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MRCP - Passmed > Haem + onc > Flashcards

Haem + onc Flashcards

1
Q

Which organisms most commonly cause neutropenic sepsis?

A

Coagulase -ve gram +ve bacteria

particularly staph epidermis

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2
Q

What are auer rods? what conditions are they seen in?

A

Large, pink / red needle shaped structures in cytoplasm of myeloid cells

Seen in AML
Also in APML, high grade myelodysplastic syndromes and myeloproliferative disorders

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3
Q

What are some features of APML?

A

Presents younger than other forms of AML (avg 25yrs)

DIC / Thrombocytopaenia at presentation

Good prognosis

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4
Q

Genetic cause of APML

A

t(15;17) translocation - fusion of PML and RAR-alpha genes –> blocks the maturation of myeloid cells and leads to an accumulation of promyelocytes.

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5
Q

APML mx?

A

Treated with all-trans retinoic acid (ATRA) -> forces immature granulocytes into maturation to resolve blast criseses prior to definitive chemo

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6
Q

Bloods in DIC? Blood film?

A

↓ platelets

↓ fibrinogen

↑ PT & APTT

↑ fibrinogen degradation products

schistocytes due to microangiopathic haemolytic anaemia

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7
Q

How can you distinguish between Waldenstrom’s macroglobulinaemia and multiple myeloma?

A

Waldenstrom’s macroglobulinaemia - Organomegaly with no bone lesions

Multiple myeloma - Bone lesions with no organomegaly

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8
Q

What are some features of Waldenstroms macroglobulinaemia?

Mx?

A

systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance (the pentameric configuration of IgM increases serum viscosity)
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s

Mx - rituximab based combination chemo

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9
Q

Ix findings for waldenstrom’s macroglobulinaemia?

A

Monoclonal IgM paraproteinaemia (M - Macroglobulinaemia)

BM biopsy - diagnostic - infiltration of BM with lymphoplasmacytoid lymphoma cells

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10
Q

What genetic abnormality is associated with chronic myeloid leukaemia (CML)?

A

Philidelphia chromosome - t(9;22)
seen in 95% of cases

NB also seen in 25% of adults with ALL - poor prognostic feature in these cases

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11
Q

What genetic abnormality is seen in Burkitt’s lymphoma?

A

t (8;14) seen in Burkitt’s lymphoma

MYC oncogene is translocated to an immunoglobulin gene

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12
Q

What genetic abnormality is seen in Mantle cell lymphoma?

A

t(11;14) is seen in mantle cell lymphoma

deregulation of BCL-1 gene

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13
Q

What genetic abnormality is seen follicular lymphoma?

A

t(14;18) - increased BCL-2 transcription

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14
Q

What are some intravascular causes of haemolytic anaemia?

A

mismatched blood transfusion
G6PD deficiency*
red cell fragmentation: heart valves, TTP, DIC, HUS
paroxysmal nocturnal haemoglobinuria
cold autoimmune haemolytic anaemia

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15
Q

Extravascular causes of haemolysis

A

haemoglobinopathies: sickle cell, thalassaemia
hereditary spherocytosis
haemolytic disease of newborn
warm autoimmune haemolytic anaemia

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16
Q

What does haptoglobin do?

A

Binds to free Hb which is released in intravascular haemolysis - hence depleted in intravascular causes

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17
Q

What happens to free Hb once haptoglobin is saturated? how is this detected?

A

As haptoglobin becomes saturated haemoglobin binds to albumin forming methaemalbumin (detected by Schumm’s test).

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18
Q

Bloods in Warfarin?

A

PT - Prolonged

APTT - Normal
Bleeding time - Normal
Plts - Normal

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19
Q

Bloods in Aspirin use?

A

Bleeding time - prolonged

PT - Normal
APTT - Normal
Plts - Normal

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20
Q

Bloods in heparin use?

A

APTT - Prolonged
PT - Normal (usually) / prolonged

Bleeding time - normal
Plts - Normal

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21
Q

How does ITP present?

