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MRCP > Haematology/ Oncology > Flashcards

Haematology/ Oncology Flashcards

1
Q

what is the most common thrombophilia?

A

Activated protein C resistance aka Factor V Leiden

  • heterozygotes - 5 x risk thrombosis
  • homozygotes - 50 x risk
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2
Q

MOA of docetaxel?

A

Acts on microtubules:

prevents microtubule depolymerisation & disassembly -> decreasing free tubulin

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3
Q

SE of docetaxel?

A

Neutropenia

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4
Q

What are the different types of cryoglobulinaemia?

A 
3 Types:
Type I (25%): monoclonal
Type II (25%): mixed monoclonal and polyclonal, usually with rheumatoid factor.
Type III (50%): polyclonal: usually with rheumatoid factor
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5
Q

What is cryoglobulinaemia?

A

immunoglobulins which undergo reversible precipitation at 4 deg, dissolve when warmed to 37 deg.

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6
Q

Type 1 Cryoglobulinaemia assoc?

A
  • monoclonal

- > Waldenstrom macroglobulinaemia, Multiple myeloma

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7
Q

Type II Cryoglobulinaemia assoc w?

A

mixed monoclonal/ polyclonal (usually with RF)

-> Hep C, Rheumatoid arthritis, Sjogren’s, lymphoma

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8
Q

Type III Cryoglobulinaemia assoc with?

A
  • polyclonal: usually with RF.

- > Rheumatoid arthritis, Sjogrens

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9
Q

Symptoms/ signs of Cryoglobulinaemia?

A
  • Raynauds (type I)
  • Cutaneous: purpura, distal ulceration
  • arthralgia
  • Renal - diffuse glomerulonephritis
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10
Q

Ix of Cryoglobulinaemia?

A
  • Low complement (esp C4)

- High ESR

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11
Q

Mx of cryoglobulinaemia?

A

Immunosuppression, plasmapheresis

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12
Q

Management of SVCO?

A
  • endovascular stenting usually to provide symptom relief

- often given steroids e.g. dexamethasone

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13
Q

most reliable lab test to screen for hereditary angioedema inbetween attacks?

A

C4 levels
- characteristically low

*C2 also low but serum C4 most reliable and widely used screening tool

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14
Q

best diagnostic test to send during acute attack of hereditary angioedema?

A

C1 inhibitor levels- low

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15
Q

Management of acute attack of hereditary angioedema?

A

IV C1-inhibitor concentrate,
2nd line: FFP

  • HAE does not respond to adrenaline, antihistamines or steroids.
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16
Q

Prophylaxis of attacks in hereditary angioedema?

A

anabolic steroid - Danazol

Or tranexamic acid

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17
Q

Diagnosis of hereditary spherocytosis?

A
  • typical clinical features (FHx) + lab Ix (spherocytes)

- if equivocal: EMA binding test and Cryohaemolysis test

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18
Q

Diagnosis of atypical presentations of hereditary spherocytosis?

A

electrophoresis analysis of erythrocyte membranes

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19
Q

Management of acute haemolytic crisis in hereditary spherocytosis?

A
  • supportive

- transfusion if necessary

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20
Q

Long term management of hereditary spherocytosis?

A

folate replacement, splenectomy

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21
Q

t(9;22) - Philadelphia chromosome

A
  • CML (present in >95%)

- poor prognostic indicator in ALL

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22
Q

t(15;17) - fusion of PML and RAR-alpha genes

A

Acute promyelocytic leukaemia

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23
Q

t(8;14) - MYC oncogene

A

Burkitt’s lymphoma

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24
Q

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

A

Mantle cell lymphoma

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25
Q

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

A

follicular lymphoma

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26
Q

causes of warm AIHA?

A
  • autoimmune disease: e.g. systemic lupus erythematosus*
  • neoplasia: e.g. lymphoma, CLL
  • drugs: e.g. methyldopa
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27
Q

Features of warm AIHA?

A
  • usually IgG antibody

- haemolysis at extravascular sites e.g. spleen

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28
Q

Management of warm AIHA?

A

steroids, immunosuppression, splenectomy

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29
Q

causes of cold AIHA?

A
  • neoplasia: e.g. lymphoma

- infections: e.g. mycoplasma, EBV

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30
Q

features of cold AIHA?

A
  • usually igM antibody
  • commonly intravascular haemolysis
  • Raynauds/ acrocynanosis
  • responds less well to steroids
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31
Q

clinical features of myelofibrosis?

A
  • symptoms of anaemia
  • massive splenomegaly
  • hyper metabolic symptoms: weight loss, night sweats
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32
Q

lab findings of myelofibrosis?

A
  • anaemia
  • high WBC, Pl count
  • blood film: tear drop poikilocytes
  • dry tap on BM biopsy-> trephine biopsy needed
  • high LDH and urate (increased cell turnover)
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33
Q

What chemical mediator is mainly responsible for the tissue oedema seen in patients in hereditary angioedema?

