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MRCP Revision > Haematology/Oncology > Flashcards

Haematology/Oncology Flashcards

1
Q

Commonest cancers

A

Breast
Lung
Colorectal
Prostate
Bladder
Non-Hodkin’s
Melanoma
Stomach
Oesophagus
Pancreas

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2
Q

Commonest deaths from cancer

A

Lung
Colorectal
Breast
Prostate
Pancreas
Oesophagus
Stomach
Bladder
non-hodgkin’s
ovarian

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3
Q

Ca15-3

A

breast ca

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4
Q

S-100

A

Melanoma, schwannoma

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5
Q

Bombesin

A

small cell lung ca, gastric, neuroblastoma

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6
Q

Ca125

A

ovarian, peritoneal

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7
Q

Li-Fraumeni

A

Aut Dom, p53, sarcomas, leukaemias

Dx: sarcoma under 45yrs, 1st deg relative any cancer under 45yrs + another family member develops cancer under 45yrs or sarcoma any age

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8
Q

BRCA1

A

Chr 17. Breast 60%, ovarian 55%

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9
Q

BRCA2

A

Chr 13. Breast 60%, ovarian 25%, prostate

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10
Q

Lynch syndrome

A

Aut dom, colon, endometrial, (80%)

Amsterdam criteria: 3 or more family members with colorectal, 1 must be 1st deg relative of other two + Two successive affected generations + One or more colon cancers diagnosed under age 50 + FAP excluded

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11
Q

Gardner’s syndrome

A

Aut Dom, colorectal polyps, osteoma, thyroid, epidermoid cysts, desmoid tumours in 15%, APC Chr 5 (FAP variant), most get colectomy prophylactically

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12
Q

List some tumour suppressor genes

A

p53 (li-fraumeni), APC (colorectal), BRCA1, 2 (breast, ovarian, prostate for 2), NF1, Rb, WT1 (Wilm’s), MTS-1, p16 (melanoma)

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13
Q

List some oncogenes

A

ABL (CML), c-MYC (Burkitt’s), n-MYC (Neuroblastoma), BCL-2 (Follicular lymphoma), RET (MEN II, III), RAS (pancreatic Ca), erb-B2/HER2/neu (breast, ovarian)

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14
Q

Where do bone mets commonly come from?

A

Prostate
Breast
Lung

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15
Q

Where are bone mets commonly seen?

A

Spine
Pelvis
Ribs
Skull
Long bones

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16
Q

Aflatoxin predisposes to which cancer?

A

HCC

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17
Q

Aniline dye - which cancer?

A

TCC

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18
Q

Asbestos - which cancer?

A

Mesothelioma
Bronchial Ca

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19
Q

Nitrosamines - which cancer?

A

oesophageal
gastric

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20
Q

Vinyl chloride - which cancer?

A

hepatic angiosarcoma

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21
Q

EBV predisposes to which cancer?

A

Burkitt’s
Hodgkin’s
Post-transplant lymphoma
Nasopharyngeal

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22
Q

HPV 16/18 predisposes to which cancers?

A

cervical
anal
penile
vulval
oropharyngeal

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23
Q

HPV8 - which cancer?

A

kaposi’s

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24
Q

Hep B, C - which cancers?

A

HCC

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25
Q

HTLV1 - predisposes to what?

A

Tropical spastic paraparesis
Adult T cell leukaemia

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26
Q

ECOG score

A

(performance status) 0-5
1: ambulatory + light work
2: ambulatory + no work
3: confined to bed/chair >50%
4: completely disabled
5: dead

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27
Q

How to diagnose multiple myeloma?

A

Major criteria: plasmacytoma (biopsy), 30% plasma cells in bone marrow sample, Elevated M proteins.

Minor criteria: 10-30% plasma cells in bone marrow sample, minor M protein elevation, osteolytic lesions, low levels of antibodies

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28
Q

Poor prognosis in myeloma

A

High B2 microglobulin
Low albumin

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29
Q

Hyposplenism blood film

A

Howell-Jolly bodies
Target cells
Pappenheimer bodies
siderocytic granules
acanthocytes

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30
Q

Hereditary spherocytosis inheritance pattern

A

Aut Dom

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31
Q

Hereditary spherocytosis features

A

Neonatal jaundice
Gallstones
Splenomegaly
Extravascular haemolysis

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32
Q

How to dx hereditary spherocytosis?

