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Phase 4 > Haematology > Flashcards

Haematology Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

malignant clonal proliferation of lymphoblastic cells (most commonly B cells)

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2
Q

What is the most common childhood cancer?

A

ALL - most common <5 years

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3
Q

What are 3 risk factors for ALL?

A

Down’s
Kleinfelter’s syndrome
Fanconi anaemia

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4
Q

what are 5 poor prognostic factors for ALL?

A

<2 years or >10 years
WBC >20
T or B cell surface markers
Non-Caucasian
Male

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5
Q

what is the management of ALL?

A

4-8 weeks corticosteroids, vincristine, doxorubicin

Up to 1 year of high dose chemo started after remission

2 years of mercaptopurine and methotrexate after remission

Bone marrow transplant

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6
Q

What are 9 presentations of leukaemia?

A

Fatigue
Fever
Pallor
Petechiae/bruising/bleeding
Frequent infections
Bone pain
Lymphadenopathy
Hepatosplenomegaly
Failure to thrive

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7
Q

what are 7 general investigations for leukaemia?

A

FBC - within 48h
Blood film
Lactate dehydrogenase
Bone marrow biopsy
CT/PET staging
Lymph node biopsy
Genetics and immunophenotyping

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8
Q

where is a bone marrow biopsy taken from?

A

iliac crest

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9
Q

what is the gold standard investigation for leukaemia?

A

bone marrow aspiration and biopsy

> 20% lymphoblasts/myeloblasts in bone marrow is diagnostic

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10
Q

what is the treatment for ALL?

A

Chemo - vincristine + corticosteroids
imatinib - if Philadelphia +ve
mercaptopurine + methotrexate => 2 years maintenance

bone marrow transplant

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11
Q

What is acute myeloid leukaemia?

A

the clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extramedullary tissues.

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12
Q

what are 5 risk factors for AML?

A

65+
previous chemo/radiation
Down’s syndrome
benzene - painters, petroleum, rubber
Myeloproliferative disorders

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13
Q

which leukaemia is associated with DIC?

A

AML - abnormal promyelocytes release granules which can cause thrombocytopenia

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14
Q

what is the most common leukaemia in Downs?

A

AML

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15
Q

what is the pathophysiology of AML?

A

Accumulation of myeloid blasts unable to differentiate into mature neutrophils, RBCs or platelets resulting in bone marrow failure

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16
Q

what is characteristically seen on blood smear in AML?

A

Auer rods

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17
Q

what is the management of AML?

A

Cytarabine and an anthracycline (daunorubicin)

All-trans retinoid acid - promyelocytic leukaemia

stem cell transplant

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18
Q

what is the AML classification system?

A

French-American-British (FAB) classification

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19
Q

what is the prognosis for AML?

A

high incidence of relapse

5 year survival - 25%

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20
Q

what is reticulocyte count?

A

measure of immature RBCs

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21
Q

What are 5 causes of microcytic anaemia?

A

TAILS

Thalassaemia
Anaemia of chronic disease
Iron deficiency
Lead Poisoning
Sideroblastic anaemia

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22
Q

what are 5 causes of normocytic anaemia?

A

AAAHH

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia - CKD, bone marrow suppression
Haemolytic anaemia
Hypothyroidism

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23
Q

what are 7 causes of macrocytic anaemias?

A

FAT RBC

Foetus - pregnancy
Alcohol - normoblastic
Thyroid disease - hypo

Reticulocytosis - haemolytic anaemia/blood loss
B12 and Folate deficiency - megaloblastic
Cirrhosis and liver disease

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24
Q

what are 3 causes of macrocytic megaloblastic anaemia?

A

B12 deficiency
Folate deficiency
Meds - methotrexate

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25
Q

what are 7 causes of macrocytic normoblastic anaemia?

A

Alcohol
Liver disease
Hypothyroidism
Pregnancy
Reticulocytosis
Myelodysplasia
Drugs - cytotoxic

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26
Q

what are 6 symptoms of anaemia?

A

often asymptomatic

Fatigue/faintness
headache, confusion, dizziness
SOB
angina/palpitations/claudication

Pica - abnormal cravings - IDA only
Hair loss - IDA only

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27
Q

what is the reference range for MCV?

A

80-100 femtolitres

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28
Q

What are the WHO the reference ranges for Hb?

A

Women - 120-165 g/litre
Men - 130-180 g/Litre

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29
Q

where is B12 found?

A

Meat
fish
dairy
eggs
NO PLANTs

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30
Q

how long do B12 stores last?

A

4 years

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31
Q

what are 3 causes of B12 deficiency anaemia?

A

diet
malabsorption
pernicious anaemia (most common)

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32
Q

How is B12 absorbed?

A

combines with INTRINSIC FACTOR secreted from PARIETAL CELLS in stomach and absorbed in TERMINAL ILEUM

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33
Q

why is B12 necessary?

A

needed for DNA and thymine synthesis

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34
Q

what are 7 neurological complications of B12 deficiency?

A

Loss of cutaneous sensation
loss of mental/physical drive
Muscle weakness
Optic neuropathy
Psychiatric disturbance
Symmetrical neuropathy affecting legs more than arms
Urinary/faecal incontinence

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35
Q

what is the name of the test for measuring total B12?

A

serum cobalamin

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36
Q

what can be seen on blood film in B12/folate deficiency?

A

Oval macrocytes, hypersegmented neutrophils and circulating megaloblasts in the blood film, as well as megaloblastic change in the bone marrow, are typical features of clinical cobalamin deficiency.

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37
Q

what are 4 conditions associated with pernicious anaemia?

A

Thyroid disease
Vitiligo
Stomach Cancer
Addisons

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38
Q

what is the test for pernicious anaemia?

A

anti-intrinsic factor anybody test

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39
Q

what is pernicious anaemia?

A

autoimmune destruction of parietal cells leading to B12 deficiency anaemia

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40
Q

what are 3 risk factors for B12 anaemia?

A

vegan
history of GI surgery
H.pylori infection

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41
Q

what are 3 differentials for B12 anaemia?

A

B9 deficiency
myelodysplatic syndrome
alcoholic liver disease

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42
Q

what is the treatment for B12 anaemia?

A

oral cyanocobalamin 50-150mg OD

IM hydroxocobalamin 1g 2x yearly

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43
Q

what are 3 complications of B12 anaemia?

A

neurological deficits
neural tube defects
optic atrophy and psychiatric symptoms

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44
Q

What is chronic lymphocytic leukaemia?

A

a malignant monoclonal proliferation of B lymphocytes

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45
Q

what is the most common leukaemia in adulthood?

A

CLL

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46
Q

what are 3 risk factors for CLL?

A

70+
male
FHx

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47
Q

what is seen on blood smear in CLL?

A

Smudge cells

also spherocytes and polychromasia

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48
Q

what are 3 treatment options for CLL?

A

monitoring - early stage

Chemo - FCR, chlorambucil and rituximab

stem cell transplant

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49
Q

what are 3 CLL specific complications?

A

Richter’s transformation to high grade B-cell lymphoma

Warm autoimmune haemolytic anaemia

Hypogammaglobulinaemia

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50
Q

what is the rule of 3s in CLL?

A

1/3 don’t progress
1/3 progress slowly
1/3 progress actively

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51
Q

what is chronic myeloid leukaemia?

A

the malignant proliferation of partially mature myeloid cells (especially granulocytes) in bone marrow and blood

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52
Q

what are the 3 phases of CML?

A

Chronic - usually asymptomatic, can last several years before progressing

Accelerated - blasts increase leading to pancytopenia

Blast phase - >20% blasts, severe symptoms often fatal pancytopenia

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53
Q

what gene is related to CML??

A

the Philadelphia chromosome

on chromosome 22
Associated with BCR-ALB1

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54
Q

what leukaemia is the Philadelphia gene related to?

A

CML

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55
Q

when is the peak incidence of CML?