A

bradykinin

  • uncontrolled release of bradykinin-> oedema of tissues
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34
Q

features of thrombotic thrombocytopenic purpura?

A
  • fever
  • fluctuating neuro signs (microemboli)
  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • renal failure
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35
Q

pathogenesis of Thrombotic thrombocytopenic purpura?

A
  • deficiency of ADAMTS13 (a metalloprotease enzyme) that breaks down large multimers of vWF
  • abnormally large and sticky multimers of vWF cause platelets to clump within vessels
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36
Q

Causes of thrombotic thrombocytopenic purpura?

A

post-infection e.g. urinary, gastrointestinal
pregnancy
drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
tumours
SLE
HIV

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37
Q

features of Waldenstrom’s macroglobulinaemia?

A
  • monoclonal IgM paraproteinaemia
  • systemic upset: weight loss, lethargy
  • hyperviscosity syndrome e.g. visual disturbance
  • hepatosplenomegaly
  • lymphadenopathy
  • cryoglobulinaemia e.g. Raynaud’s
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38
Q

features of lead poisoning?

A
  • abdo pain
  • peripheral neuropathy (mainly motor)
  • fatigue
  • constipation
  • blue lines on gum margin (only 20% of adult patients, very rare in children)
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39
Q

Ix of lead poisoning?

A
  • Blood lead level
  • FBC: microcytic anaemia
  • blood film: basophilic stippling, clover leaf morphology
  • raised serum/ urine lvls of delta aminolaevulinic acid (similar to acute intermittent porphyria)
  • urinary coproporphyrin raised
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40
Q

management of lead poisoning?

A 
various chelating agents are currently used:
dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol
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41
Q

Sickle cell: what medication is taken long term to reduce incidence of complications and acute crises?

A

Hydroxycarbamide

  • increases HbF
  • makes cells less likely to sickle
  • advised to not be taken in pregnancy
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42
Q

features of essential thrombocytosis?

A
  • Pl count >600
  • thrombosis and haemorrhage
  • characteristic symptom is burning sensation in the hands
  • JAK2 mutation in 50%
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43
Q

Management of essential thrombocytosis?

A
  • Hydroxyurea (hydroxycarbamide) to reduce pl count
  • IFN-a in younger patients
  • low dose aspirin to reduce thrombotic risk
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44
Q

how to prevent haemorrhagic cystitis in those on cyclophosphamide?

A

Mesna (2-mercaptoethane sultanate sodium)

- binds to and neutralises acrolein

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45
Q

what type of cancer does cyclophosphamide increase risk of?

A

transitional cell carcinoma

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46
Q

a Leukaemoid reaction vs leukaemia causing high WBC

A
  • high leucocyte alkaline phosphatase score
  • toxic granulation (Dohle bodies) in the white cells
  • left shift of neutrophils
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47
Q

Blood film features of hyposplenism e.g. post-splenectomy, coeliac disease?

A 
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
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48
Q

1st line management of idiopathic thrombocytopenic purpura?

A

oral prednisolone

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49
Q

management of idiopathic thrombocytopenic purpura?

A

oral pred (80% of patients respond)

  • splenectomy if platelets < 30 after 3 months of steroid therapy
  • IV immunoglobulins
  • immunosuppressive drugs e.g. cyclophosphamide
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50
Q

features of acute intermittent porphyria?

A
  • abdominal: abdominal pain, vomiting
  • neurological: motor neuropathy
  • psychiatric: e.g. depression
  • hypertension and tachycardia common
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51
Q

genetics of acute intermittent porphyria?

A
  • autosomal dominant

- defect in porphobilinogen deaminase

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52
Q

what are the toxic elements accumulated in acute intermittent porphyria?

A

delta aminolaevulinic acid

porphobilinogen

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53
Q

diagnosis of acute intermittent porphyria?

A
  • urine: raised urinary porphobilinogen + urine turns deep red in sunlight
  • assay of red cells for porphobilinogen deaminase
  • raised serum levels of delta aminolaevulinic acid and porphobilinogen
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54
Q

lead poisoning: what is raised in urine?

A

coproporphyrin level

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55
Q

what drugs may precipitate haemolysis in G6PD deficiency?

A
  • anti-malarials: primaquine
  • ciprofloxacin
  • sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
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56
Q

pathophysiology of G6PD deficiency?

A

↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress

  • reduced glutathione protects RBCs from oxidative damage
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57
Q

Features of G6PD deficiency?

A
  • neonatal jaundice is often seen
  • intravascular haemolysis
  • gallstones are common
  • splenomegaly may be present
  • Heinz bodies on blood films. Bite and blister cells may also be seen
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58
Q

diagnosis of G6PD deficiency?

A

G6PD enzyme assay

- levels should be checked around 3 months after an acute episode of hemolysis to avoid false -ve result

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59
Q

IX of choice in CLL diagnosis?

A

Flow cytometry for immunophenotyping

- to reveal cell surface markers typical of CLL, including CD5, CD19 and CD20.