A

EMA binding test
(also can do cryohaemolysis test)

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33
Q

Rx for hereditary spherocytosis

A

Supportive
Folic acid
Splenectomy

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34
Q

G6PD deficiency inheritance

A

X recessive

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35
Q

G6PD deficiency haemolysis type

A

Intravascular

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36
Q

Precipitants of G6PD deficiency

A

Primaquine
Ciprofloxacin
Sulph-drugs

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37
Q

Blood film for G6PD def

A

Heinz bodies

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38
Q

How to dx G6PD def

A

enzyme assay

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39
Q

Sickle cell genotypes

A

HbAS - carrier
HbSS - homozygous
HbSC - milderform

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40
Q

Sickle cell mutation

A

Glutamate to valine in codon 6

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41
Q

What pO2 do they sickle in sickle cell disease?

A

HbAS: at pO2 2.5-4
HbSS: at pO2 5-6

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42
Q

how to dx sickle cell disease

A

electrophoresis

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43
Q

Types of sickle cell crises

A

Thrombotic/vaso-occlusive: commonest cause of death. caused by infection, dehydration, deoxygenation, painful, infarcts in bones, lungs, spleen, brain

Acute chest: pulmonary microvasculature. Rx: supportive, abx, transfusion

Aplastic: parvovirus b19, reduced reticulocytes (BM suppressed)

Sequestration: spleen or lungs, reticulocytes seen

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44
Q

Alpha thalassaemia genetics

A

2 alpha globulin genes on each Chr 16. If 1-2 affected, Hb normal with microcytic hypochromic picture. If 3 affected, HbH. If 4 affected, hydrops fetalis/Bart’s hydrop

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45
Q

Beta thalassaemia features

A

microcytosis disproportionate to anaemia. HbA2 raised (>3.5%)

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46
Q

Causes of neutropenia

A

HIV, EBV, hepatitis, cytotoxics, carbimazole, clozapine, benign Afro-Caribbean ethnic, MDS, aplastic anaemia, SLE, Rh arthr, severe sepsis, haemodialysis

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47
Q

Causes of high leukocyte ALP

A

Myelofibrosis
Leukaemoid reactions
PRV
infections
steroids
pregnancy

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48
Q

Causes of low leukocyte ALP

A

CML
pernicious anaemia
PNH
infectious mononucleosis

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49
Q

aromatase inhibitors SE

A

osteoporosis
NICE recommends a DEXA scan when initiating a patient on aromatase inhibitors for breast cancer
hot flushes
arthralgia, myalgia
insomnia

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50
Q

n-Myc - which cancer?

A

Neuroblastoma

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51
Q

Hodkin’s classification/staging:

A

Lugano’s classification is basically Ann-Arbor + E (extranodal)/S (splenic involvement)/X (bulky disease)

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52
Q

Types of Hodkin’s:

A

Nodular sclerosing: 60-80%, Reed-Sternberg, women, lacunar cells

Mixed cellularity: 15-30%, Reed-Sternberg

Lymphocyte predominant: <5%, bet prognosis

Lymphocyte depleted: <1%, poor prognosis

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53
Q

poor prognosis in Hodkin’s

A

Male
age>45
Stage IV
Hb<105
lymphocyte<600
albumin <40
WCC>15000

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54
Q

Treatment of Hodgkin’s

A

ABVD - anthracycline (doxorubicin), bleomycin, vinblastine, dacarbazine.

BEACOPP - bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone has better remission rates with higher toxicity.

Radiotherapy, combined modality therapy (chemo + radio), haematopoietic cell transplantation (if relapsed/refractory)

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55
Q

Burkitt’s genetics

A

c-Myc translocation to immunoglobulin gene t(8:14).

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56
Q

Burkitt’s types:

A

Endemic (African) form: maxilla/mandible, EBV association

Sporadic form: abdominal, HIV association

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57
Q

What complication in common in Burkitt’s?