A

Commonly in older females - isolated thrombocytopaenia usually incidentally on bloods

If sx:
- petechiae, purpura, bleeding (epistaxis) -> catastrophic bleeding is not common

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22
Q

What is Evans syndrome?

A

When ITP is associated with AIHA

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23
Q

How can you distinguish between Ig in Cold and Warm AIHA?

A

Warm = Greece IgG
Cold = Moscow IgM

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24
Q

What can be seen in blood film in hereditary spherocytosis post surgery and why?

A

Howell-Jolly bodies - these are remnants of RBC nucleus normally removed by spleen

Hence visible post splenectomy

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25
Q

What causes raised MCHC?

A

Hereditary spherocytosis

AIHA - associated w/ spherocytosis

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26
Q

What causes reduced MCHC

A

Microcytic anaemia eg IDA

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27
Q

What is disproportionate microcytic anaemia? when is this seen?

A

This is when MCV is far more reduced than drop in Hb

Seen in B-thalassaemia trait

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28
Q

Blood film findings in hyposplenism? examples?

A

target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes

eg. coeliac / post-splenectomy

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29
Q

Bloods film features in IDA?

A

target cells
‘pencil’ poikilocytes

if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

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30
Q

‘Tear drop’ poikilocytes are suggestive of which condition?

A

Myelofibrosis

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31
Q

What blood film feature is found in intravascular haemolysis?

A

Schistocytes

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32
Q

What are hypersegemented neutrophils suggestive of?

A

Megaloblastic anaemia

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33
Q

What are some examples of aromatase inhibitors? main adverse effects?

A

Anastrazole and Letrozole - used in ER +ve breast ca -> prevents androgen conversion into oestrogens (reducing peripheral oestrogen synthesis)

Adverse:
- Osteoporosis -> DEXA recommended when initiating
- Hot flushes
- Arthralgia + Myalgia
- Insomnia

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34
Q

Which medication should be used for chemo associated N+V?

A

Low risk - metoclopramide

High risk - 5HT3 antagonists eg ondansetron esp combined w dex

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35
Q

What genetic condtion is associated w young age tumours esp sarcomas + leukaemias? gene?

A

Li-Fraumeni Syndrome

p53 gene (sarcoma <45y & 1st degree relative any cancer + other family member any cancer <45y or sarcoma any age)

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36
Q

What is BRCA2 associated with in men?

A

Prostate Ca

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37
Q

Main symptoms of SVCO?

A

MOST COMMON - Dyspnoea

Swelling of face, neck and arms - ?oedema

Headaches - worse in AM

Visual disturbance

Pulseless JV distension

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38
Q

What is a leukaemoid reaction?

A

Presence of immature cells ef myeloblasts, promyelocytes and nucleated RBCs in peripheral blood

Due to infiltration of BM -> immature cells being pushed out
OR
Raised demand for new cells

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39
Q

What are some causes of leukaemoid reactions?

A

Severe infection
Severe haemolysis
Massive haemorrhage
Met Ca w/ BM infiltraion

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40
Q

How can you distinguish between leukaemoid reaction + myelofibrosis and CML?

A

Leucocyte Alkaline phosphatase (LAP) - raised in reactive response

CML - Low LAP

Leukaemoid reaction + Myelofibrosis - High LAP

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41
Q

What is HTLV1 and what ca is it associated with?

A

HTLV1 = human T cell lymphotropic virus

Associated w/ Adult T cell leukaemia

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42
Q

What ca is EBV associated with?

A

Hodgkins lymphoma
Burkitts lymphoma
Nasopharyngeal caricinoma

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43
Q

What ca is associated with HIV-1

A

High grade B cell lymphoma

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44
Q

What ca is associated w malaria?

A

Burkitts lymphoma

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45
Q

Starry sky appearance on microscopy suggests which ca? what does this describe?

A

Burkitts lymphoma

Starry sky = lymphocyte sheets interspersed w/ macrophages with dead apoptotic tumour cells

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46
Q

Electrolyte imbalances in Tumour Lysis Syndrome?

A

hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure

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47
Q

How can you distinguish between aplastic crises and sequestration crises in Sickle cells in blood test?