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60
Q

Defect in Chronic granulomatous disease?

A

lack of NADPH oxidase -> reduces ability of neutrophils to produce reactive oxygen species
-> susceptibility to catalase-positive bacteria e.g. staph aureus and fungi e.g. aspergillus

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61
Q

features of chronic granulomatous disease?

A

recurrent pneumonias and abscesses

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62
Q

diagnosis of chronic granulomatous disease?

A

abnormal dihydrorhodamine flow cytometry test

negative nitroblue-tetrazolium test

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63
Q

features of Chediak-Higashi syndrome?

A
  • partial albinism
  • peripheral neuropathy
  • recurrent bacterial infections
  • giant granules in neutrophils and platelets
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64
Q

defect in Chediak-Higashi syndrome

A

Microtubule polymerization defect which leads to a decrease in neutrophil phagocytosis

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65
Q

features of Leukocyte adhesion deficiency?

A

recurrent bacterial infections,
delay in umbilical sloughing,
absence of neutrophil/ pus at sites of infection

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66
Q

defect in Leukocyte Adhesion deficiency?

A

LAD1: deficiency of the (LFA-1) B-2 integrin subunit (CD18) of the Leukocyte adhesion molecule

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67
Q

Features of common variable immunodeficiency?

A

hypogammaglobulinaemia: Low IgG, IgE and IgA.
- FTT, recurrent infections, autoimmune and granulomatous diseases
- may predispose to lymphoma

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68
Q

Features of Bruton’s Agammaglobulinaemia?

A
  • X linked recessive
  • recurrent bacterial infections
  • failed maturation of B cells with low IG (No antibodies)
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69
Q

Genetic defect of Bruton’s Agammaglobulinaemia?

A

X-linked defect in Bruton’s tyrosine kinase gene -> block in B cell development

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70
Q

Features of Selective IgA deficiency

A
  • most common primary antibody deficiency
  • recurrent gastro/ resp infections
  • assoc w coeliac disease (may cause false -ve coeliac screen)
  • severe reactions to blood transfusions (anti-IgA abs-> anaphylaxis)
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71
Q

Features of DiGeorge’s Syndrome?

A

CATCH - 22
Congenital heart disease - tetralogy of fallot
Abnormal facies - low set ears, cleft lip
Thymus - absent, low lvls of mature T cells -> recurrent viral/ fungal infections
Cleft palate
Hypocalcaemia

22q11.2 deletion

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72
Q

Defect in Wiskott-Aldrich syndrome?

A

Defect in WASP gene, X linked recessive

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73
Q

Features of Wiskott-Aldrich syndrome?

A

X linked recessive

  • recurrent bacterial infections
  • eczema
  • thrombocytopenia
  • Low IgM levels
  • Increased risk of autoimmune disorders/ malignancy
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74
Q

CLL: Indications for treatment

A
  • progressive marrow failure
  • 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
  • FLAWS: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats
  • autoimmune cytopaenias e.g. ITP
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75
Q

CLL: Management if treatment is indicated

A
  • fludarabine, cyclophosphamide and rituximab (FCR)

- Ibrutinib (2nd line)

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76
Q

1st line management of thrombotic thrombocytopenic purpura?

A

plasma exchange

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77
Q

Microscopy findings of Burkitt’s lymphoma?

A

‘starry sky’ appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

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78
Q

Mx of Burkitt’s lymphoma?

A

chemotherapy

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79
Q

what medication is given before chemotherapy to reduce risk of tumour lysis syndrome?

A

rasburicase (a recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin*)

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80
Q

what medication is given before chemotherapy to reduce risk of tumour lysis syndrome?

A

rasburicase (a recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin, which is 5-10x more soluble than uric acid)

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81
Q

Haptoglobin levels in haemolytic anaemia?

A
  • LOW haptoglobin levels

Haptoglobin binds to free haemoglobin released from lysed erythrocytes. The complexes are then removed from the plasma by the hepatic reticulo-endothelial cells. Haptoglobin levels decrease if the rate of haemolysis is greater than the rate of haptoglobin production.

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82
Q

rare drug cause of B12 deficiency?

A

Metformin

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83
Q

MOA of cyclophosphamide?

A

alkylating agent- causes cross linking in DNA

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84
Q

gold standard investigation in Paroxysmal nocturnal haemoglobinuria?

A

flow cytometry of blood to detect low levels of CD59 and CD55
- has now replaced Ham’s test

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85
Q

features of Paroxysmal nocturnal haemoglobinuria?

A
  • haemolytic anaemia +/- aplastic anaemia
  • pancytopaenia may be present
  • haemoglobinuria: classically dark-coloured urine in the morning (can occur throughout the day)
  • thrombosis e.g. Budd-Chiari syndrome
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86
Q

Management of Paroxysmal nocturnal haemoglobinuria?