A

Tumour lysis

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58
Q

Burkitt’s blood film feature:

A

‘Starry sky’ appearance

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59
Q

Mantle cell lymphoma genetics:

A

t(11:14) deregulation of cyclin D1 (BCL-1) gene

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60
Q

Follicular lymphoma genetics:

A

t(14:18) increased BCL-2 transcription

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61
Q

Treatment of non-Hodgkin’s lymphoma

A

R-CHOP

Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisolone

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62
Q

ALL good prognosis

A

FAB L1 type
pre-B phenotype
low WBC
del(9p)
hyperdiploidy
Trisomy 4, 10, 17
t(12:21) TEL-AML1
t(1:19)

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63
Q

ALL poor prognosis

A

FAB L3 type, T or B cell markers, t(9:22), age<2 or >10yrs, males, CNS involvement, high WBC, non-caucasian, hypodiploidy

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64
Q

AML poor prognosis

A

> 60yrs, >20%, cytogenetics: Chr 5 or 7 deletion

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65
Q

APML genetics

A

t(15:17), PML + RAR alpha genes fusion

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66
Q

APML features

A

Younger (25yrs), Auer rods (myeloperoxidase stain), DIC seen, good progosis

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67
Q

Rx of APML

A

ATRA
All trans retinoic acid

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68
Q

French-American-British classification

A

M0 undifferentiated, M1 without maturation, M2 with granulocytic maturation, M3 APML, M4 granulocytic and monocytic maturation, M5 monocytic, M6 erythroleukaemia, M7 megakaryoblastic

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69
Q

Complications of CLL

A

10-15% have warm AIHA
Hypogammaglobulinaemia –> infections
Richter’s transformation –> non-Hodgkin’s

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70
Q

CLL blood film and investigations

A

smudge cells (film)
immunophenotyping - CD5, 19, 20, 23

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71
Q

Treatment indications in CLL

A

progressive marrow failure (anaemia/thrombocytopenia)
lymphadenopathy >10cm/progressive
splenomegaly >6cm/progressive
lymphocytosis >50% increase over 2 months/doubling in less than 6 months
B symptoms (weight loss >10% in 6 months, fever 38 for >2 weeks), autoimmune cytopenias

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72
Q

Treatment of CLL

A

FCR - Fludarabine, cyclophosphamide, rituximab. Imatinib if failed.

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73
Q

Poor prognosis in CLL

A

male, age >70, lymphocytes>50, prolymphocytes >10%, lymphocyte doubling <12 months, raised LDH, CD38, TP53, del 17p13 (5-10%)

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74
Q

Good prognosis in CLL

A

del13q14 (50%)

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75
Q

CML genetics:

A

Chr 9 + 22 - t(9:22)(q34; q11), BCR-ABL fusion gene - tyrosine kinase activity

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76
Q

Complications of CML

A

Blast transformation - AML 80%, ALL 20%.

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77
Q

Treatment for CML

A

Imatinib, hydroxyurea, interferon alpha

allogenic bone marrow transplant

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78
Q

Features of hairy cell leukaemia

A

rare, B cell proliferation
Males 4:1

pancytopenia
splenomegaly
skin vasculitis in 1/3 patients
‘dry tap’ despite bone marrow hypercellularity
tartrate resistant acid phosphotase (TRAP) stain positive

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79
Q

Hairy cell leukaemia Rx:

A

chemotherapy is first-line: cladribine, pentostatin

immunotherapy is second-line: rituximab, interferon-alpha

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80
Q

Fanconi anaemia features:

A

aut recessive
aplastic anaemia, AML risk, neuro, skeletal, cafe-au-lait

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81
Q

Cryoglobulinaemia type 1

A

monoclonal with IgG or IgM
Raynaud’s, waldenstrom’s, Myeloma

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82
Q

Cryoglobulinaemia type 2:

A

mixed,
HCV, RhArth, Sjogren’s, Lymphoma

83
Q

Cryoglobulinaemia Type 3:

A

polyclonal
Rh Arthritis
Sjogren’s

84
Q

What happens to complement and ESR in cryoglobulinaemia?

