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Haematology > Passmedicine > Flashcards

Passmedicine Flashcards

1
Q

What is multiple myeloma a neoplasm of?

A

Bone marrow plasma cells

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2
Q

What age group does multiple myeloma mostly affect?

A

60-70 year olds

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3
Q

What are the clinical features of MM?

A

Remember using mnemonic CRABBI -

C = Calcium - hypercalcaemia due to increased osteoclast activity

R = Renal - monoclonal production of Ig –> light chain deposition in renal tubules (presents as dehydration + increasing thirst), other causes of renal impairment - amyloidosis, nephrocalcinosis, nephrolithiasis

A = anaemia (due to bone marrow crowding –> suppression of erythropoiesis) –> fatigue, pallor

B = bleeding (bone marrow crowding –> thrombocytopenia –> inc. risk bleeding/bruising)

B = bones (bone marrow infiltration by plasma cells + cytokine mediated osteoclast overactivity –> lytic bone lesions) –> pain + increased risk of fragility fractures

I = infection (a reduction in production of normal Ig –> susceptibility to infection)

Others - hyperviscosity

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4
Q

What is the most common type of non-hodgkins lymphoma in the UK?

A

Diffuse large B cell lymphoma

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5
Q

Is diffuse large B cell high grade or low grade and what does this mean?

A

It is high grade, i.e. aggressive

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6
Q

Who does Burkitt lymphoma tend to affect?

A

Immunocompromised or young patients

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7
Q

What is the typical presentation of follicular lymphoma?

A

It is relatively common but low grade and therefore very slow growing

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8
Q

How does cutaneous T cell lymphoma present?

A

With skin changes

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9
Q

Define lymphoma

A

Malignant proliferation of lymphocytes in lymph nodes/other organs

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10
Q

What are the two broad categories of lymphoma

A

Hodgkins

Non-hodgkins

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11
Q

What is Hodgkins lymphoma?

A

Specific type of lymphoma characterised by Reed-Sternberg cells

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12
Q

How can you further classify non-hodgkins lymphoma?

A

By whether it affects B or T cells

By whether it is high or low grade

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13
Q

True or false:

Hodgkins lymphoma is more common than non-hodgkins lymphoma

A

False

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14
Q

Who does non-hodgkins lymphoma tend to affect?

A 
Older people (>75 especially)
Slightly more common in men
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15
Q

What are risk factors for developing a non-hodgkins lymphoma?

A 
Elderly
Caucasian
History of viral infections
FH
Chemical agents, e.g. pesticides, solvents
Hx of chemo/radiotherapy
Immunodeficiency (HIV, transplant, DM)
Autoimmune dx (SLE, Sjogren's, coeliac dx)
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16
Q

What virus is associated with non-hodgkins lymphoma?

A

EBV

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17
Q

What are the symptoms of non-hodgkins lymphoma?

A 
Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
Constitutional B symptoms (fever, wt loss, night sweat, lethargy)
Extranodal disease (gastric - dyspepsia, dysphagia, wt loss, ab pain, bone marrow - pancytopenia, bone pain, lungs, skin, CNS (nerve palsies))
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18
Q

What clinical signs may indicate one of hodgkins or non-hodgkins lymphoma is more likely than the other?

A

Lymphadenopathy in HL can experience alcohol induced pain in the node

B symptoms typically occur earlier in HL

Extra-nodal disease more common in NHL

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19
Q

What are the signs of non-hodgkins lymphoma?

A

Wt loss
Lymphadenopathy (esp. cervical, axillary, inguinal)
Palpable abdominal mass - hepatomegaly, splenomegaly or LNs
Testicular mass
Fever

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20
Q

What is the diagnostic investigation of choice in non-hodgkins lymphoma?

A

Excisional node biopsy

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21
Q

What other investigations should you do in non-hodgkins lymphoma and why?

A

CT chest, abdo, pelvis (staging)
HIV test (risk factor)
FBC + blood film (check for normocytic anaemia which would rule out other haematological malignancy e.g. leukaemia)
ESR (prognostic indicator)
LDH (marker of cell turnover, prognostic indicator)
Others -
LFTs if liver mets suspected, PET CT or bone marrow biopsy to look for bone involvement, LP in neuro involvement)

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22
Q

What is the name of the staging system used in non-hodgkins lymphoma?

A

Ann Arbor

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23
Q

What is the Ann Arbor classification for staging non-hodgkins lymphoma?

A

Stage 1: 1 node affected
Stage 2: >1 node affected on same side of diaphragm
Stage 3: 1 node affected on either side of diaphragm
Stage 4: extranodal involvement, e.g. spleen, bone marrow, CNS

Stage combined with A/B
A = no B symptoms
B = B symptoms

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24
Q

What is the management of non-hodgkins lymphoma?

A

Depends on subtype
Involves watchful waiting, chemo or radio

Patients will get flu/pneumococcal vaccines

Those with neutropenia may req. prophylactic antibiotics

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25
Q

What are the possible complications of non-hodgkins lymphoma?

A

Bone marrow infiltration –> anaemia, neutropenia, thrombocytopenia
SVC obstruction
Mets
Spinal cord compression
Complications related to Rx, e.g. SEs of chemo

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26
Q

What is the appearance of the neutrophils in a megaloblastic anaemia?

A

Hypersegmented neutrophils

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27
Q

What do you see on blood film in an intravascular haemolysis?

A

Schistocytes

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28
Q

What do you see on blood film in myelofibrosis?

A

Tear drop poikilocytes

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29
Q

What do you see on blood film in iron deficient anaemia?

A

Target cells

Pencil poikilocytes

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30
Q

What do you see on blood film in hyposplenism?

A 
Target cells
Howell-Jolly bodies
Pappenheimer bodies
Siderotic granules
Acanthocytes
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31
Q

What test is most likely to confirm the diagnosis of megaloblastic anaemia?

A

Haematinics (B12 and folate)

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32
Q

What are the crises than can occur in sickle cell disease?

A 
Thrombotic (painful crises)
Sequestration 
Acute chest syndrome
Aplastic
Haemolytic
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33
Q

What are thrombotic crises? (sickle cell disease)

A

Aka. painful/vaso-occlusive crises
Precipitated by infection, dehydration deoxygenation
Infarcts occur in various organs + bones (AVN of hip, hand foot syndrome in children, lungs, spleen, brain)

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34
Q

What are sequestration crises? (sickle cell disease)

A

Sickling with organs, e.g. spleen/lungs causes pooling of blood and worsening of anaemia

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35
Q

What is acute chest syndrome? (sickle cell disease)

A

Dyspnoea, chest pain, pulmonary infiltrates, low pO2

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36
Q

What is an aplastic crisis? (sickle cell disease)

A

Caused by infection with parovirus

Sudden fall in Hb

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37
Q

What is a haemolytic crisis? (sickle cell disease)

A

Rare

Fall in Hb due to increased rate of haemolysis

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38
Q

In which ethnicity is G6PD deficiency most common?

A

Mediterranean, African

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39
Q

How is G6PD deficiency inherited?

A

X-linked recessive

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40
Q

What is the pathophysiology of G6PD deficiency?

A

Reduced G6PD –> reduced gluthathione –> increased red cell susceptibility to oxidative stress

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41
Q

What are the features of G6PD deficiency?

A

Neonatal jaundice
IV haemolysis
Gallstones
Splenomegaly

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42
Q

What can you see on blood films in G6PD deficiency?

A

Heinz bodies

Bite + blister cells

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43
Q

How do you diagnose G6PD deficiency?

A

G6PD enzyme assay

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44
Q

What drugs can cause haemolysis in G6PD deficiency?

A

Anti-malarials (primaquine)
Ciprofloxacin
Sulphonamides, sulphasalazine, SUs

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45
Q

How is hereditary spherocytosis inherited?

A

Autosomal dominant

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46
Q

What is the typical history of hereditary spherocytosis?

A

Neonatal jaundice
Chronic symptoms although haemolytic crises may be precipitated by infection
Gallstones
Splenomegaly

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47
Q

What do you see on blood film in hereditary spherocytosis?

A

Spherocytes

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48
Q

How do you diagnose hereditary spherocytosis?