A

65-75

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56
Q

what is a known risk factor for CML?

A

ionising radiation

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57
Q

what is the gold standard for CML?

A

presence of Philadelphia chromosome - cytogenetics/FISH

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58
Q

what is the treatment for CML?

A

1 - Imatinib - tyrosine kinase inhibitor

Hydroxyurea
Interferon alpha

Bone marrow transplant

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59
Q

what is the prognosis for CML?

A

75% 5 year survival

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60
Q

what is folate (B9) present in?

A

green vegetables
legumes
some fruits
yeast
liver
nuts

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61
Q

how long do folate stores last?

A

4 months

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62
Q

where in folate absorbed?

A

proximal jejunum/duodenum

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63
Q

what are 4 risk factors for folate deficiency?

A

elderly/young
poverty
chron’s/coeliac
pregnant

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64
Q

what is folate needed for?

A

DNA synthesis and repair

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65
Q

what are 7 clinical features of folate (B9) and B12 deficiency?

A

anaemia symptoms - SOB, Headache, cognitive changes, weakness
Glossitis - red smooth shiny tongue
Oropharyngeal ulceration
Diarrhoea
Heart murmur
Mild jaundice
Pallor of mucous membranes or nail beds

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66
Q

What is the management of folate deficiency anaemia?

A

oral folic acid 5mg OD for 4 months

ALWAYS CHECK B12 and replace if low - replacing folate can mark low b12 and allow neurology to develop

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67
Q

what are 3 differentials of folate deficiency?

A

B12 deficiency
hypothyroidism
alcoholic liver disease

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68
Q

what are 2 complications of folate deficiency?

A

pregnancy complications (prematurity)
Cardiovascular disease

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69
Q

what is G6PD deficiency?

A

Glucose-6-phosphate dehydrogenase deficiency

X-linked condition causing haemolytic anaemia due to susceptibility of RBCs to free radicals
common in middle eastern, Mediterranean and African populations

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70
Q

what are 3 triggers for haemolysis in G6PD deficiency?

A

Infection
Medications
Broad (fava) beans

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71
Q

what are 6 medications that can trigger haemolysis in G6PD?

A

Primaquine - antimalarial
Ciprofloxacin
Nitrofurantoin
Trimethoprim
Sulfonylureas (gliclazide)
Sulfasalazine

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72
Q

what are 5 presentations of G6PD?

A

jaundice - can be neonatal
Anaemia
Intermittent jaundice - in response to triggers
Gallstones
Splenomegaly

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73
Q

what ca be used to diagnose G6PD?

A

G6PD enzyme assay - checked 3 months after acute episode of haemolysis

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74
Q

what can be seen on blood film in G6PD deficiency?

A

Heinz bodies - blobs of denatured haemoglobin within RBCs
Bite and blister cells

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75
Q

what are 3 inherited causes of haemolytic anaemia?

A

RBC membrane defects (spherocytosis, elliptocytosis)
Enzyme defects (G6PD)
haemoglobinopathies (thalassaemia, sickle cell)

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76
Q

what are 5 acquired causes of haemolytic anaemia?

A

Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia (transfusion reaction, haemolytic disease of newborn)
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related Haemolysis

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77
Q

what are 4 risk factors for haemolytic anaemia?

A

autoimmune disorders
FHx
drugs
prosthetic heart valves

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78
Q

what are 4 manifestations of haemolytic anaemia?

A

anaemia - pallor, fatigue, dizzy, SOB
Jaundice
Dark urine
Splenomegaly

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79
Q

what is the gold standard investigation for autoimmune haemolytic anaemia?

A

Coombs test (direct antiglobulin test)

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80
Q

what is seen on blood film in haemolytic anaemia?

A

Schistocytes - fragments of RBCs

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81
Q

what are the 2 different types of autoimmune haemolytic anaemia?

A

Warm - more common, Haemolysis occurs at normal or higher temperatures

Cold - haemolysis occurs at <10 degrees after RBCs agglutinate due to antibodies attaching

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82
Q

what are 5 conditions that cold autoimmune haemolytic anaemia can be associated with?

A

Lymphoma
Leukaemia
SLE
Infection - mycoplasma, EBV, CMV, HIV

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83
Q

what is the treatment for autoimmune haemolytic anaemia?

A

Rituximab
Prednisolone
Blood transfusion
splenectomy

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84
Q

what is paroxysmal nocturnal haemoglobinuria?

A

mutation in haematopoietic stem cells in bone morrow causing loss of RBC surface proteins which inhibit complement cascade leading to RBC destruction

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85
Q

what is the classical presentation of paroxysmal nocturnal haemoglobinuria?

A

red urine in the morning - contains haemoglobin and haemosiderin

Anaemia
thrombosis
smooth muscle dystonia - oesophageal spasm, erectile dysfunction

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86
Q

what is the management of paroxysmal nocturnal haemoglobinuria?

A

Eculizumab - MAB targeting complement component 5
Bone marrow transplant

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87
Q

what is microangiopathic haemolytic anaemia?

A

destruction of RBCs due to abnormal activation of clotting cascade causing micro thrombi

associated with haemolytic uraemic syndrome, DIC, TTP, SLE, Cancer

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88
Q

What is Hodgkin’s lymphoma?

A

Uncommon haematological malignancy arising from mature B cells (lymphocytes)
Characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.

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89
Q

what are 4 risk factors for Hodgkin’s lymphoma?

A

EBV infection
FHx
HIV + immunosuppression
autoimmune conditions - RhA, Sarcoidosis

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90
Q

what is the age distribution for Hodgkin’s lymphoma?

A

Bimodal -
20-25 years
>80 years

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91
Q

what are 9 presentations of Hodgkin’s lymphoma?

A

Painless Lymphadenopathy

Hepatosplenomegally (less common than in NHL)

B symptoms - Fever, Weight loss, Night Sweats

Pruritus

Fatigue

Recurrent infections

SOB + cough

Abdo pain

Alcohol induced lymph node pain

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92
Q

what are 3 investigations for Hodgkin’s lymphoma?

A

FBC - anaemia, lymphopenia, thrombocytopenia, neutrophilia

PET/CT for staging

Bone marrow biopsy

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93
Q

what is the diagnostic investigation for Hodgkin’s lymphoma?

A

exicisional lymph node biopsy

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94
Q

what cells are seen on lymph node biopsy in Hodgkin’s lymphoma?

A

Reed-sternberg cells
- Owls eye appearance - huge multinucleated lymphocytes

Also:
Lacunar cells
popcorn cells - in nodular lymphocyte predominant hodgkin’s lymphoma

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95
Q

what are 3 differentials for Hodgkin’s lymphoma?

A

non-hodgkin’s
lymphoma
infectious mononucleosis
Other malignancy - head and neck cancer, breast cancer

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96
Q

what is the treatment for Hodgkin’s lymphoma?

A

Chemo - usually ABVD
- Adriamyacin
- Bleomycin
- Vinblastine
- Dacarbazine

Radiotherapy

bone marrow transplant

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97
Q

what are 4 complications for Hodgkin’s lymphoma?

A

Secondary malignancies
chemo related cardio toxicity
pulmonary toxicity
Superior vena cava syndrome

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98
Q

what staging is used in Hodgkin’s lymphoma?

A

Lugano classification

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99
Q

what are the Lugano classification stages?

A

For Hodgkin’s lymphoma

I - single lymph node/group
II - 2+ lymph nodes one side of diaphragm
III - nodes on both sides of diaphragm
IV - spread beyond lymph nodes

A/B => presence of B symptoms or not
E = extranodal disease
S = Spleen involvement
X = Bulky disease (large tumour)

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100
Q

what is the most common type of anaemia?

A

iron deficiency

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101
Q

what percentage of menstruating women have iron deficiency anaemia?

A

14%

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102
Q

what are 4 causes of iron deficiency?