A

low complement
high ESR

85
Q

Rx for cryoglobulinaemia

A

treat underlying
immunosuppression
plasmapheresis

86
Q

Waldenstrom’s macroglobulinaemia features

A

monoclonal IgM
hyperviscosity
hepatomegaly
T1 cryoglobulinaemia with Rayaud’s

87
Q

Waldenstrom’s macroglobulinaemia investigations

A

IgM paraproteinaemia
BM biopsy shows infiltration with lymphoplasmacytoid lymphoma cells.

88
Q

Waldensttrom’s macroglobulinaemia Rx:

A

rituximab-based chemo

89
Q

Most common form of bleeding disorder

A

von Willebrand’s (1% of population)

90
Q

Features of von willebrand’s

A

Aut Dom
Prolonged bleeding time, APTT
reduced factor 8

91
Q

Types of Von willebrand’s

A

T1: partial reduction. 80%
T2: abnormal vWF. 2A defective pltlt adhesion (vWF protein too small). 2B pathological increase of vWF-pltlt interaction. 2M reduced vWF-pltlt interaction. 2N abnormal vWF-factor 8 binding.
T3: total lack of vWF (aut rec, most severe)

92
Q

Treatment of von willebrand’s

A

tranexamic acid
Desmopressin (raises vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells)
Factor 8 concentrate

93
Q

Describe Factor V Leiden

A

Activated protein C resistance, 5% of UK pop (most common), gain of function mutation in Factor V (clotting factor), inactivated 10x more slowly by activated protein C

Heterozygous - 4x VTE risk. Homozygotes (0.05%) 10x VTE risk

94
Q

Protein C deficiency

A

aut co-dom, skin necrosis with warfarin. VTE risk x 10

95
Q

Antithrombin III deficiency

A

Aut dom. Heterogeneous group. Antithrombin III inhibits thrombin, Factor 9, 10, mediates heparin effects. Recurrent VTEs, sometimes arterial. Rx: lifelong warfarin, heparin during pregnancy (monitor anti-Xa levels), antithrombin III concentrates. VTE risk x 10-20

96
Q

Antiphospholipid syndrome

A

acquired. APTT rise due to ex-vivo lupus anticoagulant ab reaction with phospholipids. Anti-cardiolipin, beta 2 GPI, also present. Rx: Low-dose aspirin for primary thromboprophylaxis, lifelong warfarin otherwise

In pregnancy, low dose aspirin + LMWH once fetal heart seen on USS (stopped at 34 weeks)

97
Q

Thrombotic Thrombocytopenic Purpura

A

Acquired ADAMTS13 inhibition - vWF not broken down, causes widespread pltlt adhesion + thrombosis. Females

Causes: post-infection, pregnancy, ciclosporin, COCP, penicillin, clopidogrel, aciclovir, tumours, SLE, HIV.

FAT RN fever, anaemia (MAHA - low haptoglobin, schistocytes), thrombocytopenia, renal, neuro/confusion

Rx: plasma exchange

98
Q

Idiopathic Thrombocytopenic purpura

A

anti-Glycoprotein IIb-IIIa or Ib complex.

Ix: IgG autoantibodies, BM aspiration (megakaryocytes)

Rx: oral pred. Splenectomy if pltls<30 after 3 months of steroids. IVIG. cyclophosphamide

99
Q

Disseminated intravascular coagulation

A

Vascular damage exposes tissue factor to circulation, as well as in response to TNF, endotoxin, IL1. TF abundant in lungs, brain, placenta. Activates extrinsic pathway, which subsequently triggers intrinsic pathway

Causes: sepsis, trauma, obstetric compl, malignancy

Dx: low platelets, low fibrinogen, high PT, APTT, D-Dimer, schistocytes (MAHA)

100
Q

Polycythaemia rubra vera:

A

JAK2 95%, low ferritin.

If JAK2 -ve, ix with: red cell mass, arterial gas, abdo USS, serum erythropoietin, bone marrow aspirate, trephine, cytogenetic analysis, erythroid burst-forming unit culture.

Low ESR (increased red cell count, reducing relative proportion of plasma and thus reducing sedimentation rate), high leukocyte ALP. Can have neutrophilia, thrombocytophilia too

Rx: aspirin, venesection, hydroxyurea, phosphorus-32 therapy.