A

Osmotic fragility tests

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49
Q

What investigations should you do in suspected MM and what will they show?

A

FBC - anaemia, thrombocytopenia
U+E - raised urea and creatinine
Bone profile - raised Ca
Serum/urine protein electrophoresis - raised concentrations of monoclonal IgA/IgG (in the urine they are known as Bence Jones Proteins)

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50
Q

What investigation is required to make a diagnosis of MM?

A

Bone marrow aspiration and trephine biopsy

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51
Q

Aside from the initial investigations and the bone marrow aspiration to confirm the diagnosis of MM, what other investigation should be done?

A

Whole body MRI to survey the skeleton for bone lesions

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52
Q

What is a common x-ray finding in MM?

A

Rain-drop skull (due to numerous randomly placed dark spots due to bone lysis)

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53
Q

What 3 things define MM?

A

Monoclonal plasma cells in bone marrow >10%
Monoclonal protein within the serum/urine
Evidence of end organ damage, e.g. hypercalcaemia, elevated Cr, anaemia, lytic bone lesions/fractures

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54
Q

How is MM cured?

A

It cannot be cured - management aims to control symptoms and prolong life

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55
Q

For those suitable for autologous stem cell transplantation what is the induction therapy for MM?

A

Bortezomib and dexamethasone

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56
Q

For those unsuitable for autologous stem cell transplantation what is the induction therapy for MM?

A

Thalidomide and an alkylating agents and dexamethasone

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57
Q

What MM patients are typically suitable for autologous stem cell transplant?

A

Younger, healthier patients

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58
Q

What happen after MM patients are treated?

A

Monitored 3mnthly

Often go into remission for a long while

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59
Q

If an MM patient relapses after initial treatment what is the first line recommended treatment?

A

Bortezomib monotherapy

Some patients may also be suitable for a repeat autologous stem cell transplant

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60
Q

How is pain in MM treated?

A

Analgesia

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61
Q

How are pathological fractures in MM prevented/managed?

A

Zoledronic acid

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62
Q

What prophylaxis/vaccinations do MM patients recieve?

A

Flu vaccines

VTE prophylaxis

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63
Q

What are the major and minor criteria for diagnosing MM and how many do you need of each for a diagnosis?

A

MAJOR -

  • Plasmacytoma (as demonstrated on biopsy)
  • 30% plasma cells in bone marrow sample
  • Elevated levels of M protein in blood/urine

MINOR -

  • 10-30% plasma cells in bone marrow
  • Minor elevations in level of M protein in blood/urine
  • Osteolytic lesions
  • Low levels of antibodies in blood

1 major + 1 minor or 3 minor in an individual with signs/symptoms of MM

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64
Q

What largely causes hypercalcaemia in MM?

A

Increased osteoclastic bone resorption caused by local cytokines released by myeloma cells

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65
Q

What complications may patients develop after a DVT (post-thrombotic syndrome)?

A

Venous outflow obstruction, venous insufficiency –> chronic venous HTN

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66
Q

What features may be seen in post-thrombotic syndrome?

A 
Painful, heavy calves
Pruritus
Swelling
Varicose veins
Venous ulceration
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67
Q

What treatment may be used in post-thrombotic syndrome?

A

Compression stockings

Keeping the leg elevated

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68
Q

What are the classifications of blood production transfusion complications?

A

Immunological - acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis
Infective
TRALI
TACO
Others - hyperkalaemia, iron overload, clotting abnormalities

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69
Q

What does acute haemolytic transfusion reaction result from?

A

Mismatch of blood group (ABO) –> massive intravascular haemolysis (usually due to RBC destruction by IgM type antibodies)

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70
Q

When do the symptoms of AHTR occur?

A

Minute after transfusion is started

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71
Q

What are symptoms of AHTR?

A 
Fever
Abdominal pain
Chest pain 
Agitation 
Hypotension
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72
Q

What is the treatment of AHTR?

A

Immediate transfusion termination

Generous fluid resus with saline

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73
Q

What are complications of AHTR?

A

DIC, renal failure

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74
Q

What causes non-haemolytic febrile reactions?

A

White blood cell HLA antibodies

Often the result of sensitization by previous pregnancies or transfusions

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75
Q

How is non-haemolytic febrile reaction managed?

A

Paracetamol

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76
Q

When do symptoms of allergic/anaphylactic reactions to blood products begin?

A

Minutes after starting the transfusion

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77
Q

What are symptoms of allergic/anaphylactic reactions to blood products?

A

Urticaria –> anaphylaxis with hypotension, SoB, wheezing, stridor or angioedema

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78
Q

How should simple urticaria as a result of allergic reactions to blood products be treated?

A

Stop transfusion, antihistamine

After symptoms resolve, can continue transfusion

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79
Q

How should more severe allergic/anaphylactic reactions to blood products be treated?

A

Permanently discontinue transfusion
IM adrenaline
Supportive care
Consider antihistamines, corticosteroids, bronchodilators

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80
Q

What is TRALI?

A

Transfusion related acute lung injury

Defined as development of hypoxaemia/acute respiratory distress syndrome within 6 hours of transfusion

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81
Q

What are features of TRALI?

A

Hypoxia
Pulmonary infiltrates on CXR
Fever
Hypotension

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82
Q

What causes TACO (transfusion associated circulatory overload)?

A

Fluid overload –> pulmonaryoedema

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83
Q

What are features of TACO?

A

Features of pulmonary oedema + hypertension (key different from TRALI)

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84
Q

What virus may be transmitted by blood transfusion (rarely)?

A

vCJD

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85
Q

What steps have been taken to reduce the risk of vCJD being spread through blood products?

A

Leuodepletion (all white cells removed)
Plasma derivatives have been fractionated from imported FFP
Children/other group have imported FFP
Recipients of blood components cannot donate blood

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86
Q

What are causes of severe thrombocytopenia?

A

ITP
DIC
TTP
Haematological malignancy

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87
Q

What are causes of moderate thrombocytopenia?

A 
Heparin induced thrombocytopenia (HIT)
Drug induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
Alcohol
Liver disease
Hypersplenism
Viral infection (e.g. HIV, EBV, hepatitis)
Pregnancy
SLE/antiphospholipid syndrome
Vit B12 deficiency
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88
Q

Why is thrombocytopenia in liver disease a common finding?

A

Splenomegaly (due to portal hypertension) –> larger SA for splenic sequestration of thrombocytes
Thrombopoietin deficiency as liver as damaged

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89
Q

In which haematological malignancy is the philadelphia chromosome present in more than 95% of patients?

A

CML

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90
Q

What is the philadelphia chromosome?

A

Translocation between the long arm of chromosome 9 and 22 (ABL proto-oncogene from chromo9 being fused with BCR gene from chromo22) –> BCR-ABL genes for a fusion protein which has tyrosine kinase activity in excess of normal

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91
Q

At what age does CML tend to present?

A

60-70 years

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92
Q

What are the typical features of CML?

A

Anaemia –> lethargy
Wt loss, sweating
Splenomegaly –> abdominal discomfort

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93
Q

Burkitt’s lymphoma is a neoplasm of what cells?

A

B cells

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94
Q

Is Burkitt’s lymphoma high or low grade?

A

High grade

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95
Q

What are the two forms of Burkitt’s lymphoma?

A

Endemic (African) form - usually involves maxilla and mandible

Sporadic form - abdominal tumours most common, more common in those with HIV

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96
Q

What gene translocation is Burkitt’s lymphoma associated with?

A

c-myc gene translocation (usually t(8:14)

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97
Q

What virus is strongly associated with Burkitt’s lymphoma?

A

EBV

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98
Q

What microscopic findings do you see in Burkitt’s lymphoma?

A

Starry sky appearance (lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells)

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99
Q

How is Burkitt’s lymphoma managed?

A

Chemotherapy

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100
Q

What is a common complication of Burkitt’s lymphoma?

A

Tumour lysis syndrome

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101
Q

What drug may be given to those undergoing chemotherapy to prevent tumour lysis syndrome?

A

Rasburicase (recombinant urate oxidase (an enzyme which catalyses conversion of uric acid to allantoin)

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102
Q

What are complications of tumour lysis syndrome?