A

low dietary iron
impaired absorption (coeliac)
increased iron loss (bleeding)
increased iron required (children, pregnant, lactating)

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103
Q

what are 6 risk factors for Iron deficiency anaemia?

A

pregnancy
veggie/vegan diet
Menorrhagia
hook worms
CKD
gasterectomy/NSAIDs

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104
Q

where is iron absorbed?

A

duodenum and jejunum

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105
Q

what is the treatment of iron deficiency anaemia?

A

oral iron replacement
- ferrous sulphate 200mg OD PO
- Ferrou fumarate 210 mg OD PO
for 3 months after iron deficiency is corrected

absorbic acid
IV iron - iron dextran/sucrose, ferruc carboxymaltose, ferric Deriso-maltose
Packed RBC - Hb <70

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106
Q

what are 4 reasons for poor absorption of iron?

A

Absorption - coeliac and crohns
Low stomach acid - PPIs, H. Pylori

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107
Q

what is the criteria for 2 week wait in IDA?

A

Over 60

Post menopausal women Hb <100
Men Hb <110

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108
Q

what are 5 side effects of iron supplementation?

A

Nausea
Abdo pain
constipation
diarrhoea
black stools

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109
Q

what are 7 complications of iron deficiency anaemia?

A

Cognitive impairment
Impaired muscular performance
High output heart failure
Lowered immunity
Increased maternal and foetal morbidity
Preterm delivery
Maternal postpartum fatigue

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110
Q

what is the top cause of iron deficiency worldwide?

A

hook worms

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111
Q

what is malaria?

A

a parasitic infection caused by protozoa of the Plasmodium family which is transmitted to humans by the bite of an anopheles mosquito

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112
Q

what plasmid is the most common cause of malaria in the UK?

A

Plasmodium falciparum

also the most severe

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113
Q

how is malaria transmitted?

A

by female anopheles mosquitos

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114
Q

what are 5 plasmids that cause malaria?

A

Palsmodium flaciparum - most common
plasmodium vivax - second most common
plasmodium ovale
plasmodium malariae
plasmodium knowlesi

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115
Q

what is the life cycle of a malaria plasmodium?

A

Plasmodium reproduces in mosquito’s gut producing thousands of sporozoites which are injected into whoever they bite

The sporozoites travel to liver - P. vivax and P. ovale can lay dormant for months to years

Sporozoites mature in liver to merozoites which enter blood and infect RBCs where they reproduce leading the RBCs to rupture causing haemolytic anaemia

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116
Q

what are the fevers like in P. vivax and p. ovale malaria infections?

A

Spikes every 48 hours - tertian malaria

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117
Q

what are the fevers like in P. falciparum malaria?

A

spikes more frequently and less regularly - Subtertian

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118
Q

what are the fevers like in p. malariae malaria?

A

spikes every 72 hours - quartan

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119
Q

what are 6 manifestations of malaria infection?

A

High fever, sweats and rigors
Fatigue
Myalgia
Headache
nausea and vomiting

Pallor - anaemia
Hepatosplenomegaly
Jaundice - haemolysis

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120
Q

what are 2 investigations for malaria?

A

Giemsa stain blood film - gold

rapid diagnostic test
thin and thick blood smear

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121
Q

what is required to rule out malaria?

A

3 negative samples from 3 consecutive days

due to life cycle of release of merozoites

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122
Q

what is the management of uncomplicated malaria?

A

arthemether + lumefantrine
OR
Quinine + Doxycycline/clindamycin
OR
Proguanil + atovaquone
OR
Chloroquine - high resistance
OR
Primaquine

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123
Q

what is a contraindication to administration of primaquine?

A

G6PD deficiency - causes severe haemolysis

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124
Q

what is the management of severe/complicated malaria?

A

1 - IV artesunate

IV artemether
IV quinine dihydrochloride

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125
Q

what are 8 complications of malaria?

A

Cerebral malaria
seizures
reduced consciousness
AKI
pulmonary oedema
DIC
Severe haemolytic anaemia
Multi-organ failure

Death

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126
Q

what is the advice for travelling to malaria endemic areas?

A

No method 100% effective alone
Mosquito spray - 50% DEET
Mosquito nets and barriers
Seek medical advice if symptomatic
take antimalarials

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127
Q

what are 4 prophylactic antimalarials?

A

proguanil with atovaquone (malarone) - 2 days before to 7 days after

Doxycycline - 2 days before to 4 weeks after

Mefloquine - risk of psychiatric side effects - 2 weeks before to 4 weeks after

Chloroquine with proguanil - high resistance

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128
Q

what is multiple myeloma?

A

cancer of plasma cells (differentiated b lymphocytes) which causes production of large quantities of specific paraprotein (M protein), an abnormal antibody or part antibody

Multiple when affecting multiple bone marrow areas of the body

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129
Q

what is MGUS?

A

Monoclonal gammopathy of undetermined significance

production of a specific paraprotein without the features of myeloma

1% risk per year of development into myeloma

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130
Q

what is smouldering myeloma?

A

abnormal plasma cells and paraproteins without organ damage or symptoms - 10% risk of progression to myeloma per year

Monoclonal protein in serum >30 g/L
or urine >500mg/24h
or clonal bone marrow plasma cells (10-60%)

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131
Q

what are 5 risk factors for multiple myeloma?

A

MGUS - monoclonal gamopathy of undetermined significance
Smouldering myeloma
Increasing age
FHx
African ancestry

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132
Q

what is the pathophysiology of multiple myeloma?

A

There is genetic mutation in a b cell leading to production of clonal plasma cells which overproduce antibodies (immunoglobulins)

These abnormal immunoglobulins produced are called paraproteins or M proteins leading to abnormally high levels of paraprotein in the blood (paraproteinaemia)

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133
Q

what is the most common Ig produced in multiple myeloma?

A

IgG - 55%

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134
Q

what are the 3 stages of multiple myeloma?

A

1 - low levels of beta-2 migroglobulin <3.5mg/L and normal albumin

2 - neither stage 1 or 3

3 - High levels of beta-2 microglobulin >5.5 mg/L

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135
Q

what are 6 manifestation of multiple myeloma?

A

Old CRAB

  • Age 65+
  • Calcium - high
  • Renal failure - due to light chain deposition in renal tubules
  • Anaemia - bone marrow infiltration - normocytic, normochromic
  • Bone pain/Bleeding

infections
fatigue
pathological fractures
weight loss
fever

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136
Q

what causes hypercalcaemia in multiple myeloma?

A

1 - Myeloma bone disease - Cytokines released from abnormal plasma cells leads to increased osteoclastic activity leading to bone resorption, osteolytic lesions and pathological fractures

Also Renal failure - reduced excretion

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137
Q

what causes renal disease in multiple myeloma?

A

Paraproteins deposited in renal tubules
Hypercalcaemia affects kidney function
Dehydration
Glomerulonephritis
Medications

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138
Q

what is hyper viscosity syndrome?

A

Increased plasma viscosity due to increased proteins in the blood

In multiple myeloma due to paraproteins

Causes bleeding, visual symptoms and eye changes, neurological complications (stroke), heart failure

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139
Q

what are 8 investigations for multiple myeloma?

A

Urine bence-jones protein electrophoresis test

Serum protein electrophoresis

Serum-free light-chain assay

FBC
serum calcium
ESR + plasma viscosity - raised
U+E

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140
Q

What is needed to confirm a diagnosis of multiple myeloma?

A

Bone marrow biopsy

Assess bone lesions with whole body MRI/low dose CT/skeletal survey

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141
Q

what is the diagnostic criteria for multiple myeloma?

A

Clonal bone marrow plasma cells >10 or biopsy proven plasmacytoma

PLUS one of: SLIM CRAB

S - >60% plasma cells in marrow
LI - Involved:uninvolved Light chain ration >100
MRI focal lesions 2+ >5mm

C - Hypercalcaemia
Renal insufficiency
Anaemia <100
Bone lesion x1 on x-ray/CT >5mm

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142
Q

what are 3 differentials for multiple myeloma?