5-15% progress to myelofibrosis. 5-15% progress to acute leukemia (increased risk with chemo)

101
Q

Essential thrombocytosis

A

JAK2 50%. CALR 20% of JAK2 -ves. MPL less than 10%.

Rx: hydroxyurea, interferon alpha, low dose aspirin

102
Q

Myelofibrosis

A

hyperplasia of abnormal megakaryocytes, release of platelet derived growth factor and fibroblast stimulation.

Lethargy common, massive splenomegaly.

Ix: Tear drop poikilocytes, dry top bone marrow (trephine biopsy needed), high urate, LDH

103
Q

Anaphylaxis post transfusion association

A

IgA deficiency → anti-IgA abs

104
Q

TRALI feature

A

ARDS within 6hrs, hypotension, fever, no periph oedema, normal JVP

105
Q

TACO features:

A

fluid overload with raised JVP

106
Q

TRALI vs TACO

A

TRALI - hypotensive, TACO - hypertensive

107
Q

Platelet transfusion indications

A

In active bleeding, give if <30. If severe bleeding/critical sites (eg CNS) give if <100

Prophylactic pre-procedure: <50 for most, <75 if high risk, <100 if critical

No bleeding, give if <10

108
Q

Platelet transfusion contraindication

A

chronic BM failure, autoimmune thrombocytopenia, heparin-induced thrombocytopenia, TTP

109
Q

What are platelet transfusions risks for

A

bacterial contamination as stored 20-24 degrees

110
Q

Investigative features for AIHA

A

direct antiglobulin test +ve. Spherocytes, reticulocytes, high LDH, low haptoglobin, anaemia.

111
Q

Warm AIHA features

A

IgG, extravascular haemolysis. idiopathic, autoimmune (SLE- can be mixed), lymphoma, CLL, drugs (methyldopa).

Rx: treat underlying, steroids +/- rituximab

112
Q

Cold AIHA features

A

IgM, intravascular haemolysis. Raynaud’s, acronyasis. Neoplasia (lymphoma) or infections (mycoplasma, EBV)

113
Q

Paroxysmal nocturnal haemoglobinuria mechanism

A

lack of glycoprotein glycosyl-phosphatidylinositol (GPI) →complement-regulating surface proteins (eg decay-accelerating factor) not bound to cell membrane → increased sensitivity of cell membranes to complement, with lack of CD59 → pltlt aggregation

114
Q

PNH features

A

Triad of intravascular haemolytic anaemia, pancytopenia, venous thrombosis (eg Budd-Chiari). Haemoglobinuria (dark urine in morning), aplastic anaemia in some.

115
Q

PNH how to diagnose

A

CD59,55 flow cytometry (Ham’s test causing acid-induced haemolysis is old).

116
Q

PNH Rx

A

supportive, anticoagulant, eculizumab (anti-C5) being trialled, stem cell transplant

117
Q

Coagulative changes in pregnancy

A

Increase in Factors 7, 8, 10, fibrinogen, reduced protein S

uterus pressing on IVC can risk DVT/PE - Warfarin contraindicated so go for SC LMWH

118
Q

Sideroblastic anaemia causes

A

Delta-aminolevulinate synthase-2 deficiency (congenital)
MDS
alcohol
lead
TB meds

119
Q

Sideroblastic anaemia ix:

A

microcytic anaemia with high ferritin, iron, transferrin sats

basophilic stippling, Prussian blue/Perl’s staining for bone marrow film showing sideroblasts (iron deposits in mitochondria).

120
Q

Rx for sideroblastic anaemia

A

supportive, pyridoxine

121
Q

Defect in lead poisoning

A

ferrochelatase, ALA dehydrogenase defect

122
Q

Lead poisoning features

A

abdo, neuro, blue gum lines

123
Q

Lead poisoning diagnosis

A

Blood lead level>10mcg/dl, microcytic anaemia, basophilic stippling, clover leaf morphology, serum + urine delta aminolaevulinic acid (also in AIT), urinary coproporphyrin

124
Q

Lead poisoning Rx

A

DMSA, D-penicillamine, EDTA, dimercaprol

125
Q

Acute Intermittent Porphyria defect

A

aut dom, prophobilinogen deaminase defect → rise in delta aminolaevulinic acid, porphobilinogen

126
Q

Features of AIT

A

Abdo + neuropsychiatric sx in 20-40yrs. F:M 5:1. Deep red urine on standing.