A

Aplastic - BM suppression -> reduced reticulocyte count

Sequestration - raised reticulocyte count

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48
Q

Inheritance pattern and mutation in hereditary angioedema?

Ix?

A

Autosomally dominant

Low plasma levels of C1-inhibitor (C1 inh, C1 esterase inhibitor)

Ix - Low C2 and C4 - even between attacks

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49
Q

What are the main adverse effects associated with Cisplatin?

A

Nephrotoxicity - main dose limiting issue

Ototoxicity

Peripheral neuropathy

Hypomagnesaemia

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50
Q

Features of TTP? Protein involved?

A

microangiopathic haemolytic anaemia, thrombocytopaenic purpura, neurological dysfunction, renal dysfunction and fever

Inhibition of ADAMTS13 - cleaves the large multimers of vWF

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51
Q

What are the features of transfusion associated graft v host disease? how can this risk be reduced?

A

GVHD - graft v host disease

G - Gut (enteritis)
V
H - Hepatitis
D - Dermatitis

Irradiated blood products -

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52
Q

Which drugs can trigger haemolysis in those with G6PD deficiency?

A

Anti-malarials: primaquine

ciprofloxacin

sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

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53
Q

Mx of APML?

A

All-trans retinoic acid (ATRA) - forces immature granulocytes into maturation

This resolves a blast crisis before definitive chemo

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54
Q

What does anaemia with raised reticulocytes tell us?

A

Bleeding or haemolysis

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55
Q

low antibody levels, specifically in immunoglobulin types IgG, IgM and IgA. → recurrent chest and other infections

Is suggestive of which disorder?

A

Common variable immunodeficiency

can predispose to AI disorders + lymphoma

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56
Q

Most common sx of SVCO?

A

Dyspnoea

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57
Q

What does Rituximab target?

A

Mab targeting CD20 on B cells -> B cell depletion + immunosuppression

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58
Q

When is leucocyte alkaline phosphatase low?

A

chronic myeloid leukaemia
pernicious anaemia
paroxysmal nocturnal haemoglobinuria
infectious mononucleosis

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59
Q

When is leucocyte alkaline phosphatase raised?

A

myelofibrosis
leukaemoid reactions
polycythaemia rubra vera
infections
steroids, Cushing’s syndrome
pregnancy, oral contraceptive pill

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60
Q

What are the different translocations involved in blood cancers?

A

T8: 14 –> Burkitts (also has 8 letters)
T8: 21 –> Acute Myeloid L (before C)
T9: 22 –> Chronic Myeloid L
T11:14 –> Cell (Mantle) ‘Middle’ Lymphoma - 11 looks like mantlepiece
T14:18 –> Follicular Lymphoma
T15:17 –> Promyelocytic

+ Lymphomas always have a 14

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61
Q

Which ca shows raised levels of bombesin?

A

Small cell lung carcinoma

Gastric ca

Neuroblastoma

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62
Q

Which ca has raised levels of S-100?

A

Melanomas

Schwannomas

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63
Q

How can you distinguish between Waldenstroms macroglobulinaemia and MM?

A

Waldenstrom’s macroglobulinaemia (type of NHL) - Organomegaly with no bone lesions

Multiple myeloma - Bone lesions with no organomegaly

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64
Q

MoA of Irinotecan? adverse effect?

A

Inhibits topoisomerase I which prevents relaxation of supercoiled DNA

Can lead to myelosuppression

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65
Q

MoA of cyclophosphamide? Adverse effects?

A

Cyclophosphamide - alkylating agent causing crosslinking in DNA

Can cause haemorrhagic cystitis, myelosuppression and transitional cell ca

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66
Q

Which cytotoxic drugs act on microtubules?

A

Vincristine + vinblastine - inhibits formation of microtubules

Docetaxel - prevents microtubule depolymerisation + disassembly -> decreasing free tubulin

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67
Q

What is Meigs syndrome?

A

Ovarian fibroma associated w pleural effusion + ascites

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68
Q

Most common organism in neutropenic sepsis?