A

MGUS
smouldering myeloma
amyloidosis

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143
Q

what 3 things can be seen on X-ray in multiple myeloma?

A

Well defined lytic lesions - punched out
Diffuse osteopenia
Abnormal fractures

Lytic lesions in skull may be referred to a raindrop or pepper pot skull

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144
Q

Is multiple myeloma curable?

A

NO - has chronic relapsing remitting course

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145
Q

what is the treatment for multiple myeloma?

A

Good function, <70 -
Bortezomib +
Dexamethasone +/-
Thalidomide +
Stem cell transplant

Poor function, >70 -
Bortezomib +
Prednisolone +
Melphalan

Bortezomib - 1st for relapse

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146
Q

what is the management of bone disease in multiple myeloma?

A

Bisphosphonates - Zoledronic acid 1st line

radiotherapy
ortho surgery

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147
Q

what are the 2 different types of stem cell transplant?

A

Autologous - using own stem cells
Allogenic - using donor cells

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148
Q

what are 6 complications of multiple myeloma?

A

recurrent infections
Fatigue
pathological fractures
renal failure
anaemia
hypercalcaemia

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149
Q

what is non-Hodgkin’s lymphoma?

A

Malignant proliferations of B or T lymphocytes without reed-Sternberg cells

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150
Q

what are 3 different types of Non-Hodgkin’s lymphoma?

A

Diffuse large B cell lymphoma - rapidly growing painless mass in older men

Burkitt lymphoma - particularly associated with EBV

MALT lymphoma - affects mucosa-associated lymphoid tissue usually around stomach

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151
Q

What are 6 risk factors for non-Hodgkin’s lymphoma?

A

FHx
>60 years
EBV infection
Hep B/C
H. Pylori - associated with MALT lymphoma
Autoimmune disease

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152
Q

are non-hodgkin’s lymphomas more commonly of T or B cell origin?

A

B cells - 80-90%

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153
Q

what are 6 presentations of non-hodgkin’s?

A

Painless lymphadenopathy

B symptoms - weight loss, fever, night sweats

Mass effect symptoms - SOB, cough, Jaundice, hydronephrosis, vomiting and constipation

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154
Q

what are 3 investigations of nom-hodgkin’s lymphoma?

A

FBC with differential - thrombocytopenia, pancytopenia, lymphocytosis
blood smear
excision lymph node biopsy

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155
Q

How is nodal spread in Hodgkin’s versus non-Hodgkin’s lymphoma?

A

Contiguous in Hodgkin’s lymphoma

non contiguous in Non-Hodgkin’s lymphoma

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156
Q

what is the 2ww criteria for lymphadenopathy?

A

> 6 weeks lymphadenopathy
1+ nodes >2cm diameter
Rapidly increasing lymphadenopathy
Generalised lymphadenopathy
Persistent and unexplained splenomegaly

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157
Q

what are 3 differentials for non-hodgkin’s lymphoma?

A

Hodgkin’s lymphoma
ALL
infectious mononucleosis

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158
Q

what is the management of non-hodgkin’s lymphoma?

A

chemo - R-CHOP
- Rituximab
- cyclophosphamide
- Doxorubicin
- Vincristine
- prednisolone

radiotherapy
Rituximab
stem cell transplant

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159
Q

what are 3 complications of non-hodgkin’s lymphoma?

A

impaired immunity
myelosuppression and neutropenic sepsis
tumour lysis syndrome
risk of secondary malignancy

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160
Q

what are 2 causes of relative polycythaemia?

A

Dehydration
Stress - Gaisbock syndrome

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161
Q

what are 4 secondary causes of polycythaemia?

A

COPD
Altitude
OSA
Excessive EPO - cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids

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162
Q

what is Polycythaemia vera?

A

a myeloproliferative disorder of clonal proliferation of erythrocytes often accompanied by overproduction of neutrophils and platelets

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163
Q

what genetic mutation if associated with Polycythaemia vera?

A

JAK2 mutation - 95% of patients

164
Q

what are 7 presentations of Polycythaemia vera?

A

Headache
Pruritus - especially after hot bath
splenomegaly
Facial flushing
Palmar erythema
Plethroic appearance
Hypertension

165
Q

what are 4 first line investigations of Polycythaemia vera?

A

FBC - elevated Hb, neutrophils, basophils, platelets
U+E +LFTS
Serum ferritin
JAK2 mutation

166
Q

what are 4 treatments of Polycythaemia vera?

A

1 - Venesection

Aspirin 75mg OD - reduce risk of thrombotic events

Hydroxycarbamide - 1st line cytoreductive to reduce thrombosis risk in high risk

Ruxolitinub - 2nd line cytoreductive

167
Q

what are 5 complications of Polycythaemia vera?

A

Thrombosis
Haemorrhage
Leukaemia - 1-3% risk of progression
Treatment induced leukaemia
Myelofibrosis

168
Q

what is myelofibrosis?

A

myelodysplastic disorder thought to be due to hyperplasia of abnormal meakaryocytes.

Characterised by bone marrow fibrosis and extramedullar haematopoiesis often with splenomagaly

169
Q

what is seen on FBC in myelofibrosis?

A

Anaemia
high WCC
High platelets

170
Q

what can be seen on blood film in myelofibrosis?

A

Tear drop poikilocytes

171
Q

what are 6 presentations of myelofibrosis?

A

Severe fatigue
Hepatosplenomegaly
B symptoms
Thromboembolic events
Unexplained bleeding

172
Q

what may be seen on bone marrow biopsy in myelofibrosis?

A

‘dry tap’ without aspirate

173
Q

what is the management of myelofibrosis?

A

Ruxolitinib - JAK 2 inhibitor

Hydroxyurea

174
Q

what are 7 complications of myelofibrosis?

A

Haemorrhage
infection
portal hypertension
splenic infarct
gout and kidney stones due to raised urea
neurological manifestations
thrombotic events
osteosclerosis

175
Q

what is essential thrombocytosis?

A

myeloproliferative disorder of excessive mature platelet production

176
Q

what is the presentation of essential thrombocytosis?

A

Headache
Tingling in hands/feet
Fatigue
Visiual disturbance
bleeding gums, petechiae, easy bleeding
Thrombosis
Splenomegaly

177
Q

what is seen on peripheral blood smear in essential thrombocytosis?

A

platelet anisocytosis (different sizes)

178
Q

what is the management of essential thrombocytosis?

A

Aspirin - thrombosis prophylaxis

Hydroxycarbamide

Interferon alpha - in younger patients and women of childbearing age

179
Q

what is the inheritance of sickle cell anaemia?

A

Autosomal recessive

point mutation substituting hydrophilic glutamic acid for hydrophobic valine

180
Q

what is the pathophysiology of sickle cell anaemia?

A

there is a genetic abnormality which affects the gene for beta-globin on chromosome 11 leading to rather than biconcave discoid RBC for them to be sickle shaped and more prone to haemolysis

181
Q

what chromosome if affected in sickle cell anaemia?

A

chromosome 11

182
Q

what is the screening for sickle cell anaemia?

A

All babies in newborn blood spot test

At risk pregnant women may be offered genetic testing

183
Q

what triggers sickling in sickle cell disease?

A

hypoxia
acidosis
dehydration
Cold temperatures
extreme exercise
stress
Infections

184
Q

when does sickle cell disease manifest and why?

A

6 months

There is a gradual transition between foetal haemoglobin and adult but by 6 months there is very little HbF produces and most RBCs contain HbA which is mutated to HbS in sickle cell

185
Q

what haemoglobin chain is affected in sickle cell anaemia?

A

Beta haemoglobin

186
Q

what is foetal haemoglobin made up of?

A

2 alpha chains and 2 gamma chains

187
Q

what is adult haemoglobin made up of?