A 
Hyperkalaemia
Hyperphosphataemia
Hypocalcaemia
Hyperuricaemia
Acute renal failure
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103
Q

What causes tumour lysis syndrome?

A

Cell breakdown following chemotherapy which leads to release of large amount of intracellular components, e.g. K, phosp, uric acid

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104
Q

What is the first line treatment for most individuals with a VTE?

A

DOACs

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105
Q

What score should be calculated if a patient is suspected of having had a DVT?

A

Two level DVT Wells score

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106
Q

What is the two level wells score?

A

INSERT TABLE FROM PASSMED!!

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107
Q

What wells score indicates DVT is unlikely?

A

1 point or less

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108
Q

What wells score indicates DVT is likely?

A

2+

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109
Q

If DVT is ‘likely’ then what action should follow?

A

Proximal leg vein USS within 4h
If +ve –> diagnose DVT + start anticoagulants
If -ve –> D-dimer
If D-dimer + scan negative –> DVT unlikely
Scan -ve, d-dimer +ve –> stop interim therapeutic anticoagulation, repeat proximal leg vein USS in 6-8 days

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110
Q

If DVT is ‘likely’ and a proximal leg vein USS cannot be arranged what action should follow?

A

Give therapeutic anticoagulation in the interm (DOAC)

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111
Q

If DVT is ‘unlikely’ what action should follow?

A

D-dimer test within 4h (if not interim therapeutic anticoagulation)
If -ve –> DVT unlikely
If +ve –> proximal leg vein USS within 4h

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112
Q

What kinds of d-dimer tests do NICE recommend?

A

Point of care (finger prick) or lab based

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113
Q

What DOACs are offered first line for management of DVT?

A

Apixiban or rivaroxaban

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114
Q

If apixiban and rivaroxaban are CI for the treatment of DVT, what can be given?

A

LMWH, then followed by dagibatran or edoxaban or LMWH followed by vit K antagonist, e.g. warfarin

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115
Q

How is DVT in severe renal impairment managed?

A

LMWH, UF or LMWH followed by a VKA

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116
Q

How is DVT in a patient with antiphospholipid syndrome managed?

A

LMWH followed by VKA

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117
Q

All patients with DVT should be anticoagulated for how long?

A

At least 3 months

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118
Q

What is the difference between a provoked and unprovoked DVT?

A

Provoked - obvious precipitating event, e.g. immobilisation after surgery (implied after this pt no longer at risk)

Unprovoked - absence of obvious precipitating event, i.e. possibility of unknown factors making patient at risk of further clots

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119
Q

When is anticoagulation stopped for provoked DVTs?

A

After initial 3 months

3-6m for those with cancer

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120
Q

When is anticoagulation stopped for unprovoked DVTs?

A

Typically continued for a further 3 months

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121
Q

What score can be used to help to assess the risk of bleeding in a patient following a DVT?

A

HAS-BLED

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122
Q

What patients are not offered thrombophilia screening after a DVT?

A

Those on lifelong warfarin as it won’t alter the management

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123
Q

What might you consider testing for in an unprovoked DVT/PE?

A

Antiphospholipid antibodies

Or hereditary thrombophilia in those who also have a first degree relative who has had a DVT/PE

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124
Q

What is polycythaemia vera?

A

Myeloproliferative disorder caused by clonal proliferation of marrow stem cell leading to an increase in red cell volume (often accompanied by overproduction of neutrophils and platelets)

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125
Q

What mutation is present in 95% of cases of polycythaemia vera?

A

JAK2

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126
Q

When does the incidence of polycythaemia vera peak?

A

60s

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127
Q

What are features of polycythaemia vera?

A 
Hyperviscosity
Pruritus, typically after a hot bath 
Splenomegaly
Haemorrhage (secondary to abnormal platelet function)
Plethoric appearance
HTN in 1/3rd
Low ESR
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128
Q

What tests should be performed in those suspected of having polycythaemia vera?

A

FBC/film (raised haematocrit, neutrophils, basophils and platelets in half of patients)
JAK2 mutation
Serum ferritin
Renal + liver function tests

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129
Q

In suspected polycythaemia vera:

If the JAK2 mutation is negative + there is no obvious secondary cause, what tests should be done?

A 
Red cell mass
Arterial O2 saturation 
Abdominal USS
Serum erythropoietin 
Bone marrow aspiration and trephine
Cytogenetic analysis
Erythroid bursting forming unit culture
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130
Q

What is the criteria for the diagnosis of polycythaemia vera (JAK2+ve)?

A

Req both criteria:
A1 - high haematocrit (>0.52 in men, >0.48 in women) or raised red cell mass (>25% above predicted)
A2 - mutation in JAK2

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131
Q

What is the criteria for the diagnosis of polycythaemia vera (JAK2-ve)?

A

Req A1+A2+A3 + either another A or two B criteria

A1 - raised RC mass (>25% above predicted) or haematocrit >0.6 in men, >0.56 in women
A2 - absence of mutation in JAK2
A3 - no cause of secondary erythrocytosis
A4 - palpable splenomegaly
A5 - presence of an acquired genetic abnormality (excluding BCR-ABL) in haematopoietic cells

B1 - thrombocytosis
B2 - neutrophil leucocytosis
B3 - radiological evidence of splenomegaly
B4 - endogenous erythroid colonies or low serum erythropoietin

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132
Q

What things may cause a relative polycythaeia?

A

Dehydration and diuretics

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133
Q

What complications are associated with CLL?

A

Anaemia
Hypogammaglobulinaemia –> recurrent infections
Warm autoimmune haemolytic anaemia (10-15%)
Transformation to high grade lymphoma

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134
Q

What is transformation of CLL to high grade lymphoma known as?

A

Richter’s transformation

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135
Q

What happens in Richter’s transformation?

A

Leukaemia cells enter the LNs and change into high grade, fast growing non-hodgkins lymphoma + patients suddenly become v. unwell

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136
Q

What symptoms may indicate a Richter’s transformation has taken place?

A 
LN swelling
Fever without infection 
Wt loss
Night sweats
Nausea
Ab pain
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137
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand’s diease

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138
Q

How are the majority of cases of Von Willebrand’s disease inherited?

A

AD

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139
Q

How does Von Willebrand’s disease present?

A

As a platelet disorder, i.e. epistaxis, menorrhagia are common

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140
Q

What is von willebrand factor?

A

Large glycoprotein which forms massive multimers and promotes platelet adhesion to damaged endothelium
Carrier molecule for factor VIII

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141
Q

What are the types of Von Willebrand’s disease?

A

Type 1 - partial reduction in vWF (80% of patients)
Type 2 - abnormal form of vWF
Type 3 - total lack of vWF (AR)

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142
Q

What will investigations show in Von Willebrand’s disease?

A

Prolonged bleeding time
APTT prolonged
Factor VIII may be moderately reduced
Defective platelet aggregation with ristocetin

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143
Q

What treatments are available for Von Willebrand’s disease?

A

Tranexamic acid for mild bleeding
Desmopressin - raises levels of Von Willebrand’s factor by inducing its release from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate

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144
Q

Transmission of which type of infection is most likely to occur following a platelet transfusion and why?

A

Bacterial as platelet concentrations are stored at room temp so there is more of a risk of bacterial proliferation

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145
Q

What is Hodgkin’s lymphoma?

A

Malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell

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146
Q

What age group is Hodgkin’s lymphoma most common in?

A

Most common in 3rd and 7th decade

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147
Q

What are the histological classifications of Hodgkin’s lymphoma?

A

Nodular sclerosing
Mixed cellularity
Lymphocyte predominant
Lymphocyte depleted

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148
Q

What is the most common type of Hodgkin’s lymphoma?

A

Nodular slcerosing

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149
Q

What type of Hodgkin’s lymphoma has the worst prognosis?

A

Lymphocyte depleted

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150
Q

What cells are associated with nodular sclerosing Hodgkin’s lymphoma?

A

Lacunar cells

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151
Q

What type of Hodgkin’s lymphoma has the best prognosis?

A

Lymphocyte predominant

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152
Q

What kind of symptoms in Hodgkin’s lymphoma imply a poor prognosis?

A

B SYMPTOMS
Wt loss >10% in last 6 months
Fever >38C
Night sweats

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153
Q

What are poor prognostic factors in Hodgkin’s lymphoma?