A

Coagulase+ve Gram +ve cocci usually staph epidermis then other staph and strep species

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69
Q

Activated protein C resistance is also known as?

A

Factor V leiden

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70
Q

Antiphospholipid syndrome in pregnancy mx?

A

Aspirin + LMWH

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71
Q

Anti-emetic that is a neurokinin1 receptor blocker (NK1)?

A

Aprepitant

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72
Q

How do Metoclopramide, domperidone, and haloperidol act as anti-emetics?

A

Dopamine blocking effecs

73
Q

How are acute haemolytic crises managed in hereditary spherocytosis?

A

Generally supportive - IVF + high dose folic acid

Transfusion if necessary

74
Q

What effect does the philipdelphia chromosome have on mortality in blood cancers?

A

Philadelphia translocation, t(9;22):

good prognosis in CML

poor prognosis in AML + ALL

75
Q

recurrent bacterial infections (e.g. Chest)
eczema
thrombocytopenia

Is suggestive of which condition? blood also show what? gene?

A

Wiskott-Aldrich syndrome - primary immunodeficiency due to combined B+T cell dysfunction (mutated WASP gene)

Also see low IgM

76
Q

What is the major determining factor in the use of cryoprecipitate in bleeding?

A

Fibrinogen levels <1.5g/L

77
Q

Which type of hodgkins lymphoma has the best and worst prognosis?

A

Lymphocyte predominant - best
Lymphocyte depleted - worst

Mixed cellularity
Nodular sclerosing
Both offer good prognosis

78
Q

Ix of choice for CLL?

A

Immunophenotyping - flow cytometry

79
Q

Smudge / Smear cells are seen in which haem condition?

A

CLL

80
Q

Mx of ITP?

A

Platelet count >30*109/L = Observation

Platelet count <30*109/L = Oral prednisolone

Emergency treatment: life-threatening or organ threatening bleeding = Platelet transfusion, IV methylprednisolone and intravenous immunoglobulin

81
Q

What is Evans syndrome?

A

ITP associated with AIHA

82
Q

What is given to prevent haemorhagic cystitis seen with cyclophosphamide?

A

Mesna

83
Q

Raynauds is seen in which type of cryoglobulinaemia?

A

Type 1

84
Q

What should be used instead of an osmotic fragility test for hereditary spherocytosis?

A

EMA binding test

85
Q
A
86
Q

What are the diagnostic criteria for MM?

A

The diagnostic criteria for multiple myeloma requires one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of multiple myeloma.

Major criteria
Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine

Minor criteria
10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.

87
Q

How can you distinguish between MM and MGUS?

A

Differentiating features from myeloma
> normal immune function
> normal beta-2 microglobulin levels
> lower level of paraproteinaemia than myeloma (e.g. < 30g/l IgG, or < 20g/l IgA)
> stable level of paraproteinaemia
> no clinical features of myeloma (e.g. lytic lesions on x-rays or renal disease)

88
Q

the presence of HCV infection (PCR positive), positive rheumatoid factor, low complement levels, and purpuric rash

Suggests which condition?

A

hepatitis C virus (HCV) associated mixed cryoglobulinaemia - TYPE 2

89
Q

What medications can be safe in G6PD deficiency?

A

hepatitis C virus (HCV) associated mixed cryoglobulinaemia

90
Q

Mx of acute and prophylaxis against hereditary angioedema?

A

Acute - IV C1 inh concentrate or FFP if not available

Prophylaxis - Anabolic steroid danazol

91
Q

What are the features of lead poisoning?

A

abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue
constipation
blue lines on gum margin (only 20% of adult patients, very rare in children)

92
Q

How can you distinguish between TTP and HUS?

A

TTP has neurological signs too

93
Q

Most common presenting complaint in myelofibrosis?

A

lethargy

94
Q

Where is B12 absorbed?

A

B12 is actively absorbed in terminal ileum

95
Q

What can occur in 10-15% of those with Waldenstroms macroglobulinaemia?

A

Hyperviscosity syndrome

96
Q

Why does CKD increase risk of VTE?

A

Loss of antithrombin III - most common cause of this deficiency

97
Q

Mx of neoplastic spinal cord compression?