A
  • blood product replacement
  • anticoagulation
  • eculizumab, a monoclonal antibody directed against terminal protein C5, being trialled
  • stem cell transplantation
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87
Q

Cisplatin: what electrolyte abnormality as SE?

A

Hypomagnesaemia

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88
Q

Chemotherapy related N+V?

A

low risk - metoclopramide 1st line

high risk - 5HT3R antagonists eg. ondansetron +/- dexamethasone

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89
Q

Aflatoxin (produced by Aspergillus) - increased risk of which ca?

A

HCC

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90
Q

Aniline dyes - increased risk of which ca?

A

Bladder (Transitional cell ca)

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91
Q

Asbestos - increased risk of which ca?

A

Mestothelioma, bronchial ca

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92
Q

Nitrosamines - increased risk of which ca?

A

Oesophageal and gastric ca

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93
Q

Vinyl chloride - increased risk of which ca?

A

Hepatic angiosarcoma

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94
Q

Ix in multiple myeloma?

A
  • monoclonal proteins in serum and urine (bence jones proteins)
  • Whole body MRI
  • XR: “rain drop skull”
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95
Q

Diagnostic criteria for multiple myeloma?

A

1 major, 1 minor OR 3 minor

Major criteria

  • Plasmacytoma
  • 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.
  • Low levels of antibodies (not produced by the cancer cells) in the blood.
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96
Q

MX of antiphospholipid syndrome in pregnancy?

A

aspirin + LMWH (until 34/40 gestation)

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97
Q

metastatic spinal cord compression: UMN or LMN?

A

Lesions above L1 usually result in upper motor neuron signs in the legs and a sensory level. Lesions below L1 usually cause lower motor neuron signs in the legs and perianal numbness. Tendon reflexes tend to be increased below the level of the lesion and absent at the level of the lesion

98
Q

Ix of metastatic spinal cord compression?

A

urgent MRI: MRI whole spine within 24h of presentation

99
Q

Mx of metastatic spinal cord compression?

A

high dose oral dexamethasone

- urgent onco assessment for consideration of radiatx or surgery

100
Q

clinically cryoprecipitate is most commonly used to replace?

A

fibrinogen

101
Q

Desmopressin- in von Hillebrands disease?

A

induces release of von Willebrand’s factor from endothelial cells

102
Q

SE of cisplatin?

A

Ototoxicity, peripheral neuropathy, hypomagnesaemia

103
Q

Anastrozole/ letrozole -MOA?

A

aromatase inhibitors that reduces peripheral oestrogen synthesis
- used for ER+ve breast ca

104
Q

SE of anastrozole/ letrozole?

A

osteoporosis: DEXA scan when initiating pt
- hot flushes
- arthralgia, myalgia
- insomnia

105
Q

SEs of Tamoxifen (SERM)?

A
  • menstrual disturbance: vaginal bleeding, amenorrhoea
  • hot flushes
  • venous thromboembolism
  • endometrial cancer
106
Q

Management of polycythaemia vera?

A
  • aspirin - reduces risk of thrombotic events
  • 1st line: venesection
  • chemotherapy: hydroxyurea, phosphorus-32 therapy
107
Q

What may polycythaemia vera progress to …?

A

myelofibrosis, AML (risk increased w hydroxyurea)

108
Q

immune thrombocytopenia: what are antibodies directed against?

A

gpIIb/IIIa or Ib-V-IX complex

109
Q

Hereditary angioedema - what is the best screening test inbetween attacks?

A

c4 levels - low

110
Q

Management of choice in thrombotic thrombocytopenic purpura ?

A

Plasma exchange

111
Q

Management overview of TTP?

A

no antibiotics - may worsen outcome
plasma exchange is the treatment of choice
steroids, immunosuppressants
vincristine

112
Q

most common tumour of the anterior mediastinum?

113
Q

Thymoma associations?

A

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

114
Q

Thymoma leading to death…? causes

A

compression of airway

cardiac tamponade

115
Q

management of hereditary angioedema?

A

IV C1-inhibitor concentrate- otherwise, FFP.

  • Note adrenaline/ antihistamines/ steroids not useful!
116
Q

prophylaxis of angioedema attacks in hereditary angioedema?

A

anabolic steroid (Danazol)

117
Q

Hodgkin’s lymphoma: most common subtype?

A

Nodular sclerosing (around 70%).

followed by mixed cellularity (~20%), lymphocyte predominant (~5%), lymphocyte depleted (rare)

118
Q

Hodgkin’s lymphoma: subtype with best prognosis?

A

Lymphocyte predominant

119
Q

Hodgkin’s lymphoma: subtype with worst prognosis?

A

Lymphocyte depleted

120
Q

Hodgkin’s lymphoma: subtype associated with lacunar cells?

A

Nodular sclerosing

121
Q

Hodgkin’s lymphoma: subtype associated with large number of Reed-Sternberg cells?

A

Mixed cellularity

122
Q

Hodgkin’s lymphoma: what factors are associated with poor prognosis?