A 
Age >45
Stage IV disease
Hb <10.5g/dl
Lymphocyte count <600/ul or <8%
Male
Albumin <40g/l
White blood count >15, 000/ul
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154
Q

What are indications for cryoprecipitate?

A

Massive haemorrhage

Uncontrolled bleeding due to haemophilia

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155
Q

What does cyroprecipitate contain?

A

Factor VIII
Fibrinogen
vWF
Factor XIII

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156
Q

What causes CLL?

A

Monoclonal proliferation of well differentiate lymphocytes which are almost always B cells

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157
Q

What is the commonest form of leukaemia in adults?

A

CLL

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158
Q

What are features of CLL?

A

Often none
Constitutional - anorexia, wt loss
Bleeding, infections
Lymphadenopathy more marked than CML

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159
Q

What are complications of CLL?

A

Anaemia
Hypogammglobulinaemia –> recurrent infections
Warm autoimmune haemolytic anaemia (10-15%)
Transformation to high grade lymphoma (Richter’s)

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160
Q

What investigations should be done for suspected CLL and what will they find?

A

Blood film - smudge cells (aka smear cells)

Immunophenotyping

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161
Q

What blood film finding is associated with MM?

A

Rouleaux formation

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162
Q

What are the 3 novel oral anticoagulants (NOACs)?

A

Dabigatran, rivaroxaban and apixaban

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163
Q

What is the mechanism of action of dabigatran?

A

Direct thrombin inhibitor

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164
Q

What is the mechanism of action of rivaroxaban?

A

Direct factor Xa inhibitor

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165
Q

What is the mechanism of action of apixiban?

A

Direct factor Xa inhibitor

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166
Q

How are the NOACs excreted from the body?

A

Dabigatran - mostly renal
Rivaroxaban - mostly liver
Apixaban - mostly faecal

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167
Q

What are indications for NOACs?

A

Prevention of VTE following hip/knee surgery
Treatment of DVT/PE
Prevention of stroke in non-valvular AF (if prior stroke/TIA, age75+, HTN, DM, heart failure)

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168
Q

Should compression stockings be routinely offered to all those with DVT?

A

No

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169
Q

In which ethnicity is hereditary spherocytosis most common?

A

Northern European

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170
Q

What is hereditary spherocytosis?

A

AD defect of RBC cytoskeleton where the biconcave disc is replaced by a sphere spaced RBC –> reduced RBC survivial due to destruction by spleen

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171
Q

How does hereditary spherocytosis present?

A 
FTT
Jaundice, gallstones
Splenomegaly
Aplastic crisis precipitated by parovirus (severe anaemia, reduced retic count)
Degree of haemolysis variable 
MCHC elevated
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172
Q

What is the investigation of choice for diagnosing hereditary spherocytosis?

A

Osmotic fragility test (if equivocal –> cyrohaemolyis test + EMA binding)

(But if someone has a FH of HS, typical clinical features + lab investigations (spherocytes, raised MCHC + increased retics) they do not req. additional tets

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173
Q

How is an acute haemolytic crisis in hereditary spherocytosis managed?

A

Supportive

Transfusion may be req.

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174
Q

What is the long term treatment of hereditary spherocytosis?

A

Folate replacement

Splenectomy

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175
Q

In which condition(s) do you see the following on blood film:
Target cells?

A

Sickle cell/thalassaemia
Iron deficiency anaemia
Hyposplenism
Liver disease

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176
Q

In which condition(s) do you see the following on blood film:
Tear drop poikilocytes?

A

Myelofibrosis

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177
Q

In which condition(s) do you see the following on blood film:
Spherocytosis?

A

Hereditary spherocytosis

Autoimmune haemolytic anaemia

178
Q

In which condition(s) do you see the following on blood film:
Basophilic strippling?

A

Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia

179
Q

In which condition(s) do you see the following on blood film:
Howell-Jolly bodies?

A

Hyposplenism

180
Q

In which condition(s) do you see the following on blood film:
Heinz bodies?

A

G6PD deficiency

Alpha thalassaemia

181
Q

In which condition(s) do you see the following on blood film:
Schisocytes?

A

IV haemolysis
Mechanical heart valve
DIC

182
Q

In which condition(s) do you see the following on blood film:
Pencil poikilocytes?

A

Fe deficient anaemia

183
Q

In which condition(s) do you see the following on blood film:
Burr cells?

A

Uraemia

Pyruvate kinase deficiency

184
Q

In which condition(s) do you see the following on blood film:
Acanthocytes?

A

Abetalipoproteinaemia

185
Q

In which condition(s) do you see the following on blood film:
Hypersigmented neutrophils?

A

Megloblastic anaemia

186
Q

Basics of lymphatic drainage

A

Superficial lymphatic vessels drain the skin and follow venous drainage

Superficial lymphatic vessels + drainage of internal organs –> deep lymphatic vessels that follow arteries –> LNs –> lymphatic trunks which unite to form right lymphatic duct/thoracic duct

Right lymphatic duct drains R side of head + R arm, thoracic duct does rest

Both lymphatic ducts drain into venous system

187
Q

Where do the superficial inguinal lymph nodes drain?

A 
Anal canal below pectinate line
Perineum
Skin of thigh
Penis
Scrotum
Vagina
188
Q

Where do the deep inguinal LNs drain?

A

Glans penis

189
Q

Where do the para-aortic LNs drain?

A

Testes, ovaries
Kidney
Adrenal gland

190
Q

Where do the axillary LNs drain?

A

Lateral breast

Upper limb

191
Q

Where do the internal iliac LNs drain?

A

Anal canal above pectinate line
Lower part of rectum
Pelvic structures, incl. cervix + inferior part of rectus

192
Q

Where do the superior mesenteric LNs drain?

A

Duodenum

Jejunum

193
Q

Where do the inferior mesenteric LNs drain?

A

Descending colon
Sigmoid colon
Upper rectum

194
Q

Where do the coeliac LNs drain?

195
Q

What do you typically see on blood film in G6PD deficiency?

A

Blister and bite cells

196
Q

Raised ESR and osteoporosis = what until otherwise proven?

197
Q

What is immune thrombocytopenia (ITP) in adults?

A

Immune-mediated reduction in platelet count

198
Q

What causes ITP?

A

Antibodies directed against glycoprotein IIb/IIIa or Ib-V-IX complex

199
Q

What are the two forms of ITP?

A

Acute

Chronic

200
Q

Who is acute ITP more commonly seen in ?

201
Q

What is the natural course of acute ITP?

A

Self-limiting course over 1-2 weeks

202
Q

What may acute ITP follow?

A

Infection/vaccination

203
Q

Who is chronic ITP more common in?

A

Young/middle aged women

204
Q

What is the course of chronic ITP?

A

More relapsing/remitting nature

205
Q

What is Evan’s syndrome?

A

ITP in associated with autoimmune haemolytic anaemia

206
Q

What is the most common cause of an isolated thrombocytopenia?

207
Q

What are the main causes of Fe deficient anaemia?

A

Blood loss
Inadequate dietary intake, e.g. vegan
Poor intestinal absorption, e.g. coeliac
Increased Fe requirements, e.g. children growing, pregnancy

208
Q

What is the most common cause of blood loss causing Fe deficient anaemia?

A

Pre-menopausal women - menorrhagia

Men/post-menopausal women - GI blood loss (always suspect colon cancer)

209
Q

What are clinical features of Fe deficient anaemia?

A 
Fatigue
SoBoE
Palpitations
Pallor
Nail changes, incl. koilonychia (spoon shaped nails) 
Hair loss
Atrophic glossitis
Post-cricoid webs
Angular stomatitis
210
Q

What things are really important to ask about in an Fe deficient anaemia?

A 
Changes in diet
Medications
Menstrual history 
Wt loss
Change in bowel habit
211
Q

What does FBC show in an Fe deficient anaemia?

A

Hypochromic microcytic anaemia

212
Q

What is the ferritin level in Fe deficient anaemia normally?

A

Low (as serum ferritin correlates with iron stores)

NB - ferritin increased during inflammation

213
Q

What will the total iron binding capacity (TIBC) be in Fe deficient anaemia?