A

High dose oral dex whilst awaiting MRI

98
Q

Difference between TACO and TRALI?

A

TACO - HTN

TRALI - Hypotension

99
Q

Which drugs can cause methaemoglobinaemia?

A

sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine

100
Q

B12 replacement regime?

A

if no neurological involvement 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months

if a patient is also deficient in folic acid then it is important to treat the B12 deficiency first to avoid precipitating subacute combined degeneration of the cord

101
Q

Can you get aplastic anaemia with PNH?

A

Yes - though not necessarily directly

102
Q

What are the main bloods in tumour lysis syndrome?

A

Raised urate
Raised K
Raised PO4

Low Ca

Laboratory TLS is defined by ≥2 of the following occurring 3 days prior or 7 days post initiation of treatment for cancer:
Uric acid ≥ 476 µmol/L or 25% increase from baseline
Potassium ≥ 6.0 mmol/L or 25% increase from baseline
Phosphate ≥ 1.45 mmol/L (adults) or ≥2.1 mmol/L (children) or 25% increase
Calcium ≤ 1.75 mmol/L or 25% decrease from baseline

Clinical TLS is defined by: laboratory TLS plus at least one of the following:
Creatinine ≥1.5 x the upper limit of normal
Cardiac arrhythmia
Seizure
Sudden death

103
Q

How can you distinguish between higher spinal cord compression than L1 v cauda equina?

A

Presence of UMN signs

104
Q

Mx of alcoholic hepatitis?

A

Glucocorticoids eg pred

Pentoxyphylline sometimes used -not shown to improve surivial rates whereas glucocorticoids do

105
Q

Mx of hyatid cysts?

A

Surgery is mainstay - cyst walls must not be ruptured during removal and contents sterilised first

106
Q

Different genotypes of sickle cell disease?

A

normal haemoglobin: HbAA
sickle cell trait: HbAS

homozygous sickle cell disease: HbSS - severe disease
milder sickle cell disease: HbSC - Some patients inherit one HbS and another abnormal haemoglobin (HbC)

in the deoxygenated state the HbS molecules polymerise and cause RBCs to sickle
HbAS patients sickle at p02 2.5 - 4 kPa
HbSS patients at p02 5 - 6 kPa

107
Q

Drug causes of pancytopaenia?

A

If you give a patient bad news about him suffering from Panyctopenia- It will cause ACS.

Antibiotics: Trimethoprim, Chloramphenicol.
AED: Carbamazepine (can also cause agranulocytosis)
anti-rheumatoid: Penicillamine, Gold

Carbimazole
Cytotoxics

Sulfonylureas: Tolbutamide

AAA CC S

108
Q

Which lung ca can secrete HCG? prognosis

A

Large cell lung ca - anaplastic + poorly differentiated w/ poor prognosis

109
Q

Which non small cell lung ca is found central v peripheral

A

central - SCC

peripheral - adeno + large cell

110
Q

What are the different primary immunodeficiencies affecting T+B cells?

A

SCID,
Wiskott-Aldrich syndrome,
ataxic telangiectasia
Hyper IgM Syndromes

111
Q

B-cell primary immunodeficiencies?

A

Common variable immunodeficiency

Bruton’s (x-linked) congenital agammaglobulinaemia

Selective immunoglobulin A deficiency

112
Q

Neutrophil associated primary immunodeficiencies?

A

Chronic granulomatous disease

Chediak-Higashi syndrome

Leukocyte adhesion deficiency

113
Q

What ethnicity is benign ethnic neutropaenia seen in?

A

black African and Afro-Caribbean ethnicity

114
Q

CLL why may there be increased risk of infections?

A

hypogammaglobulinaemia

115
Q

Features + mx of essential thrombocytosis?

A

platelet count > 600 * 109/l
both thrombosis (venous or arterial) and haemorrhage can be seen
a characteristic symptom is a burning sensation in the hands
a JAK2 mutation is found in around 50% of patients

Overlaps with CML, polycythaemia + myelofibrosis

Mx = hydroxyurea (hydroxycarbamide), Inf-a in younger pts + low dose aspirin to reduce thrombosis risk

116
Q

Which cytotoxic agent - Degrades preformed DNA? adverse effect?