A

2 alpha chains
2 beta chains

188
Q

what is the gold standard investigation for sickle cell anaemia?

A

haemoglobin electrophoresis

189
Q

what are 3 investigations for sickle cell?

A

blood film
Hb isoelectric focusing
peripheral blood smear
Newborn Guthrie heel prick test (5 days)

190
Q

what is the general management of sickle cell?

A

Avoid triggers
Vaccination
Antibiotic prophylaxis - phenoxymethylpenicillin
analgesia
oral hydroxycarbamide (increase foetal haem)
Crizanlizumab - prevents RBCs from sticking
blood transfusions - for anaemia
Bone marrow transplant
Folic acid suplementation

191
Q

what medication in sickle cell anaemia is used to increase foetal haemoglobin?

A

Hydroxycarbamide (usually 15mg/kg)

192
Q

what vaccination should those with sickle cell anaemia get every 5 years?

A

pneumococcal

193
Q

what is the management for sickle cell crisis?

A

Supportive

Low threshold for hospital admission
Tx trigger
keep warm
Good hydration
Analgesia

194
Q

what are 4 sickle cell crises?

A

Vaso-occlusive crisis
Splenic sequestration crisis
Aplastic crisis
Acute chest syndrome

195
Q

what is the management of splenic sequestration crisis in sickle cell anaemia?

A

blood transfusion
IV fluids

Splenectomy if recurrent

196
Q

what can be seen on FBC in splenic sequestration crisis?

A

increased reticulocyte count

197
Q

what virus can trigger aplastic crisis in sickle cell anaemia?

A

Parvovirus B19

198
Q

what is the presentation of acute chest syndrome?

A

Hx of sickle cell

Chest pain
cough
SOB
fever
hypoxia
Pulmonary infiltrates on CXR

199
Q

what is the management of Acute chest syndrome?

A

Analgesia
Resp support
Abx
Transfusion

200
Q

what is the presentation of an aplastic crisis?

A

Sudden fall in Hb

Bone marrow suppression causes reduced reticulocyte count

201
Q

what are 11 complications of sickle cell?

A

Anaemia
Increased risk of infection
CKD
Sickle cell crisis
Acute chest syndrome
Stroke
Avascular necrosis
Pulmonary HTN
Gallstones
Priapism

202
Q

what are the vitamin K dependant clotting factors?

A

X, IX, VII, II - 1972

203
Q

can warfarin be used in pregnancy?

A

NO it’s teratogenic

204
Q

what is the name of the histological feature that appears like stacked coins?

A

Rouleaux formation

205
Q

what disease do you see rouleaux formation in?

A

multiple myeloma

stacked coin appearance of RBCs due to high levels of immunoglobulins causing cells to stick together

206
Q

what are 5 generic signs of anaemia?

A

pale skin and conjunctiva
tachycardia
bounding pulse
raised resp rate
postural hypotension

207
Q

what are 5 signs of iron deficiency anaemia?

A

koilonychia - spoon shaped nails
angular chelitis
atrophic glossitis - smooth large tongue
Brittle hair and nails
Pica

208
Q

what is a sign of haemolytic anaemias?

A

jaundice

209
Q

what is a sign of thalassaemia?

A

bone deformities

210
Q

what are 8 investigations for anaemia?

A

FBC - Hb and MCV
Blood film
Reticulocyte count
Ferritin
B12 and folate
Bilirubin - up in haemolysis
Direct Coombs test - autoimmune haemolytic
Haemoglobin electrophoresis

211
Q

what are the 4 iron studies?

A

serum iron
serum ferritin
total iron binding capacity (increased in anaemia)
transferrin saturation

212
Q

what is total iron binding capacity?

A

the total space available for ferric ions (Fe3+) to bind to transferrin molecules

213
Q

what is the formula for transferrin saturation?

A

serum iron / total iron binding capacity

214
Q

what is normal transferrin saturation?

A

30%

215
Q

what affects ferritin levels?

A

inflammation can lead to raised serum ferritin => A patient with normal ferritin can still have IDA

216
Q

what is hereditary spherocytosis?

A

condition where RBCs are sphere shaped making hem fragile and easily destroyed in spleen

common in northern europeans

217
Q

what is the inheritance pattern of hereditary spherocytosis?

A

majority autosomal dominant

218
Q

what are 6 presentation of hereditary spherocytosis?

A

jaundice
anaemia
gallstones
splenomegally
haemolytic crisis - triggered by infection
Aplastic crisis - usually triggered by parvovirus

219
Q

what are 2 investigations for hereditary spherocytosis?

A

EMA binding test
Cryohaemolysis test

not needed if there is FHx and typical features

220
Q

what is the management of hereditary spherocytosis?

A

phototherapy or exchange transfusion - neonates with jaundice

folic acid supplementation

Splenectomy

221
Q

what are 3 complications of hereditary spherocytosis?

A

gallstones
aplastic crisis
bone marrow expansion

222
Q

what are 4 precipitators of G6PD deficiency?

A

Fava beans
soy
red wine
infections

223
Q

what does the urine look like in G6PD deficiency?

A

tea coloured

224
Q

what is aplastic anaemia?

A

stem cell disorder characterised by pancytopenia

225
Q

what are 4 causes of aplastic anaemia?

A

radiation and toxins
drugs
infections
fanconi’s anaemia

226
Q

what condition are Howell-jolly bodies seen in?

A

Sickle cell

227
Q

what is a histological sign go sickle cell disease?

A

Howell-jolly bodies

228
Q

what is the most common cause of osteomyelitis in sickle cell crisis?

A

salmonella

229
Q

what do Reed-Sternberg cells look like?

A

owl eye nuclei

230
Q

what is found in the urine in multiple myeloma?

A

bench-jones proteins - Ig light chains

231
Q

what is found on the serum electrophoresis in multiple myeloma?

A

monoclonal paraprotein band

232
Q

what is the treatment of severe malaria?

A

Artemisinin combination therapy (ACT)

quinine

233
Q

what is the inheritance pattern for haemophilia?

A

X-linked recessive

234
Q

which factor is there a deficiency in in haemophilia A?

A

VIII - 8

235
Q

which factor is there a deficiency in in haemophilia B?

A

IX - 9

236
Q

what are 7 clinical manifestations of haemophilia?

A

Abnormal, prolonged bleeding
Easy bruising (ecchymosis)
Spontaneous haemorrhage
Muscular haematomas
Hemarthrosis
Epistaxis
GI symptoms

237
Q

what is needed for diagnosis of haemophilia?

A

Bleeding scores
coagulation factor assays
genetic testing

238
Q

what clotting time will be prolonged in haemophilia?

A

activated partial thromboplastic time - aPTT

Prothrombin time (PT) will be normal

239
Q

what are 3 differentials for haemophilia?

A

Von Willebrand disease
Platelet dysfunction disorders
Scurvy

240
Q

what is the management for haemophilia?

A

IV infusion of clotting factor

desmopressin in mild haemophilia A as promotes vWF

Lifestyle changes, RICE for MSK, analgesia, physio

241
Q

what medications should be avoided in haemophilia?

A

Aspirin and NSAIDs - increase bleeding risk

242
Q

what are 3 complications of haemophilia?

A

Intracranial haemorrhage
Compartment syndrome
arthropathy from haemarthrosis

243
Q

what chromosome codes for von willebrand factor?

A

chromosome 12

244
Q

what are the 3 different types of von willebrand disease?

A

1 - partial deficiency
2 - reduced function of vWF
3 - complete deficiency

245
Q

what does von willebrand factor do?

A

Mediated platelet adhesion

Stabilises factor VIII

246
Q

what is the presentation of von Willebrand disease?

A

Bleeding gums
epistaxis
easy bruising
menorrhoagia
heavy bleeding

FAMILY HISTORY

247
Q

what is the most common bleeding disorder?

A

Von Willebrand disease

248
Q

what can be used to treat von willebrand disease?