A

High (high TIBC reflects low iron stores)

214
Q

What will the transferrin saturation be in Fe deficient anaemia?

215
Q

What might you see on blood film in Fe deficient anaemia?

A

Anisopoikilocytosis (RBCs of different sizes/shapes)
Target cells
Pencil poikilocytes

216
Q

What other investigation should be done in those with Fe deficiency anaemia who are male/post-menopauseal?

A

Endoscopy to rule out GI cancer

217
Q

How is Fe deficient anaemia managed?

A

Treat underlying cause
Oral ferrous sulphate (taken until 3m after anaemia corrected to replenish iron stores)
Iron rich diet

218
Q

What foods are rich in iron?

A

Dark leafy veg, meat, iron fortified bread

219
Q

What are common SEs of iron supplementation?

A

Nausea, ab pain, constipation, diarrhoea

220
Q

Can tumour lysis syndrome occur even before starting chemotherapy?

A

Yes - can occur alone or even on initiation of steroid therapy

221
Q

What cancers is tumour lysis syndrome most common in?

A

High grade leukaemias/lymphomas

222
Q

What medications should be given to those at high risk of TLS?

A

IV allopurinol or IV rasburicase prior to and during first days of chemo

223
Q

How does rasburicase work?

A

Converts uric acid into allantoin

Allantoin much more water soluble than uric acid so more easily excreted by kidneys

224
Q

What should those at low risk of TLS be given?

A

Oral allupurinol during chemo cycles

225
Q

How does TLS present?

A

High K, high Phosp, low Ca

Suspect in any patient with AKI in presence of high phosphate and high uric acid level

226
Q

What grading system is used for TLS?

A

Cairo-Bishop

227
Q

How is laboratory tumour lysis syndrome defined?

A

Abnormality in 2+ of the following occuring within 3 days before or 7 days after chemo:

  • High uric acid
  • High K
  • High phosp
  • Low ca
228
Q

How is clinical tumour lysis syndrome defined?

A

Lab tumour lysis syndrome + 1+ of the following:

  • Increased serum CK (>1.5x normal)
  • Cardiac arrhythmia/suddnen death
  • Seizure
229
Q

What is neutropenic sepsis usually a complication of?

A

Common complication of cancer treatment (usually chemo)

230
Q

When does neutropenic sepsis tend to occur?

A

7-14 days after chemo

231
Q

Define neutropenic sepsis

A

Neutrophil count of <0.5x10^9 in a patient having anticancer treatment and who has 1 of the following:

  • Temp 38C+
  • Other signs/symptoms consistent with significant sepsis
232
Q

Who should be offered prophylaxis against neutropenic sepsis?

A

Those who are anticipated to have a neutrophil count of <0.5x10^9 due to their treatment

233
Q

What is the prophylaxis for neutropenic sepsis?

A

Fluoroquinolone

234
Q

How is neutropenic sepsis managed?

A

Empirical antibiotics (piperacillin + tazobactam) immediately
If still unwell + febrile >48h meropenem prescribed +/- vancomycin
If not responding within 6-8d investigate for fungal infections
May be role for G-CSF in selected patients

235
Q

Should all patients with sickle cell crises be admitted to hospital?

A

Yes - unless they are a well adult with mild/moderate pain and temp of <=38C or child with no fever + mild pain

236
Q

Who should you have a low threshold for admitting in sickle cell crises?

A

A child
If temp >38C (risk of rapid deterioriation)
If person has chest symptoms (as acute chest syndrome can develop quickly)

237
Q

What is the general management of a sickle cell crisis?

A

Analgesia, e.g. opiates
Rehydrate
Oxygen
Consider antibiotics if evidence of infection
Blood transfusion
Exchange transfusion, e.g. if neurological complications

238
Q

What is the abnormality in beta-thalassaemia major?

A

Absence of beta chains

defect on chromo 11

239
Q

How does beta-thalasaemia major present?

A

In first year of life with FTT and hepatosplenomegaly
Microcytic anaemia
HbA2 and HbF raised
HbA absent

240
Q

How is beta-thalasaemia major managed?

A

Repeated transfusions (–> iron overload so also req. iron chelation with s/c desferrioxamine)

241
Q

What are the types of polycythaemia?

A

Relative
Primary - PV
Secondary

242
Q

What are causes of secondary polycythaemia?

A

COPD
Altitude
Obstructive sleep apnoea
Excessive EPO - cerebellar haemangioma, hypernephroma, heptatoma, uterine fibroids

243
Q

What studies are used to differentiate between true and relative polycythaemia?

A

Red cell mass studies

true polycythaemia total RC mass >35ml/kg in men, >32ml/kg in women

244
Q

What are the two categories of causes of a macrocytic anaemia?

A

Megaloblastic causes –> vit B12 deficiency, folate deficiency

Normoblastic causes –> alcohol, liver disease, hypothyroidism, pregnancy, reticulocytosis, myelodysplasia, drugs - cytotoxics

245
Q

What tests might you do if you are suspecting a megaloblastic anaemia?

A

FBC
Blood film
Haematinics

246
Q

What is the pathophysiology of G6PD deficiency?

A

Deficiency of glucose-6-phosphate dehydrogenase means red cells have less ability to respond to oxidative stress + therefore live for less time and are more susceptible to haemolysis (particularly in response to drugs, infection, acidosis and certain foods)

247
Q

Who should be offered platelet transfusion?

A

Those with platelet count <30x10^9 + clinically significant bleeding

Maximum of <100x10^9 for those with severe bleeding/bleeidng in critical sites (e.g. CNS)

May be given to some patients with thrombocytopenia before surgery

If <10x10^9 if no active bleeding/surgery/no alternative medications for their condition

248
Q

In which conditions is platelet transfusion CI?

A

Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin induced thrombocytopenia
TTP

249
Q

What haematological malignancy is h. pylori associated with?

A

Gastric lymphoma (MALT)

250
Q

What cancers is EBV associated with?

A

Hodgkin’s + Burkitt’s lymphoma, nasopharyngeal cancer

251
Q

What cancer is HIV associated with?

A

High grade B cell lymphoma

252
Q

What cancer is malaria associated with?

A

Burkitt’s lymphoma

253
Q

What are the recommended thresholds for transfusion of red cells in patients with and without ACS?

A

Without - 70g/L

With - 80g/L

254
Q

In a non-urgent situation how long is a unit of RBC transferred over?

A

90-120 minutes

255
Q

At what temperature are RBCs stored prior to infusion?

256
Q

Why is it important to transfuse patients who are anaemia and having an ACS?

A

Anaemia can worsen ischaemia (as less oxygen is carried by haemoglobin)

257
Q

What factors are affected by heparin?

A

Prevents activation of factors 2, 9, 10, 11

258
Q

What factors are affected by warfarin?

A

Affects synthesis of factors 2, 7, 9, 10

259
Q

What factors are affected in DIC?

A

1, 2, 5, 8, 11

260
Q

What factors are affected in liver disease?

A

1, 2, 5, 7, 9, 10, 11

261
Q 
What are the typical findings in the following:
a. APTT
b. PT
c. Bleeding time
In haemophilia?
A

APTT - increased
PT - normal
Bleeding time - normal

262
Q 
What are the typical findings in the following:
a. APTT
b. PT
c. Bleeding time
in von Willebrand disease?
A

APTT - increased
PT - normal
Bleeding time - increased

263
Q 
What are the typical findings in the following:
a. APTT
b. PT
c. Bleeding time 
in vitamin K deficiency?
A

APTT - increased
PT - increased
Bleeding time - normal

264
Q

When might patients receive packed red cells?

A

Chronic anaemia and cases where large vols of fluid may –> CV compromise

265
Q

How are packed red cells obtained?

A

Centrifugation of whole blood

266
Q

Who is platelet rich plasma given to?

A

Thrombocytopaenic patients who are bleeding/req. surgery

267
Q

How is platelet rich plasma obtained?

A

Low speed centrifugation

268
Q

How is platelet concentrate obtained?

A

High speed centrifugation

269
Q

Who is given platelet concentrate?

A

Thrombocytopaenic patients

270
Q

What is FFP prepared from?

A

Single units of blood

271
Q

What does FFP contain?