127
Q

AIT Ix and dx

A

aised urinary porphobilinogen, red cell assay for porphobilinogen deaminase, raised serum delta aminolaevulinic acid, porphobilinogen

128
Q

Drugs precipitating AIT

A

BOBAHS barbiturates, oral contraceptives, benzos, alcohol, halothane, sulphonamides

(safe to use: paracetamol, aspirin, codeine, morphine, chlorpromazine, beta-blockers, penicillin, metformin)

129
Q

Rx for AIT

A

IV haematin/haem arginate, IV glucose if not available

130
Q

Porphyria cutanea tarda:

A

uroporphyinogen decarboxylase def. Hepatic damage precipitates it. (HCV, alcohol)
Sx: Photosensitive rash with blistering and skin fragility on face + dorsal aspect of hands. Hypertrichosis, hyperpigmentation.

Ix: Elevated urinary uroporphyrinogen and pink fluorescence of urine under Wood’s lamp. serum ferritin guides Rx

Rx: chloroquine venesection if ferritin>600

131
Q

Variegate porphyria

A

Aut dom. Protoporphyrinogen oxidase def. South African

132
Q

List alkylating agents

A

Cyclophosphamide
Cisplatin

133
Q

What do alkylating agents do?

A

causes DNA crosslinking

134
Q

Cyclophosphamide SE:

A

bladder (haemorrhagic cystitis - can give Mesna (inactivates acrolein) to reduce risk - TCC), bone marrow (myelosuppression)

135
Q

Cisplatin SE:

A

ototoxicity
peripheral neuropathy
hypoMg (via renal toxicity and reduced Mg reabsorption in ascending Loop of Henle and DCT)

136
Q

List purine analogues

A

Methotrexate
6-mercaptopurine

137
Q

Methotrexate MoA

A

inhibits dihydrofolate reductase → blocks thymidylate synthesis.

138
Q

Methotrexate SE:

A

Lung (fibrosis), liver (fibrosis), bone (myelosuppression), mucositis

139
Q

6-mercaptopurine is activated by what?

A

HGPRTase

140
Q

SE of 6-mercaptopurine

A

myelosuppression

141
Q

Pyrmidine analogues

A

Fluorouracil
Cytarabine

142
Q

Fluorouracil MOA

A

blocks thymidylate synthase (works during S phase).

143
Q

Fluorouracil SE

A

bone (myelosuppression), skin (dermatitis), mucositis

144
Q

Relation of capecitabine to 5-fluorouracil

A

orally administered and converted to 5-fluorouracil in tumour.

145
Q

Cytarabine MOA

A

interferes with DNA synthesis at S phase phase, inhibits DNA polymerase.

146
Q

Cytarabine SE:

A

Myelosuppression, ataxia

147
Q

Vincristine, vinblastine MOA

A

inhibits microtubule formation

148
Q

SE of vincristine/vinblastine

A

vincristine - periph neuropathy (reversible), ileus.

Vinblastine: myelosuppression

149
Q

Taxanes (docetaxel) MOA:

A

Prevents microtubule dissassembly/depolymerisation

150
Q

Taxane SE

A

neutropenia

151
Q

Anthracycline MOA

A

stabilises topoisomerase II complex, inhibits DNA/RNA synthesis.

152
Q

Anthracycline SE:

A

CDM

153
Q

Irinotecan MOA

A

topoisomerase I inhibitor, prevents relaxation of supercoiled DNA.