A

1 - Desmopressin - stimulated release of vWF from endothelial cells
OR
Tranexamic acid

2 - von Willebrand factor infusion
OR
Factor VIII + vWF infusion

Hysterectomy may be required in very severe menstrual bleeding

249
Q

what is thalassaemia?

A

a genetic defect in the protein chains that make up haemoglobin leading to haemolytic anaemia

250
Q

what is the inheritance of thalassaemia?

A

Autosomal recessive

251
Q

what demographic is alpha thalassaemia most common in?

A

asian and African descent

252
Q

what demographic is beta thalassaemia most common in?

A

SE asian, Mediterranean and Middle Eastern descent

253
Q

what are 6 manifestations of thalassaemia?

A

Microcytic anaemia
Anaemia symptoms - fatigue, pallor, SOB, palpitations
Neonatal Jaundice
Gallstones
Splenomegaly
poor growth and development
Chipmunk faces - compensatory extramedullary haematopoiesis in skull

254
Q

what are 4 investigations for thalassaemia?

A

FBC
Iron studies
Haemoglobin electrophoresis
DNA testing

255
Q

why is there iron overload in thalassaemia?

A

Increased iron absorption in GI tract
Blood transfusions

256
Q

what are 6 complications of iron overload?

A

Liver cirrhosis
Hypogonadism
Hypothyroidism
Heart failure
Diabetes
Osteoporosis

257
Q

defects on what chromosome cause alpha thalassaemia?

A

chromosome 16

258
Q

what can all 4 alpha globulin chains in alpha thalassaemia cause?

A

in utero death

259
Q

what are the 3 different severities of beta thalassaemia?

A

thalassaemia minor (trait)
thalassaemia intermedia
thalassaemia major

260
Q

what is the clinical picture in beta thalassaemia minor?

A

carriers with mild microcytic anaemia - may have microcytosis without anaemia

261
Q

what is the clinical picture in beta thalassaemia intermedia?

A

2 defective or 1 defective and 1 deletion gene

leads to significant microcytic anaemia - may need occasional blood transfusion

262
Q

what is the clinical picture in beta thalassaemia major?

A

homozygous for deletion genes

Severe anaemia and failure to thrive in early childhood

May have bone changes

263
Q

what are 4 bone changes in thalassaemia major?

A

Frontal bossing
enlarged maxilla
depressed nasal bridge
protruding upper teeth

264
Q

what can be given to thalassaemia patients to prevent iron overload?

A

deferoxamine

265
Q

what are 5 complications of thalassaemia?

A

Heart failure
hypersplenism
aplastic crisis
iron overload
gallstones

266
Q

when are people screened for thalassaemia?

A

all women in pregnancy

267
Q

what can reverse heparin overdose?

A

protamine sulphate

268
Q

what is the antidote to warfarin?

A

vitamin K1

269
Q

What is the Philadelphia chromosome?

A

Translocation of a part of chromosome 9 to chromosome 22

270
Q

is primaquine safe in pregnancy?

A

NO

271
Q

what is given as tumour lysis prophylaxis in AML?

A

allopurinol

272
Q

what are the histological signs of G6PD?

A

bite cells
Heinz bodies
reticulocytes

273
Q

what antibiotic is contraindicated in G6PD?

A

trimethoprim

274
Q

What leukaemia is tumour lysis syndrome most common in?

A

AML

275
Q

what cells are seen in CLL?

A

smudge cells

276
Q

what cells are seen in ALL?

A

blast cells

277
Q

what is antiphospholipid syndrome?

A

acquired autoimmune disease that causes thrombophilia - blood to be hypercoagulable. increases risk of PE, DVT, Stroke, MI and MISCARRIAGE

278
Q

what autoimmune condition is associated with antiphopholipid syndrome?

A

SLE - 30% of people with SLE have antiphospholipid antibodies

279
Q

what skin finding can be seen in antiphospholipid syndrome?

A

livedo reticularis

lacey, net like discolouration - like mottling

280
Q

what 3 antibodies cause antiphospholipid syndrome?

A

anticardiolipin antibodies
Lupus anticoagulant
Anti-beta-2 glycoprotein I antibodies

281
Q

what is seen on clotting profile in antiphospholipid syndrome?

A

normal PT

paradoxical prolonged APTT

thombocytopenia is also common

282
Q

what is the management of antiphospholipid syndrome?

A

Primary prophylaxis - low dose Aspirin

Secondary prevention - Lifelong warfarin INR 2-3
LMWH + high dose Aspirin in pregnancy

If thromboembolic events despite warfarin - target increased to 3-4

283
Q

what is the inheritance pattern of haemochromatosis?

A

autosomal recessive mutations of HFE gene on both copies of chromosome 6

284
Q

what is haemochromatosis?

A

genetic condition due to mutation of human haemochromatosis protein gene on chromosome 6 causing iron overload

285
Q

what are 8 presentation so of haemochromatosis?

A

Chronic tiredness
Joint pain
Pigmentation of skin
testicular atrophy
erectile dysfunction
amenorrhoea
cognitive symptoms
hepatomegaly

usually presents >40 years can be later in women

286
Q

what are 6 causes of raised serum ferritin?

A

haemochromatosis
infection
chronic alcohol consumption
non-alcoholic fatty liver disease
hepatitis c
cancer

287
Q

what 2 investigations can be used for haemochromatosis?

A

Iron studies - transferrin saturation raised, raised ferritin, low total iron binding capacity

Genetic testing for HFE mutation

288
Q

what can be used to establish iron concentration in liver in haemochromatosis?

A

liver biopsy with perl’s stain - helps stage fibrosis and exclude other liver pathology

289
Q

what are 7 complications of haemochromatosis?

A

secondary diabetes
liver cirrhosis
endocrine and sexual problems - hypogonad, ED, amenorrhoea, reduced fertility
Cardiomyopathy and heart failure
Hepatocellular carcinoma
Hypothyroidism
Chondrocalcinosis - calcium pyrophosphate deposits in joints

290
Q

what is the management of haemochromatosis?

A

1- Venesection - initially weekly
- keep transferrin saturation <50% and serum ferritin <50 ug/l

2 - deferoxamine - iron chelation

291
Q

what stem cell do all blood cells differentiate from?

A

Multipotential haematopoietic stell cell - hemocytoblast

292
Q

what 2 cells does the multipotential haematopoietic stem cell differentiate into first?

A

Common myeloid progenitor

Common lymphoid progenitor

293
Q

what 2 cells does the common lymphoid progenitor differentiate into?

A

Natural killer cell - large granular lymphocyte

small lymphocyte

294
Q

what 2 cells does the small lymphocyte cell differentiate into?

A

T lymphocyte

b lymphocyte

295
Q

what do B lymphocytes differentiate into?

A

Plasma cells

Memory b lymphocytes

296
Q

what is the role of plasma cells?

A

Produce antibodies against specific antigens

297
Q

what 4 cells does the common myeloid progenitor cell differentiate into?

A

Megakaryocyte
Erythrocyte
Mast cell
Myeloblast

298
Q

what do megakaryocytes differentiate into?

A

Platelets

299
Q

what 4 cells do myeloblasts differentiate into?

A

Basophils
Neutrophils
Eosinophils
Monocytes

300
Q

what do monocytes differentiate into?

A

Macrophages

301
Q

What are 6 blood transfusion reactions?

A

Non-haemolytic febrile reaction
Minor allergic reaction
Anaphylaxis
Acute haemolytic reaction
Transfusion associated circulatory overload (TACO)
Transfusion related acute lung injury (TRALI)

302
Q

what is acute haemolytic transfusion reaction?

A

Due to ABO mismatch

Causes massive intravascular haemolysis due to RBC destruction by IgM antibodies

Symptoms begin minutes after transfusion - fever, abdo and chest pain, agitation, hypotension

303
Q

what is the management and 2 complication of acute haemolytic transfusion reaction?