A
  • B symptoms: fever >38, night sweats, weight loss >10% in last 6 months
  • male
  • low albumin, Hb (<10.5), lymphocyte count (<8%)
  • age >45, WCC >15
  • stage IV disease
123
Q

Essential thrombocytosis: what gene mutations are assoc?

A

mainly JAK-2.

in ~20% of JAK-2 negative patients: CALR (calreticulin) gene mutation

124
Q

Cisplatin MOA?

A

cross-linking in DNA

125
Q

Cisplatin SE?

A

Ototoxicity, peripheral neuropathy, hypoMg

126
Q

Hydroxyurea/ Hydroxycarbamide MOA?

A

inhibits ribonucleotide reductase -> Decreasing DNA synthesis

127
Q

Hydroxyurea/ Hydroxycarbamide SE?

A

myelosuppression

128
Q

Myeloma: what marker is useful for prognostication?

A

B2-microglobulin - useful, poor prognosis if raised.

Low albumin -poor prognosis

129
Q

Most common tumour causing bone mets?

A

Prostate

followed by breast then lung

130
Q

Most common site of bone met

A

spine

then pelvis
ribs
skull
long bones

131
Q

Poor prognostic features of AML?

A
  • > 60 yo
  • > 20% blasts after first course of chemo
  • cytogenetics: deletions of chr 5 or chr 7
132
Q

Associations of Acute promyelocytic leukaemia?

A
  • t(15;17)
  • Auer rods (seen with myeloperoxidase stain)
  • DIC/ thrombocytopenia
  • good prognosis, presents younger than other types of AML (~25yo)
133
Q

Genetics of beta thal major?

A
  • chr 11

- absence of beta chains

134
Q

Features of beta thal major?

A
  • HbA2 + HbF raised
  • microcytic anaemia
  • HbA absent
135
Q

Mx of beta thal major?

A

repeated transfusion -> iron overload

- s/c infusion of desferrioxamine

136
Q

Inheritance of antithrombin III deficiency?

A

autosomal dominant

137
Q

Features of antithrombin III deficiency?

A
  • recurrent venous thromboses
  • arterial thromboses may occur
  • deficiency of antithrombin III -> heparin does not work
138
Q

Management of antithrombin III deficiency?

A

lifelong warfarinisation

  • heparinisation during pregnancy
  • antithrombin III concentrates (often used during surgery/ childbirth)
139
Q

Ix of immune thrombocytopenic purpura?

A
  • antiplatelet antibodies

- bone marrow aspiration: megakaryocytes. (should be performed prior to steroid initiation to rule out leukaemia)

140
Q

Mx of immune thrombocytopenic purpura?

A

1st line: oral prednisolone

  • splenectomy if Pl<30 after 3 mo of steroid therapy
  • IVIG
  • Immunosuppressive drugs: e.g. cyclophosphamide
141
Q

latex allergy more common in which condition?

A

children w myelomeningocele spina bifida

142
Q

Latex-fruit syndrome: what fruits are implicated?

A

those allergic to latex also allergic to fruits: particularly banana, pineapple, avocado, kiwi, chestnut, mango, passion fruit, strawberry

143
Q

SVC obstruction - what is the most common symptom?

144
Q

Irradiated blood products are depleted of what? and why are they used?

A

depleted of T lymphocytes

- used to avoid transfusion assoc graft vs host disease caused by viable donor T lymphocytes

145
Q

features of Porphyria cutanea tarda (PCT)?

A
  • may be caused by hepatocyte damage e.g. alcohol, oestrogens
  • classically photosensitive rash with bullae, skin fragility on face and dorsal aspect of hands
146
Q

Defect in Porphyria cutanea tarda (PCT)?

A

defect in uroporphyrinogen decarboxylase

147
Q

management of Porphyria cutanea tarda?

A

chloroquine

148
Q

Ix of Porphyria cutanea tarda?

A

urine: elevated uroporphyrinogen and pink fluorescence of urine under Wood’s lamp

149
Q

Features of Variegate porphyria?

A
  • auto dominant
  • photosensitive blistering rash
  • abdo and neurological symptoms
    more common in South Africans
150
Q

Enzyme affected in variegate porphyria?

A

defect in protoporphyrinogen oxidase

151
Q

pathophysiology of methaemoglobinaemia?

A

Hb: oxidised from Fe2+ to Fe3+.

  • normally regulated by NADH methaemoglobin reductase
  • tissue hypoxia as Fe3+ cannot bind oxygen, and hence the oxidation dissociation curve is moved to the left
152
Q

Causes of methaemoglobinaemia?

A

Congenital causes:

  • Hb chain variants: HbM, HbH
  • NADH methaemoglobin reductase deficiency

Acquired causes

  • drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite ‘poppers’), dapsone, sodium nitroprusside, primaquine
  • chemicals: aniline dyes
153
Q

Features of methaemoglobinaemia?