154
Q

Indication for Irinotecan

A

Used as part of FOLFIRI regimen for colorectal Ca (with folinic acid + 5-fluorouracil)

155
Q

Irinotecan SE

A

myelosuppression

156
Q

Hydroxyurea MOA

A

inhibits ribonucleotide reductase, reducing DNA synthesis

157
Q

Hydroxyurea SE

A

myelosuppression

158
Q

Bleomycin MOA

A

degrades preformed DNA

159
Q

Bleomycin SE

A

Lung fibrosis

160
Q

features of SVC obstruction

A

SOB commonest, facial/neck/arm swelling with conjunctival + periorbital oedema, headache (worse in mornings), visual disturbances, pulseless JVP distension

161
Q

Causes of SVC obstruction

A

SCC, lymphoma, metastatic seminoma, Kaposi’s, breast Ca, aortic aneurysm, mediastinal fibrosis, goitre, SVC thrombosis.

162
Q

SVC obstruction Rx

A

endovascular stenting, radical chemo/radioRx

163
Q

Tumour lysis syndrome electrolytes/biochemistry

A

high uric acid, K, PO4, low Ca.

164
Q

Tumour lysis syndrome Rx

A

high risk- rasburicase (recombinant version of urate oxidase - metabolises uric acid to allantoin)

low risk - allopurinol

165
Q

Spinal cord compression management

A

urgent MRI, high dose oral dex, urgent onc r/v

166
Q

Commonest organism for neutropenic sepsis

A

G +ve coag -ve (s epidermidis)

167
Q

Methaemoglobinaemia - what happens

A

Fe2+ →Fe3+, cannot bind oxygen

dissociation curve left-shift

168
Q

Causes of methaemoglobinaemia

A

Congenital: HbM, HbH, NADH methaemoglobin reductase def

Acquired: sulphonamides, dapsone, nitrates, sodium nitroprusside, primaquine, aniline dyes

169
Q

Features of methaemoglobinaemia

A

Chocolate cyanosis, sob, anxiety, headache, acidosis, arrhythmias, seizures, comas, normal pO2, reduced sO2

170
Q

Rx of methaemoglobinaemia

A

acquired: IV methylthioninium chloride (methylene blue)

Ascorbic acid if congenital

171
Q

Neutrophil -based immunodeficiencies

A

chronic granulomatous diseasse
chediak-Higashi syndrome
Leukocyte adhesion deficiency

172
Q

Chronic granulomatous disease:

A

NADPH oxidase def
Pneumonias, abscesses (esp S aureus, fungi)

-ve nitroblue-tetrazolium test
abnormal dihydorhodamine flow cytometry test

173
Q

Chediak-Higashi syndrome:

A

Microtubule polymerization defect, reduced phagocytosis. Partial albinism in children, peripheral neuropathy. Recurrent bacterial inf, giant granules in neutrophils + platelet

174
Q

Leukocyte adhesion deficiency:

A

LFA-1 integrin (CD18) defect on neutrophils. Recurrent bacterial infections, delay in umbilical cord sloughing, absence of pus/neutrophils.

175
Q

B cell immunodeficiencies

A

CVID
Bruton’s X-linked congenital agammaglobulinaemia
Selective IgA def

176
Q

CVID

A

Common variable immunodeficiency: low abs (IgG, M, A), recurrent pneumonia, can predispose to autoimmune and lymphoma

177
Q

Bruton’s X linked congenital agammglobulinaemia

A

Bruton’s tyrosine kinase defect → severe B cell development block. X-recessive, recurrent bacterial infections, absent B-cells

178
Q

Selective IgA def

A

B cell maturation defect. Primary ab deficiency, recurrent sinus and respiratory infections. Coeliac association (False -ve coeliac). Anaphylaxis with transfusiosns

179
Q

DiGeorge’s syndrome

A

Aut Dom, (CATCH 22) Cardiac abnormalities (Tetralogy of Fallot, Truncus arteriosus), Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcaemia/hypoparathyroidism, 22q11.2 microdeletion. failure to develop 3rd, 4th pharyngeal pouches. LD. recurrent viral/fungal inf.