A

Bleomycin

Can lead to lung fibrosis

117
Q

Which cytotoxic drug Causes cross-linking in DNA?

A

Cisplatin

118
Q

Which cytotoxic drug Inhibits ribonucleotide reductase, decreasing DNA synthesis?

A

Hydroxyurea (hydroxycarbamide)

119
Q

Which Ca is Warm AIHA associated with? Blood film findings?

A

CLL is associated w warm AIHA in 10-15% of cases

Blood film can be normal as haemolysis occurs extravascularly

120
Q

What is the main advantage of using capecitabine instead of fluorouracil?

A

This is a pro drug for 5-FU means it can allow for oral administration hence OP mx

121
Q

Possible mx in ITP?

A

oral prednisolone (80% of patients respond)
splenectomy if platelets < 30 after 3 months of steroid therapy
IV immunoglobulins
immunosuppressive drugs e.g. cyclophosphamide

122
Q

Widespread lymphadenopathy + multi organ problems - what dx?

A

IgG4 disease

Examples include:
Riedel’s Thyroiditis
Autoimmune pancreatitis
Mediastinal and Retroperitoneal Fibrosis
Periaortitis/periarteritis/Inflammatory aortic aneurysm
Kuttner’s Tumour (submandibular glands) & Mikulicz Syndrome (salivary and lacrimal glands)
Possibly sjogren’s and primary biliary cirrhosis

123
Q

Haemodilution in pregnancy - bloods show what?

A

Normocytic anaemia

124
Q

Most common cause of SVCO?

A

Primary tumour - Most common is non-small cell lung cancer, small cell lung cancer and non-Hodgkin lymphoma.

125
Q

CALR is associated with what?

A

JAK2 -ve essential thrombocytosis

126
Q

Hypo / hyperdiploidy in ALL effect on prognosis

A

Hypo = poor prognosis

Hyper = good prognosis

127
Q

SCID prognosis?

A

Patients are afflicted with significant infections from infancy and do not survive into childhood unless the condition is diagnosed early and treated with bone marrow transplantation.

128
Q

Chromosome changes and prognosis in CLL? Other poor prognostic features?

A

Short arm of chromosome 17 (del 17p) - Poor prognosis

long arm of chromosome 13 (del 13q) - Good prognosis

Poor prognostic
male sex
age > 70 years
lymphocyte count > 50
prolymphocytes comprising more than 10% of blood lymphocytes
lymphocyte doubling time < 12 months
raised LDH
CD38 expression positive
TP53 mutation

129
Q

Desmopressiin (DDAVP) use in VWD?

A

desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

130
Q

Poor prognosis in HL?

A

‘B’ symptoms also imply a poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats

Other factors associated with a poor prognosis identified in a 1998 NEJM paper included:
age > 45 years
stage IV disease
haemoglobin < 10.5 g/dl
lymphocyte count < 600/µl or < 8%
male
albumin < 40 g/l
white blood count > 15,000/µl

130
Q

Reed sternberg cells seen in?

A

Hodgkins lymphoma

130
Q

Mx of CLL, CML, Hodgkins and NHL?

A

FCR (Fludarabine, Cyclophosphamide and Rituximab) - CLL (Ibrutinib or Venetoclax instead of FCR if TP53)

AVBD (Doxorubicin, bleomycin, vinblastine, dacarbazine) - Hodgkins

R-CHOP - Non-Hodgkins

Imatinib - CML

131
Q

Anterior mediastinal mass + symptoms of myasthenia

Suggestive of what dx?

A

Thymoma

132
Q

Ix findings in VWD?

A

prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin

133
Q

Ham’s test used for what?

A

diagnose paroxysmal nocturnal hemoglobinuria (PNH)

134
Q

What to check when thinking of lead poisoning?

A

Coproporphyrin

135
Q

He is initially treated with low-molecular weight heparin but is switched after three days to warfarin. He then develops necrotic skin lesions on his lower limbs and forearms.