A
  • ‘chocolate’ cyanosis (chocolate-colored blood)
  • dyspnoea, anxiety, headache
  • severe: acidosis, arrhythmias, seizures, coma
  • normal pO2 on gas but decreased oxygen saturation
154
Q

Mx of acquired methaemoglobinaemia?

A

IV methylene blue

155
Q

Mx of congenital methaemoglobinaemia secondary to NADH - methaemoglobinaemia reductase deficiency?

A

ascorbic acid (Vitamin C)

156
Q

Differences between transfusion related lung injury (TRALI) and transfusion associated circulatory overload (TACO)

A

TACO:- usually rapid transfusions/ more units
- assoc w HYPERtension

TRALI:

  • assoc w HYPOtension
  • assoc w fever, low WCC
157
Q

Mx of Non-haemolytic febrile reaction post- blood transfusion?

A

slow/ stop the transfusion, paracetamol, monitor.

158
Q

mx of Minor allergic reaction post blood transfusion e.g. urticaria, pruritus?

A

Temporarily stop the transfusion, Antihistamine

Monitor.

159
Q

Mx of anaphylaxis post blood transfusion? e.g. Hypotension, dyspnoea, wheezing, angioedema

ie. in IgA deficiency

A

Stop transfusion

*IM adrenaline

ABC: oxygen, fluids

160
Q

Mx of Acute haemolytic reaction post blood transfusion (ie. ABO incompatibility)?
Fever, abdominal pain, hypotension

A

Stop transfusion.

  • Confirm diagnosis: send blood for direct Coombs test, repeat typing and cross-matching
  • Supportive: fluid resuscitation
161
Q

Mx of TACO post blood transfusion?

A

Slow/ stop tranfusion. +/- IV furosemide

162
Q

Mx of TRALI post blood transfusion?

A

stop transfusion, supportive. give O2

163
Q

Features of CML?

A

anaemia, lethargy, weight loss, sweating common

  • splenomegaly -> abdo discomfort
  • an increase in granulocytes at different stages of maturation +/- thrombocytosis
  • decreased leukocyte alkaline phosphatase
  • may undergo blast transformation (->AML in 80%, ALL in 20%)
164
Q

1st line mx of CML?

A

imatinib

  • inhibitor of the tyrosine kinase associated with the BCR-ABL defect
  • very high response rate in chronic phase CML
165
Q

Mx of CML?

A

Imatinib,
Hydroxyurea,
IFN-alpha
Allogenic Bone marrow transplant

166
Q

ESR in polycythaemia vera?

167
Q

Diagnostic criteria for Polycythaemia vera?

A
  • JAK2 mutation (present in 95%) -> 1st line

- High Hct / raised red cell mass

168
Q

Other lab features of polycythaemia vera?

A

Low ESR, high leukocyte alkaline phosphatase

169
Q

what type of lymphoma is assoc w increased BCL-2 transcription?

A

Follicular lymphoma

- t(14;18)

170
Q

Peripheral neuropathy + urinary hesitancy / bladder atony - Which chemotherapy drug SE?

A

vincristine

171
Q

diverse organ manifestations linked by similar histological characteristics:
e.g. riddle’s thyroiditis, autoimmune pancreatitis, mediastinal/ retroperitoneal fibrosis, periaortitis, Kuttner’s tumour (submandibular glands) + Mikulicz syndrome (salivary, lacrimal glands), sjogrens/ primary biliary cirrhosis

A

IgG4-related disease

172
Q

BRCA 1/2 associations?

A

Breast cancer (60% risk)

  • Ovarian Ca (55% w BRCA 1, 25% with BRCA 2)
  • Prostate Ca in men (BRCA 2)
173
Q

Which chromosomes are BRCA 1 and 2 on?

A

BRCA 1 = Chr 17

BRCA 2 = Chr 13

174
Q

Features of Li-Fraumeni Syndrome?

A
  • auto dominant
  • sarcomas and leukaemias
  • sarcoma <45yo or relative w cancer <45yo + another family member w cancer <45yo/ sarcoma at any age
175
Q

Genetics of Li-Fraumeni Syndrome?

A
  • auto dom

- mutations to p53 tumour suppressor

176
Q

Family history of cancers + sarcomas

A

Li-Fraumeni syndrome

177
Q

Features of Lynch syndrome?

A
  • auto dominant
  • Colon ca + endometrial cancer
  • Amsterdam criteria shows high risk individuals: 3 or > family members with colorectal cancer in 2 generations. (FAP excluded, one must be diagnosed under age 50)
178
Q

Features of Gardners syndrome?

A
  • auto dominant
  • colorectal polyps -> risk of colorectal ca
  • assoc skull osteoma, thyroid ca, epidermoid cysts
  • Desmoid tumours
179
Q

genetics of Gardners syndrome?

A

auto dom, mutation of APC gene on chr 5

180
Q

What extra colonic features are seen in Gardners syndrome?

A

skull osteoma,
thyroid ca,
epidermoid cysts/ dermoid tumours

181
Q

disproportionately low MCV microcytic anaemia, with high ferritin

A

Beta thal trait

182
Q

Good prognostic factors of ALL?