A

Clotting factors
Albumin
Ig

272
Q

How much is a unit of FFP?

273
Q

When is FFP usually used?

A

To correct clotting deficiencies in those with hepatic synthetic failure who are due to undergo surgery

274
Q

How is cryoprecipitate prepared?

A

Formed from supernatant of FFP

275
Q

What is SAG-mannitol blood?

A

Removal of all plasma from a blood unit + substitute with:

  • Sodium chloride
  • Adenine
  • Anhydrous glucose
  • Mannitol
276
Q

How many units of SAG-mannitol blood can be transfused?

A

Up to 4 (use whole blood thereafter, and consider platelets + clotting factors after 8 units)

277
Q

What are cell saver devices?

A

Devices that collect patients own blood lost during surgery and re-infuse it

278
Q

What are the two types of cell saver devices?

A

Those which wash blood cells prior to infusion (reduced risk of contamination)
Those which do not

279
Q

When are cell saver devices CI?

A

In malignant disease due to risk of facilitating disease dissemination `

280
Q

What should be done in immediate/urgent surgery in a patient taking warfarin?

A

Stop warfarin
Vit K reversal (IV takes 4-6h, oral up to 24h)
FFP (only if human prothrombin is not available)
Human prothrombin complex (reversal within 1h) - give with vit K

281
Q

What should be given in between every other unit of packed red cells and why?

A

Furosemide (to prevent fluid overload)

282
Q

Which features should prompt a very urgent FBC in someone 0-24 years old to investigate for leukaemia?

A 
Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain 
Unexplained bruising/bleeding 
Hepatosplenomegaly
283
Q

What is haemophilia?

A

X-linked recessive disorder of coagulation

284
Q

What is haemophilia A due to?

A

Deficiency of factor VIII

285
Q

What is haemophilia B due to?

A

Deficiency of factor IX

286
Q

What are the clinical features of haemophilia?

A

Haemoarthroses, haematomas

Prolonged bleeding after surgery/trauma

287
Q

Up to 10-15% of patients with haemophilia A develop what problem?

A

Develop antibodies against factor VIII treatment

288
Q

What are granulocyte colony stimulating facotrs?

A

Factors used to increase neutrophil count in those who are neutropenic secondary to chemo or other factors

289
Q

What are e.g.s of G-CSFs?

A

Filgrastim

Pergilgrastim

290
Q

What are RFs for developing febrile neutropenia?

A

Elderly
Specific malignancies - non-Hodgkin’s lymphoma, ALL
Prev neutropenic episodes
Those receiving recombinant chemo + radiation therapy

291
Q

Define acute transfusion reactions

A

Adverse signs/symptoms during or within 24h of blood transfusion

292
Q

What are the most frequent acute transfusion reactions?

A

Fever
Chills
Pruritus
Urticaria

293
Q

What are the immune mediated acute transfusion reactions?

A 
Pyrexia
Alloimmunisation 
Thrombocytopaenia
TRALI
Graft vs host disease
Urticaria
Acute/delayed haemolysis
ABO incompatability
Rhesus incompatability
294
Q

What are the non-immune mediated acute transfusion reactions?

A

Hypocalcaemia
CCF
Infections
Hyperkalaemia

295
Q

What are causes of thrombophilia?

A 
Factors V leiden (most common cause) 
Prothrombin gene mutation 
Antithrombin III deficiency 
Protein C deficiency 
Protein S deficiency

Acquired - antiphospholipid syndrome

Drugs - COCP

296
Q

What is Factor V Leiden aka?

A

Activated protein C resistance

297
Q

What causes factor V leiden?

A

Gain of a function mutation in the factor V leiden protein –> activated factor V (a clotting factor) is inactivated 10x more slowly by activated protein C than normal

298
Q

How does being homozygous/heterozygous for factor v leiden affect the risk of having a VTE?

A

Homozygote - 10x

Heterozygote - 4-5x

299
Q

What are the thalassaemias?

A

Group of genetic disorders characterised by reduced production of either alpha or beta chains

300
Q

What is beta-thalassaemia trait?

A

Autosomal recessive condition characterised by a mild hypochromic, microcytic anaemia (usually asymptomatic)

301
Q

What are the features of beta-thalassaemia triat?

A

Mild hypochromic, microcytic anaemia

HbA2 raised

302
Q

What is sickle cell anaemia?

A

AR condition that results in synthesis of an abnormal Hb chain called HbS

303
Q

In which group of people is sickle cell anaemia more common and why?

A

People of African descent as the heterozygous condition offers protection against malaria

304
Q

When do the symptoms of sickle cell anaemia in homozygotes present?

A

After 4-6 months when abnormal HbSS molecules take over from fetal Hb

305
Q

What is the pathophysiology of sickle cell anaemia?

A

Polar AA glutamate is substituted by non-polar valine in each of the two beta chains –> decreased water solubility of deoxy-Hb –> HbS molecules polyermise and cause RBCs to sickle
Sickle cells are fragile and haemolyse and block small BVs –> infarction

306
Q

How is sickle cell disease definitively diagnosed?

A

Hb electrophoresis

307
Q

What are causes of massive splenomegaly?

A 
Myelofibrosis
CML
Visceral leishmaniasis
Malaria
Gaucher's syndrome
308
Q

What are the two subtypes of acquired haemolytic anaemias?

A

Immune and non-immune causes

309
Q

What are immune causes of acquired anaemias?

A

Autoimmune - warm/cold antibody type
Alloimmune - transfusion reaction, haemolytic disease of newborn
Drug - methyldopa, penicillin

310
Q

What are non-immune causes of acquired anaemias?

A 
Microangiopathic haemolytic anaemia - TTP/HUS, DIC, malignancy, PET
Prosthetic cardiac valves
Paroxysmal nocturnal haemoglobulinuria
Infections - malaria 
Drugs - dapsone
311
Q

What does thrombin do?

A

Converts fibrinogen to fibrin

312
Q

What does the fibrinolytic system do?

A

Breakdown fibrinogen and fibrin by producing plasmin

313
Q

What does the breakdown of fibrinogen and fibrin result in?

A

Fibrin degradation products

314
Q

What happens in DIC?

A

Dysregulation of coagulation and fibrinolysis –> widespread clotting with resultant bleeding

Transmembrane glycoprotein (tissue factor) is released. TF is present on surface of many cells and generally is not exposed to the circulation, but after vascular damage it is.

TF binds with coagulation factors that then trigger the extrinsic pathway (via factor VII) which subsequently triggers the intrinsic pathway (XII to XI to IX) of coagulation

315
Q

What are causes of DIC?

A

Sepsis
Trauma
Obstetric complications, e.g. aminotic fluid embolism, HELLP syndrome
Malignancy

316
Q

What does the typical blood picture look like in DIC?

A

Low platelets
Prolonged APTT, prothrombin + bleeding time
Fibrin degradation products raised
Schistocytes due to microangiopathic haemolytic anaemia

317
Q

A Hb below what in a man should be referred for upper and lower GI endoscopy as a 2ww?

318
Q

What is thought to cause myelofibrosis?

A

Hyperplasia of abnormal megakaryocytes

The resultant release of platelet derived growth factor is thought to stimulate fibroblasts

319
Q

Haematopoiesis develops in which two places in myelofibrosis?

A

Liver and spleen

320
Q

What are features of myelofibrosis?

A

Tends to be older person presenting with symptoms of anaemia (e.g. fatigue)
Massive splenomegaly
Hypermetabolic symptoms, e.g. night sweats, wt loss

321
Q

What are common lab findings in myelofibrosis?

A

Anaemia
High WBC and platelet count early in disease
Tear drop poikilocytes on blood film
Unobtainable bone marrow biopsy - dry tap - therefore trephine biopsy req.
High urate + LDH (reflect increased cell turnover)

322
Q

What is aplastic anaemia characterised by?

A

Pancytopaenia + a hypoplastic bone marrow

323
Q

When is the peak incidence of aplastic anaemia?

A

30 years old

324
Q

What are features of aplastic anaemia?

A

Normochromic, normocytic anaemia
Leukopaenia, with lymphocytes relatively spared
Thrombocytopaenia

325
Q

What may aplastic anaemia be a presenting feature of?