T cell immunodeficiency

180
Q

Combined B and T cell-based immunodeficiency

A

(SCID WAS ataxic + hyper IgM)

SCID
Wiskott-Aldrich
Ataxic telangiectasia
Hyper IgM syndrome

181
Q

SCID

A

Severe combined immunodeficiency (SCID): most common X-linked defect in common gamma chain, others include adenosine deaminase def. Recurrent inf, reduced T-cell receptor excision circles. Stem cell transplantation may be required

182
Q

Wiskott-Aldrich syndrome

A

WASP gene defect, X recessive, recurrent bacterial, eczema, low pltlts, autoimmune + malignancy risk. Low IgM

183
Q

Ataxic telangiectasia:

A

DNA repair enzyme defect, aut rec, cerebellar ataxia, telangiectasia (spider angiomas), recurrent chest infections (IgA def, combined B, T cell def), 10% risk lymphoma/leukaemias

184
Q

Hyper IgM syndrome

A

CD40 mutations. Infection/pneumocystis pneumonia, hepatitis, diarrhoea

185
Q

SERMs example and SEs

A

Tamoxifen (oestrogen rec antagonist/partial agonist)

SE: menstrual disturbance (vaginal bleeding, amenorrhoea) hot flushes, VTE, endometrial Ca (Raloxifene pure oestrogen receptor antagonist, lower risk for endomet ca)

186
Q

Aromatase inhibitor is best for which type of women?

A

post-menopausal

187
Q

Thymoma associations

A

myathenia, red cell aplasia, dermatomyositis, SLE, SIADH

188
Q

What causes death in thymomas

A

tamponade, airway obstruction

189
Q

megaloblastic macrocytic anaemia causes

A

Vit B12 def, folate def, methotrexate

190
Q

Normoblastic macrocytic anaemia causes

A

ETOH, liver, hypothyroid, pregnancy, reticulocytosis, myelodysplasia, cytotoxics

191
Q

Aplastic anaemia rx

A

supportive, anti-thymocyte globulin (ATG), anti-lymphocyte globulin (ALG), stem cell transplant

192
Q

Drug-induced pancytopenia causes

A

cytotoxics, trimethoprim, chloramphenicol, gold, penicillamine, carbimazole, carbamazepine, tolbutamide

193
Q

Cervical cancer pathophysiology

A

HPV 16, 18, 33 inhibits tumour suppressor gene p53 and RB.

koilocyte development (enlarged irregular nucleus with darker stains and perinuclear halo)

194
Q

Describe erythropoiesis:

A

in bone marrow, in flat bones + proximal ends of long bones, in foetal liver. Haematocytoblast (multipotent) → proerythroblast → basophilic erythroblast (ribosomes accumulate, nucleus shrinks) → polychromatophilic erythroblast → normoblast (nucleus ejected) → reticulocyte → erythrocyte.

195
Q

How long does erythropoiesis take

A

1 wk

196
Q

Lifespan of RBCs

A

120 days

197
Q

Aprepitan MOA

A

blocks neurokinin 1 receptor

198
Q

IgG4-related disease examples

A

Riedel’s thyroiditis, autoimmune pancreatitis, mediastinal and retroperitoneal fibrosis, periaortitis/periarteritis/inflammatory aortic aneurysm, Kuttner’s Tumour, Mikulicz syndrome, Sjogren’s, PBC

199
Q

What is skin prick testing good for

A

Food/pollen allergies (type 1 hypersensitivity). Tests a number of them.

Takes 15 mins

200
Q

What is RAST good for

A

determines IgE level specifically to suspected allergen, graded 0 to 6.

For food allergies, inhaled allergens, wasp/bee venom

201
Q

What is skin patch testing good for?

A

contact dermatitis (type IV)

30-40 allergens/irritants placed on back, removed 48hrs later

202
Q

Latex-fruit syndrome

A

Latex allergy associated with fruit allergies: banana, pineapple, avocado, chestnut, kiwi, mango, passion fruit, strawberry

203
Q

Pattern of tryptase level in anaphylaxis

A

peaks 1hr, returns to baseline 12-24hrs. Take 1st within 1hr, 2nd no later than 4hrs, 3rd more than 24hrs

204
Q

What to consider for risk stratified discharge for anaphylaxis

A

fast-track (2hrs) if single dose with good resolution, auto-injector training, support

6 hrs if 2 adrenaline or previous biphasic

12 hrs if severe, >2 adrenaline, severe asthma, ongoing reaction potential (slow-release medications), access issues (late night presentation, emergency access care difficult at home)

MRCP Revision (10 decks)

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