Which one of the following conditions is characteristically associated with this complication?

A

development of necrotic skin lesions in this patient after being switched to warfarin suggests the presence of a condition known as warfarin-induced skin necrosis (WISN)

Associated with Protein C deficiency

136
Q

What are thymomas associated with?

A

myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH

137
Q

When to use skin patch v skin prick testing?

A

If it goes in you (food) > skin prick T1 HS 15-30 mins

If it goes on you (e.g. contact) > skin patch

138
Q

MoA of HPV 16/18 mediated cervical ca?

A

HPV 16 & 18 produces the oncoproteins E6 and E7 genes respectively which inhibit TSGs

E6 inhibits p53 tumour suppressor gene
E7 inhibits RB tumour suppressor gene

139
Q

Defect in which enzyme in AIP and PCT?

A

Acute intermitten porphyria - porphobilinogen deAminase

PCT - uroporphyrinogen deCarboxylase

140
Q

Indications for tx in CLL?

A

progressive marrow failure: the development or worsening of anaemia and/or thrombocytopenia

massive (>10 cm) or progressive lymphadenopathy

massive (>6 cm) or progressive splenomegaly

progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months

systemic symptoms: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats

autoimmune cytopaenias e.g. ITP

141
Q

What is the most common inherited bleeding disorder?

A

VWD

142
Q

Prognostic marker in Myeloma?

A

B2 microglobulin - raised levels imply poor prognosis.

Low levels of albumin are also associated with a poor prognosis

143
Q

Which haem malignancy shows:

there is an increase in granulocytes at different stages of maturation +/- thrombocytosis?

A

CML

AML -less time, less platelets,
CML - more time, more platelets

Band cells are an immature form of neutrophils

144
Q

How are acute painful vaso occlusive crises diagnosed in sickle cell?

A

Diagnosed clinically

145
Q

ESR in polycythaemia rubra vera?

A

Low - increased number of red blood cells that reduce the relative proportion of plasma and thus sedimentation

146
Q

AML poor prognostic features?

A

AML poor prognostic features:

> 60 years
20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7

147
Q

TTP mx?

A

Plasma exchange = 1st line

Steroids, immunosuppressants and Vincristine may also help

148
Q

Rasburicase - use case and moa?

A

Used to reduce risk of tumour lysis syndrome in high risk pts (allopurinol given in lower risk - DO NOT GIVE TOGETHER AS REDUCES EFFECT OF RASBURICASE)

Recombinant urate oxidase -> conversion of urate to allantoin

149
Q

Primary peritoneal ca - marker?

A

Ca125 + monitor disease progression / response to tx

150
Q

The universal donor of fresh frozen plasma is what?

A

AB RhD -ve

151
Q

Test for diagnosis and prognosis in AML?

A

Diagnosis - Immunophenotyping

Prognosis - Cytogenetics

152
Q

What are the most common types of transformations seen in patients with polycythaemia vera?

A

5-15% progress to myelofibrosis or AML

153
Q

When to use VKAs for DVT?

A

if renal impairment is severe (e.g. < 15/min) then LMWH, unfractionated heparin or LMWH followed by a VKA

if the patient has antiphospholipid syndrome (specifically ‘triple positive’ in the guidance) then LMWH followed by a VKA should be used

154
Q

Is Histamine implicated in Hereditary angioedema (HAE)?

A

No

However Kallikrein + High molecular weight kininogen + CNHesterase inh + bradykinin are implicated

155
Q

Other than High LAP what are other features of leukaemoid reactions?

A

toxic granulation (Dohle bodies) in the white cells

‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus

156
Q

Which one of the following causes of thrombophilia is associated with resistance to heparin?

A

Antithrombin III deficiency

anti-Xa levels should be monitored carefully to ensure adequate anticoagulation

157
Q

Causes of warm v cold AIHA?

A

Causes of cold AIHA
- neoplasia: e.g. lymphoma
- infections: e.g. mycoplasma, EBV

Causes of Warm AIHA
- Idiopathic
- autoimmune disease: e.g. systemic lupus erythematosus*
- neoplasia (lymphoma, chronic lymphocytic leukaemia)
- drugs: e.g. methyldopa

158
Q

Prevalence of Factor V leiden?