A

ALL or AML

Minority of patients go on to develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

326
Q

What are causes of aplastic anaemias?

A

Idiopathic
Congenital - Faconi anaemia, dyskeratosis congenita
Drugs - cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
Toxins - benzene
Infections - parovirus, hepatitis
Radiation

327
Q

How does Fe deficiency anaemia differ from anaemia of chronic disease in:

  • Serum iron
  • TIBC
  • Transferrin saturation
  • Ferritin?
A

Fe deficient anaemia -

  • Serum iron low <8
  • TIBC high
  • Transferrin saturation low
  • Ferritin low

Anaemia of chronic disease -

  • Serum iron low <15
  • TIBC low
  • Transferrin saturation low
  • Ferritin high
328
Q

What are presenting features of Hodgkin’s lymphoma?

A

Asymptomatic lymphadenopathy
Cough, Pel Ebstein fever, haemopytsis, SoB
B symptoms - wt loss, fever, night sweats

329
Q

How are patients with Hodgkin’s lymphoma staged?

A

CT chest, abdo, pelvis

Ann Arbor staging

330
Q

What is Ann Arbor stage 1?

A

Single LN region

331
Q

What is Ann Arbor stage 2?

A

2+ regions on same side of diaphragm

332
Q

What is Ann Arbor stage 3?

A

Involvement of LN regions on both sides of diaphragm

333
Q

What is Ann Arbor stage 4?

A

Involvement of extra nodal sites

334
Q

How is Hodgkin’s lymphoma treated?

A

Chemo + radio

335
Q

How is Hodgkin’s lymphoma diagnosed?

A

Excision and histological evaulation of a complete LN

336
Q

What virus is associated with Hodgkin’s lymphoma?

337
Q

What is prothrombin complex concentrate mainly used for?

A

Emergency reversal of anticoagulation in patients with severe bleeding/head injuries

338
Q

What is hand-foot syndrome?

A

Caused by sickling of red blood cells interfering with circulation to the hands and feet in those with sickle cell disease
Leads to swelling, pain + erythema of sudden onset

339
Q

What types of crises can you get in sickle cell disease?

A 
Thrombotic (painful) crises
Sequestration 
Acute chest syndrome
Aplastic
Haemolytic
340
Q

What are thrombotic crises aka?

A

Painful/vaso-oclusive crises

341
Q

What can precipitate thrombotic crises?

A

Infection
Dehydration
Deoxygenation

342
Q

What happens in thrombotic crises?

A

Infarcts occur in various organs, incl the bones (e.g. avascular necrosis of hip, hand foot syndrome in children, lungs, spleen, brain)

343
Q

What are sequestration crises?

A

Sickling within organs, e.g. spleen/lungs –> pooling of blood with worsening anaemia

344
Q

What are clinical features of acute chest syndrome?

A

SoB, chest pain, pulmonary infiltrates, low pO2

345
Q

What causes an aplastic crisis?

A

Infection with parovirus

346
Q

What happens in aplastic crises?

A

There is a sudden fall in Hb

347
Q

What happens in haemolytic crises?

A

There is a fall in Hb due to increased rate of haemolysis

348
Q

What can elicit a haemolytic crisis in someone with hereditary spherocytosis and what features may be suggestive of it?

A

Parovirus infection

Features may include splenomegaly, fatigue, jaundice

349
Q

What does repeated sequestration and infarction of spleen in sickle cell disease lead to?

A

Auto-splenectomy

350
Q

What are features of a sequestration crisis?

A

Severe anaemia, marked pallor, abdominal pain, CV collapse due to ineffective circulating volume

351
Q

What kind of anaemia does sickle cell disease cause?

A

Normocytic anaemia with raised reticulocyte count (due to haemolysis)

352
Q

Why are pregnant women more at risk of having a DVT/PE?

A

Pregnancy is a hypercoagulable state

Increase in factors VII, VIII, X, fibrinogen, decrease in protein S, uterus presses on IVC –> venous stasis in legs

353
Q

When do the majority of VTEs occur in a pregnancy?

A

Last trimester

354
Q

How is DVT/PE in pregnancy managed?

355
Q

What are causes of hyposplenism?

A 
Splenectomy
Sickle cell 
Coeliac disease, dermatitis herpetiformis
Grave's disease
SLE
Amyloid
356
Q

What drugs can precipitate renal failure in those with MM?

357
Q

Which of the NOACs has an antedote and what is it called?

A

Dabigatran

It is called Idarucizumab

358
Q

What is the universal donor of FFP?

359
Q

What is myelodysplasia?

A

Acquired neoplastic disorder of haematopoietic stem cells where a line of myeloid blasts are produced rapidly but do not mature + die –> deficiency in that line of cells

360
Q

What may myelodysplasia progress to?

361
Q

How does myelodysplasia present?

A

Bone marrow failure (anaemia, neutropaenia, thrombocytopaenia)

362
Q

What transfusion gene is seen in acute promyelocytic leukaemia?

A

t(5;17) - fusion of PML and RAR alpha genes

363
Q

What bone profile will you see in myeloma without metastasis?

A

High calcium, normal/high phosphate, normal alk phosp

NB alk phosp only elevated in metastatic dx

364
Q

What are causes of microcytic anaemias?

A 
Fe deficiency anaemia
Thalassaemia
Congenital sideroblastic anaemia
AOCD
Lead poisoning
365
Q

A hx of normal Hb with microcytosis should raise suspicion of what?

A

Thalassaemia or polycythaemia vera (with bleeding)

366
Q

What is Waldenstrom’s macroglobulinaemia?

A

Uncommon condition seen in elderly men

Lymphoplasmcytoid malignancy where there is secretion of a monoclonal IgM paraprotein

367
Q

What are features of Waldenstrom’s macroglobulinaemia?

A 
Monoclonal IgM paraproteinaemia
Systemic upset - wt loss, lethargy
Hyperviscosity syndrome, e.g. visual disturbance
Hepatosplenomegaly
Lymphadenopathy
Cyroglobulinaemia, e.g. Raynauds
368
Q

In which type of lymphoma does alcohol consumption cause pain in the enlarged LNs?

A

Hodgkin’s (only in a minority)

369
Q

What does the reticulocyte count do in a sequestration crisis vs an aplastic crisis?

A

Increased in sequestration, reduced in aplastic

370
Q

What is the major criterion determining the use of cyroprecipitate in bleeding?

A

Low fibrinogen level

371
Q

What should be given to patients with iron deficiency anaemia prior to surgery who cannot tolerate oral iron/time interval is too short?

372
Q

What is the shortest time interval for oral iron to work?

373
Q

What general risk factors are associated with an increased risk of VTE?

A 
Advancing age
Obesity
FH VTE
Pregnancy 
Immobility
Hospitalisation 
Anaesthesia
Central venous catheter
374
Q

What conditions are associated with an increased risk of VTE?

A 
Malignancy 
Thrombophilia
Heart failure
Antiphospholipid syndrome
Behcet's
Polycythaemia
Nephrotic syndrome
Sickle cell disease
Paroxysmal nocturnal haemoglobinuria
Hyperviscosity syndrome
Homocystinuria
375
Q

What medications increase the risk of having a VTE?

A

COCP
HRT
Raloxifene, tamoxifen
Antipsychotics, esp olanzopine

376
Q

What are the two types of autoimmune haemolytic anaemia?

A

Warm
Cold

(according to the temperature at what the antibodies best cause haemolysis)

377
Q

What is the aetiology of autoimmune haemolytic anaemia?

A

Commonly idiopathic, may be secondary to a lymphoproliferative disorder, infection or drugs

378
Q

What test is always +ve in AIHA?

A

Direct antiglobulin test (Coombs’ test)

379
Q

Where does haemolysis tend to occur in warm AIHA?

A

IgG causes haemolysis best at room temp and haemolysis tends to occur in extravascular sites, e.g. the spleen

380
Q

What are management options for warm AIHA?

A

Steroids
Immunosupressants
Splenectomy

381
Q

What are causes of warm AIHA?

A

Autoimmune dx, e.g. SLE
Neoplasia, e.g. lymphoma, CLL
Drugs, e.g. methyldopa

382
Q

What antibody is usually implicated in warm AIHA?