A
  • French-American-British (FAB) L1 type
  • common ALL
  • pre-B phenotype
  • low initial WBC
  • del(9p)
183
Q

Poor prognostic factors of ALL?

A
  • FAB L3 type
  • T or B cell surface markers
  • t(9;22), Philadelphia translocation
  • age <2y or >10y
  • male
  • CNS involvement
  • High initial WBC (>100 x10^9)
  • Non caucasian
184
Q

tumour marker most associated with primary peritoneal cancer

185
Q

AFP - what cancers?

A

HCC, teratoma

186
Q

S-100 - what cancers?

A

melanoma, schwannomas

187
Q

Bombesin- what cancers?

A

small cell lung ca, gastric ca, neuroblastoma

188
Q

Raynauds - assoc w which type of cryoglobulinaemia?

189
Q

Cryoglobulinaemia - mixed IgM and IgG

190
Q

Reed-Sternberg cell

A

Hodgkins lymphoma

191
Q

Ann-arbor staging of Hodgkins lymphoma?

A

I: single lymph node
II: 2 or more lymph nodes/regions on same side of diaphragm
III: nodes on both sides of diaphragm
IV: spread beyond lymph nodes

A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)
192
Q

Haptoglobin levels during a haemolytic crisis?

A

accelerated break down of red blood cells releases haemoglobin which haptoglobin binds to –> reducing the levels of haptoglobin in the blood.

193
Q

e.g.s of G-CSF

A

filgrastim

perfilgrastim

194
Q

When should G-CSF be considered?

A

those at high risk of neutropenia (>20%):

  • the elderly
  • specific malignancies e.g. Non-Hodgkins lymphoma, ALL
  • Prev neutropenic episodes
  • Receiving combination chemo and radiotx

*NOTE G-CSF not recommended for myeloid malignancies as may stimulate some cancers

195
Q

How does HPV cause cervical cancer?

A

HPV 16 and 18 triggers carcinogenesis by inhibiting the tumor suppressor gene p53 and RB.

196
Q

MGUS: Differentiating features from myeloma?

A
  • 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)
197
Q

ix for suspected spinal mets?

A

with neurological features: urgent MRI whole spine

Without neurological features: whole spine MRI within 1 week

198
Q

what cancers are assoc w EBV?

A

Hodgkins, Burkitts lymphoma, nasopharyngeal ca

199
Q

what cancers are assoc w H pylori?

A

gastric lymphoma (MALT)

200
Q

what cancers are assoc w malaria?

A

Burkitt’s lymphoma

201
Q

Management of tumour lysis syndrome?

A

IV allopurinol OR IV rasburicase + IV fluids

202
Q

management of smouldering multiple myeloma?

A

watch and wait

203
Q

skin prick test - can be interpreted after how long?

A

after 15 minutes

204
Q

Aprepitant - what is this medication?

A

anti-emetic, which blocks the Neurokinin 1 (NK1 receptor. used in chemotherapy induced n+V

205
Q

Poor prognostic factors of CLL?

A
  • 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
  • *deletion of 17p
206
Q

What chromosomal changes is assoc w good prognosis of CLL?

A

deletion of long arm of chromosome 13 (del 13q)

- seen in ~50%

207
Q

What chromosomal changes is assoc w poor prognosis of CLL?

A

deletions of short arm of chromosome 17 (del 17p) seen in 5-10%

208
Q

PET scan: looks at…?

A

glucose uptake

209
Q

Causes of sideroblastic anaemia?

A

acquired:

  • myelodysplasia
  • alcohol
  • lead
  • anti TB medications

congenital: delta-aminolevulinate synthase-2 deficiency

210
Q

Blood film findings of sideroblastic anaemia?

A

basophilic stippling of RBCs

211
Q

bone marrow findings of sideroblastic anaemia?

A

Prussian blue stain: ringed sideroblasts

212
Q

management of sideroblastic anaemia?

A

supportive, tx underlying cause

- pyridoxine may help

213
Q

most common cause of neutropenic sepsis?

A

staphylococcus epidermidis

- gram +v cocci

214
Q

features of transfusion associated graft vs host disease?

A
  • 2-6 wks post transfusion
  • diarrhoea, liver damage, rash
  • pancytopenia
215
Q

diagnosis of transfusion assoc graft vs host disease?

A

biopsy of skin - abundant necrotic keratinocytes
biopsy of bone marrow- marked hypocellularity with macrophage infiltration

  • both can be diagnostic
216
Q

what is IFN-a used in the management of?

A

hepatitis B & C, Kaposi’s sarcoma, metastatic renal cell cancer and hairy cell leukaemia

217
Q

What is hairy cell leukaemia?

A

rare malignant proliferation disorder of B cells

- more common in males (4:1)

218
Q

Features of hairy cell leukaemia?

A

pancytopenia, splenomegaly.

