx?
```
lead poisoning
(gastrointestinal and neuropsychiatric symptoms and anaemia)
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211
15F abdominal pain. Mother known to have hereditary spherocytosis. The pain is located in the upper abdomen and is episodic in nature, but has become severe today. There has been no change to her bowel habit and no nausea or vomiting. dx?
biliary colic
she has HS which lead to chronic gallstone formation
212
lump in neck worse when drink alcohol?
hodgkins lymphoma
213
Target cells and Howell-Jolly bodies may be seen in??
coeliac disease - hyposplenism
214
TRALI is differentiated from TACO by the presence of??
BP
Hypotension = TRALI
Hypertension = TACO
215
in unwell child with temp with known sickle cell - mx?
admit urgently
216
acute haemolytic transfusion reaction - confirm dx?
coomb's test
| IgM
217
criteria for giving cryoprecipitate>
low fibrinogen levels
218
What is the mechanism of action of Dabigatran?
direct thrombin inhibitor
| can be used to reduce stroke risk in AF
219
Antiphospholipid syndrome in pregnancy: mx?
aspirin + LMWH
220
dyspnoea, chest pain, cough, hypoxia and new pulmonary infiltrates seen on chest x-ray. dx?
sickle cell - acute chest syndrome
| vaso-occlusive crises may mimic cold aiha sx
221
preferred NOAC for patients with renal impairment due to minimal renal drug clearance???
apixaban
222
if fever (slight) and itching during a transfusion - mx?
stop temporarily and give antihistamine
223
A raised ESR and osteoporosis represents
myeloma until proven otherwise
224
primary vs secondary polycythaemia?
patient in this example has a normal SpO2 and EPO which suggest that the polycythaemia is not secondary in nature.
225
what should be used in patients who are found to have iron deficiency anaemia prior to surgery where oral iron either can't be tolerated or the time interval is too short?
IV Iron 1g repeated 1 week later
226
Malaria prophylaxis (e.g. primaquine) can trigger haemolytic anaemia in those with?
G6PD
227
anaphylactic reaction to transfusion mx?
permanently stop the transfusion
| IM adrenaline, antihistamines, corticosteroids, bronchodilators and supportive care
228
22-year-old man with sickle cell anaemia presents with pallor, lethargy and a headache. Blood results:
Hb 4.6 g/dl
Reticulocytes 3%
Infection with a parvovirus is suspected. What is the likely diagnosis?
aplastic crisis
229
which AP specifically RF for VTE?
olanzipine
230
pentad of fever, neuro signs, thrombocytopenia, haemolytic anaemia and renal failure???
TTP
| Thrombotic thrombocytopaenia purpura
231
most common form of lymphoma in the UK?
diffuse large B-cell lymphoma
| NHL
232
stage 2 Hodgkin's lymphoma???
2+ lymph nodes involved on same side of diaphragm
233
isolated rise in haemoglobin?
polycythaemia vera
| also itching after shower
234
Platelet transfusion threshold: no active bleeding or planned invasive procedure -
10 x 10>9 for patients not bleeding or having an invasive procedure- except where CI or alternative treatments for their condition
235
CI for plts transfusion:
TTP
chronic bone marrow failure
Heparin-induced thrombocytopaenia
236
Irradiated blood products are used because??
they are depleted of T-lymphocytes to reduce the risk of transfusion GvH disease
237
Sickle cell patients should receive the pneumococcal polysaccharide vaccine every?
5yrs
238
tx TACO?
IV loop diuretic (furosemide)
239
reduce pyrexia in cases of non-haemolytic febrile transfusion reaction?
paracetamol
240
multiple episodes sinusitis as well as pneumococcal pneumonia in a young, non-smoking patient with no other explanation for immunodeficiency. This should prompt a screen for?
primary immunodeficiency - including serum immunoglobulins
241
how does myeloma -> stroke?
paraproteinaemia, hyperviscous state
242
IgA deficiency increases the risk of ?
anaphylactic blood transfusion reaction
243
low platelets
| prolonged prothrombin time, APTT and bleeding time??????????
DIC
244
Renal impairment in myeloma has 4 causes?
AL type amyloidosis
Bence jones nephropathy
nephrocalcinosis
nephrolithiasis
245
A 22-year-old man is having a blood transfusion after losing blood from haemorrhoids. He is normally fit and well. 3h during the transfusion he complains of sudden onset abdominal pain and nausea. His temperature is 39 degrees, Blood pressure 98/42 mmHg, HR 105 bpm and saturations 94% air. His urine appears dark. dx?
acute haemolytic transfusion reaction
246
A 32 year male with leukaemia attends the day unit for a blood transfusion. Five days after the transfusion he attends A&E with a temperature of 38.5, erythroderma and desquamation.
dx?
graft v host
247
Hereditary angioedema: the acute management is ?
IV C1-inhibitor concentrate
| ffp if this isn't available
248
the lack of urticaria, no signs of anaphylaxis, good air entry on auscultation, the family history, and low serum C4, which is used to help diagnose the condition.?
hereditary angiodema
249
It is important in a patient who is also deficient in both vitamin B12 and folic acid to treat which first?
B12 deficiency first - prevents subacute combined degeneration of the cord
250
what is associated with ‘tear drop’ poikilocytes on blood film?
myelofibrosis
251
first line ix for all people with IDA?
coeliac screen
252
IDA and b12 def at same time->
mixed anaemia
253
DVT in preg - tx?
LMWH
254
which HL has worst prog?
lymphocyte-depleted
255
kid with VWD awaiting ORIF - tx?
desmopressin
| stimulates release of VWF
256
Waldenstrom's macroglobulinaemia?
in CLL pts can cause a stroke - hyperviscous state
| IgM paraprotein
257
The jaundice, fatigue, and splenomegaly strongly suggest a?
haemolytic crisis
258
commonest type of HL?
nodular sclerosing
259
anticardiolipin abs??
APS
| SLE
260
DVT investigation: if the scan is negative, but the D-dimer is positive →
stop anticoagulation and repeat USS in 1 week