A

management
- stop transfusion - check patient identity and blood products
- Repeat typing and cross matching
- Send blood for direct coombs test
- generous fluid resus
- inform lab

complications
- DIC
- renal failure

304
Q

what causes non-haemolytic febrile reaction?

A

antibodies reacting with white cell fragments in blood products and cytokines that have leaked in storage

causes fever and chills

stop/slow transfusion
give paracetamol
monitor

305
Q

what is the management of minor allergic reaction to blood transfusion?

A

Temporarily stop transfusion
Antihistamines
monitor

306
Q

what causes anaphylaxis in blood transfusion?

A

patient with IgA deficiency who have anti-IgA antibodies

307
Q

what is transfusion associated circulatory overload (TACO)?

A

Due to fluid overloading - causes pulmonary oedema

may also be hypertensive

308
Q

What is the management of transfusion associated circulatory overload?

A

Slow/stop transfusion
Consider IV loop diuretics - furosemide
O2

309
Q

what is Transfusion related acute lung injury (TRALI)?

A

Non-cardiogenic pulmonary oedema secondary to increased vascualr permeability caused by host neutrophil activation by donated blood

Causes hypoxia, pulmonary infiltrates on CXR, fever, hypotension - within 6 hours of transfusion

310
Q

what is the management of transfusion related acute lung injury?

A

Stop transfusion
Oxygen and supportive care

311
Q

what blood product is most likely to be contaminated with bacteria?

A

Platelets as stored at room temp

312
Q

Is prothrombin time affected by the intrinsic or extrinsic coagulation pathway?

A

extrinsic pathway

PT - Play Tennis Outside = extrinsic

INR is a standardised version of PT

313
Q

is activated partial thromboplastin time (APTT) affected by the intrinsic or extrinsic coagulation pathway?

A

intrinsic pathway

aPTT = Play Table Tennis inside = intrinsic

314
Q

what factors are involved in the intrinsic coagulation pathway?

A

XII
XI
IX
VIII

12, 11, 9, 8

315
Q

what factors are involved in the extrinsic coagulation pathway?

A

III
VII

5 + 7

316
Q

what factors are involved in the common coagulation pathway?

A

X
V
II
I
XIII

10, 5, 2, 1, 13

317
Q

what triggers the intrinsic coagulation pathway?

A

Contact with damaged endothelial surfaces

318
Q

what triggers the extrinsic coagulation pathway?

A

Tissue factor III activation via endothelial tissue damage to IIIa

319
Q

what is disseminated intravascular coagulation?

A

simultaneous coagulation and haemorrhage caused formation of thrombi which consume clotting factors (V, VIII) and platelets leading to bleeding

320
Q

what are 5 causes of DIC?

A

Sepsis
Malignancy
Trauma - major surgery, burns, shock, AAA dissection
Severe organ dysfunction - Liver disease
Obstetric complications - abruption, HELLP syndrome

321
Q

what is seen on bloods in DIC?

A

Low platelets
Prolonged aPTT, PT and bleeding time
Decreased fibrinogen
D-dimer - raised

322
Q

what is the management of DIC?

A

Tx underlying cause
Anticoagulation - low dose heparin - due to microthrombi

Blood product transfusion

323
Q

what are 4 complications of DIC?

A

Intracranial bleed
Life threatening haemorrhage
Multi-organ failure
Gangrene or digital loss - due to microthrombi

324
Q

what is the most common cause of thrombocytopenia?

A

Immune thrombocytopenia

325
Q

what is thrombocytopenia?

A

Platelets <150 x10^9/L

326
Q

what is the normal range for platelets?

A

150-450 x10^9 /L

327
Q

what are 8 causes of thrombocytopenia due to reduced platelet production?

A

Viral infections - EBV, CMV, HIV
B12/folate deficiency
Liver failure + alcohol abuse- reduced thrombopoietin production
Bone marrow infiltration - leukaemia
Myelodysplastic syndrome
Chemotherapy
Genetics - wiskott-aldrich syndrome, faconi anaemia

328
Q

what are 6 causes of thrombocytopenia due to increased platelet destruction?

A

Medications - sodium valporate, methotrexate
Alcohol
Immune thrombocytopenic purpura
Thrombotic thrombocytopenia purpura
Heparin induced thrombocytopenia
Haemolytic uraemic syndrome

329
Q

What low platelet count is usually symptomatic?

A

<50 x10^9/L

330
Q

At what platelet count is there high risk for spontaneous bleeding?

A

<10 x10^9/L

331
Q

what are the top 4 differentials for abnormal bleeding?

A

thrombocytopenia
von willebran disease
Haemophillia
Disseminated intravascular coagulation

332
Q

what is wiskott-aldrich syndrome?

A

X-linked condition - eczema, thrombocytopenia, immunodeficiency

333
Q

what is faconi aanemia?

A

inherited bone marrow failure syndrome, characterised by pancytopenia

334
Q

what are 4 drugs that can induce thrombocytopenia?

A

Heparin
Quinine
Sulfonamides
Naproxen

335
Q

what is immune thrombocytopenia purpura?

A

Autoimmune condition where autoantibodies are created against platelets leading to their destruction and ultimately thrombocytopenia

336
Q

what is the course of ITP usually in children?

A

Usually triggered by recent viral infection and resolves typically within 6 months but in adults is usually more chronic

337
Q

what is primary versus secondary ITP?

A

Primary - no clear cause
Secondary - antibody medicated due to SLE, CLL, Drugs, Viruses, Pregnancy

338
Q

How is immune thrombocytopenia purpura diagnosed?

A

Diagnosis of exclusion

1 - FBC, peripheral blood smear

Bone marrow aspiration, blood borne virus serology

339
Q

what is the management of immune thrombocytopenia purpura?

A

1 - Prednisolone 1mg/kg OD reducing dose

Adjunct - IV immunoglobulins

With significant bleeding - Platelet transfusion 4-6 units of pooled platelets

2 - Splenectomy - if not responding to medical management
Immunosuppression - rituximab

340
Q

what is thrombotic thrombocytopenic purpura?

A

A microangiopathy where tiny thrombi develop in small vessels, consuming platelets and leading to thrombocytopenia

341
Q

deficiency of what protein causes thrombotic thrombocytopenic purpura?

A

ADAMTS13

342
Q

what is the role of ADAMTS13?

A

Inactivated von willebrand factor
Reduces platelet adhesion to vessel walls
reduces clot formation

343
Q

what can cause ADAMTS13 deficiency?

A

Inherited genetic mutation
Autoimmune disease against protein

344
Q

what is the classic pentad of thrombotic thrombocytopenic purpura ?

A

Thrombocytopaenia purpura
Microangiopathic haemolytic anaemia
Neurological dysfunction
Renal dysfunction
Fever

345
Q

what is the management of thrombotic thrombocytopenic purpura ?

A

1 - Plasma exchange
1 - orticosteroids
1 - Caplacizumab

2 - Rituximab

346
Q

what is heparin induced thrombocytopenia?

A

Development of autoantibodies to platelets in response to heparin (either UH or LMWH)

Heparin induced autoantibodies target platelet factor 4 protein on platelets

347
Q

what is the presentation of heparin induced thrombocytopenia?

A

5-10 days post starting heparin

HIT antibodies bind to platelets and activate clotting system

Hypercoagulability - DVT, PE
Thrombocytopenia - bleeding

348
Q

How is heparin induced thrombocytopenia diagnosed?

A

Testing serum heparin induced thrombocytopenia antibodies

349
Q

what anticoagulation can be used as an alternative to heparin in heparin induced thrombocytopenia?

A

Fondaparinux or argatroban

350
Q

what is the MOA of aspirin?

A

COX-1 (and 2) inhibitor

Cyclooxygenase is responsible for prostaglandin, prostacyclin and thromboxane synthesis - blocking thromboxane formation in platelets reduces ability to aggregate

351
Q

what is the secondary prevention dose of aspirin?