  • skin vasculitis in 1/3 patients
  • ‘dry tap’ despite BM hypercellularity
  • Tartrate resistant acid phsophatase (TRAP) stain positive
219
Q

Management of hairy cell leukaemia?

A

1st line: chemotherapy - cladribine, pentostatin

2nd line: immunotherapy - rituximab, IFN-alpha

220
Q

what condition is associated with skin necrosis after the commencement of warfarin?

A

Protein C deficiency

  • when warfarin is first started, biosynthesis of protein C is reduced -> temporary procoagulant state, usually avoided by concurrent heparin administration.
  • thrombosis in venules-> skin necrosis
221
Q

what chromosome are alpha-globulin genes located on?

alpha thalassaemia

A

chromosome 16

222
Q

key initial therapy in Acute promyelocytic leukaemia (APML)?

A

ATRA - all-trans retinoid acid +/- anthracycline (daunorubicin/ idarubicin)

  • causes immature promyelocytes to mature, alleviating the symptoms of DIC.
223
Q

management of alteplase OD/ related haemorrhage?

A

Tranexamic acid

- at high concentrations, non competitive inhibitor of plasmin

224
Q

management of chronic lead poisoning?

A

oral DMSA

- 500mg twice per day

225
Q

above what levels is suggestive of lead poisoning?

A

lead level above 3.4 µmol/l

226
Q

management of acute lead poisoning?

A

Activated charcoal

IV disodium EDTA

227
Q

what test to use to estimate glucose control if HbA1c is unreliable because of disturbed erythrocyte turnover or abnormal haemoglobin type?

A
  • quality-controlled plasma glucose profiles
  • total glycated haemoglobin estimation (if abnormal haemoglobins)
  • fructosamine estimation’
228
Q

Hyper IgM syndrome causes

A

high IgM, low IgG and IgA

  • due to defective class switching
  • most common form is an X-linked recessive trait due to mutation of CD40LG gene affecting roughly 2/1,000,000 males
229
Q

what infections are common in hyper IgM syndrome?

A
  • often presents with recurrent infections
  • PCP pneumonia
  • cryptosporidium
  • CMV, and may lead to liver disease

also associated with increased risk of cancers (hepatocellular carcinoma, cholangiocarcinoma and neuroectodermal tumours)

230
Q

management of acute intermittent porphyria in acute attack?

A

IV haematin/haem arginate

- IV glucose should be used if haematin/haem arginate is not immediately available

231
Q

What medication should be given alongside fludarabine, cyclophosphamide and rituximab (FCR) in CLL?

A

Co trimoxazole

- In view of fludarabine causing profound lymphopenia and increasing risk of opportunistic infections (PCP)

232
Q

What degree of lymphocytosis in CLL suggests need for management?

A

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

233
Q

Prophylaxis for tumour lysis syndrome?

A

Ensure good renal perfusion

  • IV fluids prior to starting chemotherapy
  • stop NSAIDs
  • prophylactic IV rasburicase OR allopurinol to prevent uric acid accumulation (don’t give together as will reduce effect of rasburicase)
234
Q

What scoring system is used for classification of tumour lysis syndrome?

A

Cairo-Bishop scoring system:

Laboratory TLS

2 or more of: occurring within three days before or seven days after chemotherapy.
uric acid > 475umol/l or 25% increase
potassium > 6 mmol/l or 25% increase
phosphate > 1.125mmol/l or 25% increase
calcium < 1.75mmol/l or 25% decrease

Clinical TLS

laboratory tumour lysis syndrome plus one or more of the following:

  • increased serum creatinine (1.5 times upper limit of normal)
  • cardiac arrhythmia or sudden death
  • seizure
235
Q

Eculizumab - what vaccination should be prescribed to patients on this?

A

meningococcal strains A, C, W, and Y

- due to eculizumab binding to C5

236
Q

Fastest way to increase platelet count in immune thrombocytopenia?

A

IVIG

Faster than steroids

237
Q

Hodgkin’s lymphoma (past/ present): what type of blood products should they receive?

A

Irradiated
- otherwise donor T cells might cause transfusion associated Graft vs host disease where donor T lymphocytes react against pt’s lymphoid tissue causing tissue / organ damage

238
Q

Indications for gamma irradiated blood products?

A
  • immunocompromised marrow or organ transplant recipients
  • pts w haem disorders due to undergo allogeneic marrow transplantation imminently
  • intrauterine transfusions
  • Hodgkin’s lymphoma
  • pts treated w purine analogue drugs (E.g. fludarabine)
239
Q

isolated prolonged APTT, reduced FvIII levels, evidence of fVIII inhibitor activity?

A

Acquired haemophilia A

  • autoimmune disorder with formation of abs attacking clotting factors
  • mixing studies: coagulation test remains abnormal due to presence of factor inhibitor. in inherited disorders w factor deficiency, addition of normal plasma restores clotting parameters
240
Q

most useful agent in preventing the delayed phase of chemotherapy-induced emesis

A

Dexamethasone

MRCP (21 decks)

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