A

5%

159
Q

When to discharge after anaphylaxis?

A

fast-track discharge (after 2 hours of symptom resolution):
- good response to a single dose of adrenaline
- complete resolution of symptoms
- has been given an adrenaline auto-injector and - trained how to use it
- adequate supervision following discharge

minimum 6 hours after symptom resolution
- 2 doses of IM adrenaline needed, or
- previous biphasic reaction

minimum 12 hours after symptom resolution
- severe reaction requiring > 2 doses of IM adrenaline
- patient has severe asthma
- possibility of an ongoing reaction (e.g. slow-release medication)
- patient presents late at night
- patient in areas where access to emergency access care may be difficult
- observation for at 12 hours following symptom resolution

160
Q

What causes sequestration crises and what may be seen?

A

Sequestration crises occur due to sickling of cells within the splenic vasculature, and resultant trapping of significant blood volume within the spleen itself.

You can see splenomegaly and hypotension

161
Q

Sideroblastic anaemia can be caused by what? what stain to use?

A

Congenital cause: delta-aminolevulinate synthase-2 deficiency

Acquired causes
myelodysplasia
alcohol
lead
anti-TB medications

Ix = Prussian blue / Perls staining will show ringed sideroblasts

162
Q

Can INF-A inhibtors be used for Hairy cell leukaemia (rare B cell disorder)?

A

No

163
Q

NHL is associated with which AIHA?

A

Warm AIHA + Cold AIHA - look at IgG v IgM

164
Q

The patient’s symptoms of purplish discolouration of her peripheries and nose, along with fatigue and lethargy, combined with the laboratory results showing spherocytes on her blood film

? dx

A

Possible AIHA

165
Q

vCJD risk in blood transfusion?

A

There had previously been a small risk of vCJD transmission but the risk has now been eliminated through screening

166
Q

a + b thalassaemia gene located where?

A

alpha thalassemia = chromosome 16
beta thalassemia = chromosome 11

167
Q

Mx of aplastic anaemia?

A

Blood products
Infection prevention
Anti-thymocyte globulin (ATG) and anti-lymphocyte globulin (ALG)
Stem cell transplants

168
Q

Mx of hereditary spherocytosis?

A

Acute haemolytic crises - supportive + transfusion if req

Long term - Folate replacement + splenectomy

169
Q

ECOG - performance status for future onc therapies scale

A

0 Fully active, able to carry on all pre-disease performance without restriction

1 Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, e.g., light house work, office work

2 Ambulatory and capable of all selfcare but unable to carry out any work activities; up and about more than 50% of waking hours

3 Capable of only limited selfcare; confined to bed or chair more than 50% of waking hours

4 Completely disabled; cannot carry on any selfcare; totally confined to bed or chair

5 Dead

170
Q

Causes of hyposplenism?

A

splenectomy
sickle-cell
coeliac disease, dermatitis herpetiformis
Graves’ disease
systemic lupus erythematosus
amyloid

171
Q

Which BRCA causes prostate ca in men?

A

BRCA 2

172
Q

Features and cause of myelofibrosis?

A

e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc

a myeloproliferative disorder
thought to be caused by hyperplasia of abnormal megakaryocytes
the resultant release of platelet derived growth factor is thought to stimulate fibroblasts
haematopoiesis develops in the liver and spleen

173
Q

Latex fruit syndrome?

A

It is recognised that many people who are allergic to latex are also allergic to fruits, particularly banana, pineapple, avocado, chestnut, kiwi fruit, mango, passion fruit and strawberry.

174
Q

experiencing intense pruritis especially after a hot shower and her skin appearing red and flushed over the past year.

Suggests what?

A

PML

175
Q

Methaemoglobinaemia mx?

A

NADH methaemoglobinaemia reductase deficiency: ascorbic acid

IV methylthioninium chloride (methylene blue) if acquired

176
Q
A

MRCP - Passmed (17 decks)

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