A

75mg OD

352
Q

what is the loading dose of aspirin in MI/Stroke?

A

300mg

353
Q

what are 4 examples of P2Y12 inhibitors?

A

Clopidogrel
Parasugrel
Ticagrelor
Ticlopidine

354
Q

what medications can reduce the efficacy of clopidogrel?

A

PPIs - especially omeprazole and esomeprazole

355
Q

what is the reversal agent for ticagrelor?

A

Bentracimab

356
Q

what is the MOA of warfarin?

A

Vitamin K antagonist

357
Q

what is the normal warfarin target?

A

Between 2-3

Aim 2.5

358
Q

what are 5 contraindications to warfarin?

A

liver disease
p450 enzyme inhibitors/inducers
cranberry juice
drugs which displace warfarin from plasma albumin - NSAIDs
Drugs that inhibit platelet function

359
Q

what are 7 cytochrome P450 inducers?

A

Antiepileptics - phenytoin, carbamezapine
Barbiturates -phenobarbitone
Rifampicin
St johns wart
chronic alcohol intake
griseofulvin
Smoking

360
Q

what are 11 p450 inhibitors?

A

Antibiotics - ciprofloxacin, erythromycin
Isoniazid
Omeprazole
Amiodarone
Allopurinol
Imidazoles - ketoconazole, fluconazole
SSRIs
Ritonavir
sodium valporate
quinupristine

361
Q

what is the MOA of LMWH and heparin?

A

Activates antithrombin III - forms complex that inhibits factor Xa

362
Q

what are 3 adverse effects of LMWH and heparin?

A

Thrombocytopenia
osteoporosis
hyperkalaemia

363
Q

what is the antidose for LMWH and heparin?

A

Protamine sulphate

364
Q

how is anticoagulation with heparin monitored?

A

activated partial thromboplastin time - APTT

365
Q

what is the reversal agent for dabigatran?

A

Idarucizumab

366
Q

what is the reversal agent for DOACs?

A

AdeXanet alfa

Xa - what DOACs inhibits

367
Q

what is the MOA of fondaparinux?

A

Activates antithrombin III which inhibits coagulation factor Xa

368
Q

what is the MOA of DOACs?

A

Direct factor Xa inhibitors

369
Q

What is the MOA of Dabigatran?

A

direct thrombin inhibitor

370
Q

what increases the efficacy of riveroxaban?

A

taking it with food

371
Q

what are 3 scenarios where dose reduction may be required in DOAC prescribing?

A

Elderly - >75-80 years
Reduced body weight
Severe renal impairment - 15-50 ml/min creatinine clearance

372
Q

what is pancytopenia?

A

Reduction in all 3 major cellular components of blood
RBCs <120 (F) OR <130 M
WBCs <4
Platelets <150

373
Q

what are 6 production causes of pancytopenia?

A

Vitamin deficiency - severe B12, folate or iron deficiency
Aplastic anaemia
Myelodysplastic syndromes
Bone marrow infiltration - cancers
Viral infections - HIV, parvovirus B19, CMV, EBV

374
Q

what are 2 causes of pancytopenia due to increased destruction?

A

Splenic sequestration - sickle cell, cirrhosis, malaria, TB
Autoimmune conditions - SLE, RhA

375
Q

what are 8 risk factors for pancytopenia?

A

Age - children and older adults
Male
Autoimmune disease
Recent viral infection
Alabsorption syndromes
FHx of aplastic anaemia
Hx of cancer
drugs/toxins

376
Q

what are 5 presentations of pancytopenia?

A

Fatigue
B symptoms
recurrent infections
epistaxis
Pallor
petechia
splenomegaly

377
Q

what is aplastic anaemia?

A

Bone marrow hypocellularity secondary to primary haematopoietic failure

Causes pancytopenia -
Normochromic normocytic anaemia
Thrombocytopenia
leukopenia

378
Q

what is the most common hereditary cause of aplastic anaemia?

A

Fanconi anaemia

379
Q

what is the inheritance of fanconi anaemia?

A

autosomal recessive

380
Q

what is the most common cause of aplastic anaemia?

A

idiopathic

381
Q

what are 6 drugs that can cause aplastic anaemia?

A

Carbimazole
Carbamezapine and phenytoin
Chloramphenicol
Chemo
Benze chemicals
Radiation

382
Q

what is seen on bone marrow biopsy in aplastic anaemia?

A

Hypocellular one marrow with fat cells and fibrosis replacing normal bone marrow

383
Q

what is the management of aplastic anaemia?

A

blood products and prevention and treament of infections

Anti-thymocyte globulin and anti-lymphocyte globulin
Stem cell transplant

384
Q

what is the difference between petechiae, purpura and ecchymosis?

A

petechiae = <3mm - burst capillaries

Purpura = 3-10mm

Ecchymosis = >1cm

385
Q

what 4 electrolyte disturbances are seen in tumour lysis sydrome?

A

High uric acid
Hyperkalaemia
Hyperphosphatemia
Hypocalcaemia

386
Q

Treatment of what cancers can cause tumour lysis syndrome?

A

Leukaemia
Lymphomas

387
Q

what can be used as prevention for tumour lysis syndrome?

A

IV fluids
Allopurinol or rasburicase

388
Q

what is clinical tumour lysis syndrome?

A

Lab finding of tumour lysis syndrome PLUS increased serum calcium, cardiac arrythmia/sudden death or seizure

389
Q

what 4 things are present in cryopricipitate?

A

Factor VIII
Factor XIII
Fibrinogen
vWF

390
Q

what is a normal neutrophil count?

A

2-7.5 x10^9

391
Q

what is classes as neutropaenia?

A

<1.5

392
Q

what is classed as mild neutropenia?

A

1-1.5

393
Q

what is classed as moderate neutropenia?

A

0.5-1

394
Q

what is classed as severe neutropenia?

A

<0.5

395
Q

what are 8 causes of neutropenia?

A

Viral - HIV, EBV, hepatitis
Drugs
Benign ethnic neutropenia
Haematological malignancy - myelodysplastic, aplastic anaemia
SLE
RhA - hypersplenism
Severe sepsis
Haemodialysis

396
Q

what is the most common cause of neutropenic sepsis?

A

Coagulase negative, gram positive bacteria - staphylococcus epidermis most common

397
Q

what is neutropenic sepsis?

A

neutrophils <0.5 usually in a patient 7-14 days post chemo with a temp >38 and other presentations of sepsis

398
Q

what antibiotics are used as neutropenic sepsis prophylaxis?

A

fluoroquinolones - ciprofloxacin

399
Q

what is the management of neutropenic sepsis?

A

start Abx immediately

IV - piperacillin with tazobactam

Alternative abx if still pyrexial at 48 hours - meropenem, vancomycin

If still unresponsive, investigate fungal infection

400
Q

what is the most common cause of thrombophilia?

A

factor V leiden

401
Q

what are 7 examples of thrombophilias?

A

Factor V leiden - most common
Prothrombin gene mutation - second most common
Protein C deficiency
Protein S deficiency
Antithrombin III deficiency

Antiphospholipid syndrome - acquired

402
Q

what is factor V leiden also known as?

A

activated protein c resitance

403
Q

what is the pathophysiology of factor V leiden?

A

Mis-sense mutation in activated factor V leading to inactivation to be 10x slower than normal by activated protein C

404
Q

what are the 2 different types of factor V leiden?

A

heterozygous - more common, 4-5x more likely VTE

homozygous - less common - 10x more likely VTE

405
Q

What additional treatment needs to be given in p.ovalae and p. Vivax malaria?

A

Primaquine - to prevent liver hypnozoites and therefore prevent relapse

406
Q

What additional treatment needs to be given in p.ovalae and p. Vivax malaria?

A

Primaquine - to prevent liver hypnozoites and therefore prevent relapse

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