383
Q

What antibody is usually implicated in cold AIHA?

384
Q

At what temperature do the IgM in cold AIHA cause haemolysis?

385
Q

Complete the sentence -

In cold AIHA - haemolysis is mediated by ____ and is more commonly _______.

A

Complement

Intravascular

386
Q

What are features of cold AIHA?

A

Raynaud’s

Acrocyanosis

387
Q

What are causes of cold AIHA?

A

Neoplasia, e.g. lymphoma

Infections, e.g. mycoplasma, EBV

388
Q

What is B12 mainly used for in the body?

A

RBC development and maintenance of the nervous system

389
Q

How is B12 absorbed in the body?

A

Binds to intrinsic factor (secreted from parietal cells in the stomach) + actively absorbed in the terminal ileum

390
Q

What are causes of B12 deficiency?

A 
Pernicious anaemia (most common)
Post-gastrecomy
Vegan diet/poor diet
Disorders of terminal ileum (e.g. CD)
Metformin (rare)
391
Q

What are features of a B12 deficiency?

A

Macrocytic anaemia
Sore tongue + mouth
Neurological symptoms (dorsal column affected first –> distal paraesthesia)
Neuropsychiatric symptoms, e.g. mood disturbances

392
Q

How is B12 deficiency treated?

A

No neurological involvemenet –> 1mg IM hydroxocobalamin 3x week for 2 weeks, then 3mnthly

If also deficient in folate must treat B12 deficiency first to avoid precipitating subacute combined degeneration of the cord

393
Q

What kind of anaemia can alcohol excess cause?

A

Megaloblastic

394
Q

What kind of anaemia does hypothryoidism cause?

A

Macrocytic

395
Q

What do Reed-Sternberg cells look like?

A

Mirror image nuclei

396
Q

Which of platelets and packed red cells is more likely to cause iatrogenic septicaemia with a gram positive and which with a gram negative bacteria?

A

Platelets - gram +ve

RBC - gram -ve

397
Q

What are negative prognostic factors for Hodgkin’s lymphoma?

A

Presence of B symptoms (wt loss, night sweats, fever)
Male gender
Age >45y at diagnosis
High WCC, low Hb, high ESR or low blood albumin

398
Q

What are causes of a normocytic anaemia?

A 
AOCD
CKD
Aplastic anaemia
Haemolytic anaemia
Acute blood loss
399
Q

What iron profile is typical of AOCD?

A

Normocytic anaemia, with low serum iron, low TIBC, raised ferritin

400
Q

What is the pathophysiology of AOCD?

A

Reduced iron released from bone marrow
Inadequate secretion of EPO
Reduced red cell survival

401
Q

What kind of symptoms does lead poisoning lead to?

A

Abdominal and neurological

402
Q

What does lead poisoning lead to?

A

Defective ferrochelatase and ALA dehydratase function

403
Q

What are features of lead poisoning?

A 
Abdominal pain 
Peripheral neuropathy (mainly motor)
Fatigue
Constipation 
Blue lines on gum margin
404
Q

What test is used to diagnose lead poisoning?

A

Blood lead level

Levels greater than 10mcg/dl are considered significant

405
Q

What kind of anaemia do you get in lead poisoning?

A

Microcytic anaemia

406
Q

What abnormalities do you see on the blood film in lead poisoning?

A

Basophilic stripping + clover leaf morphology

407
Q

Where can the lead accumulate in children who have lead poisoning?

A

Metaphysis of bones

408
Q

What chelating agents are used to treat lead poisoning?

A

Dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
Dimercaprol

409
Q

Why do you get anaemia in lead poisoning?

A

Due to interruption of the haem biosynthesis pathway

410
Q

In which cell is CMV transmitted in during blood transfusion?

A

Leucocytes

411
Q

Apart from to reduce spread of CMV, what is the other reason that most blood products are irradiated?

A

To avoid transfusion graft vs host disease

412
Q

What is the lymphadenopathy like in Hodgkin’s lymphoma?

A

Painless, non-tender, assymmetrical

413
Q

What is the preferred NOAC for patients with renal impairment?

A

Apixiban (due to minimal renal drug clearance)

414
Q

What is thrombocytosis?

A

An abnormally high platelet count (usually >400x10^9/l)

415
Q

What are causes of thrombocytosis?

A 
Reactive, e.g. in response to severe infection, surgery, or Fe deficiency anaemia
Malignancy 
Essential thrombocytosis
CML
Hyposplenism
416
Q

What is essential thrombocytosis?

A

A myeloproliferative disorder where megakaryocyte proliferation results in overproduction of platelets

417
Q

What are features of essential thrombocytosis?

A

Platelet count >600x10^9
Arterial and venous thromboses + haemorrhage
Burning in hands

418
Q

What mutation is found in 50% of patients with essential thrombocytosis?

419
Q

What is the management of essential thrombocytosis?

A

Hydroxyurea (hydroxycarbamide) to reduce platelet count
Interferon-a also used in younger patients
Low dose aspirin to reduce risk of thrombosis

420
Q

All people with Fe deficiency anaemia should be screened for what GI disease?

A

Coeliac disease (anti-TTG test)

421
Q

What is tranexamic acid a synthetic derivative of?

422
Q

What is the mode of action of tranexamic acid?

A

Antifibrinolytic that reversibly binds to lysine receptor sites on plasminogen or plasmin which prevents plasmin binding to and degrading fibrin

423
Q

When might tranexamic acid be used?

A

Menorrhagia

Bleeding trauma <3h (where it is given as an IV bolus followed by a slow infusion)

424
Q

Why is the COCP stopped before surgery?

A

To reduce risk of having a PE

425
Q

What is involved in the long term management of sickle cell disease?

A

Hydroxyurea (increases levels of HbF)

Pneumococcal vaccine every 5 years

426
Q

What are thymomas associated with?

A

Myasthenia gravis
Red cell asplasia
Dermatomyositis
SLE, SIADH

427
Q

How can thymomas cause death?

A

Compression of airway

Cardiac tamponade

428
Q

Non-hodgkins lymphoma affects either of which two cells?

A

B or T cells

429
Q

What are RFs for non-hodgkin’s lymphoma?

A 
Elderly
Caucasian
Hx viral infections, esp. EBV
FH
Certain chemicals - pesticides, solvents
Hx chemo/radio
Immunodeficiency, e.g. HIV, transplant, DM
Autoimmune dx (SLE, Sjogren's, coeliac dx)
430
Q

What are symptoms of NHL?

A 
Painless lympadenopathy (non-tender, rubbery, asymmetrical)
B symptoms
Extranodal dx (dyspepsia, dysphagia, wt loss, ab pain), pancytopenia, bone pain, nerve palsies etc.
431
Q

What are the B symptoms?

A

Fever, night sweats, wt loss, lethargy

432
Q

Are extranodal symptoms more common in NHL or HL?

433
Q

What are signs of NHL?

A

Wt loss
Lympadenopathy (typically cervical, axillary, inguinal)
Palpable ab mass - hepatomegaly, splenomegaly, LNs
Testicular mass
Fever

434
Q

What is the diagnostic investigation for NHL?

A

Excisional node biopsy

435
Q

What is the classical appearance of Burkitt’s lymphoma on biopsy?

A

Starry sky appearance

436
Q

What is used to stage NHL?

A

CT chest, abdo, pelvis

437
Q

Testing for what virus is often done on diagnosis of NHL?

A

HIV (RF for NHL)

438
Q

What blood tests may be done in NHL?

A

FBC + blood film (rule out other haematological malignancy)
ESR (prognostic indicator)
LDH (marker of cell turnover - prognostic indicator)
Other investigations based on symptoms, e.g. LFTs if suspecting liver mets

439
Q

What are infective causes of lympadenopathy?

A 
IM
HIV
Eczema with secondary infection 
Rubella
Toxoplasmosis
CMV
TB
Roseola infantum
440
Q

What are neoplastic causes of lymphadenopathy?

A

Leukaemia

Lymphoma

441
Q

What are other causes of lymphadenopathy?

A

Autoimmune conditions - SLE, RA
Graft vs Host disease
Sarcoidosis
Drugs, e.g. phenytoin

Haematology (14 decks)

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