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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?

A

X-linked condition causing haemolytic anaemia due to susceptibility to free radicals
common in Kurdish jews and sub-saharan Africa

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70
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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71
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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72
Q

what are 4 risk factors for haemolytic anaemia?

A

autoimmune disorders
FHx
drugs
prosthetic heart valves

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73
Q

what are 4 manifestations of haemolytic anaemia?

A

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

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74
Q

what is the gold standard investigation for autoimmune haemolytic anaemia?

A

Coombs test (direct antiglobulin test)

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75
Q

what is seen on blood film in haemolytic anaemia?

A

Schistocytes - fragments of RBCs

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76
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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77
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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78
Q

what is the treatment for autoimmune haemolytic anaemia?

A

Rituximab
Prednisolone
Blood transfusion
splenectomy

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79
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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80
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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81
Q

what is the management of paroxysmal nocturnal haemoglobinuria?

A

Eculizumab - MAB targeting complement component 5
Bone marrow transplant

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82
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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83
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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84
Q

what are 4 risk factors for Hodgkin’s lymphoma?

A

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

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85
Q

what is the age distribution for Hodgkin’s lymphoma?

A

Bimodal -
20-25 years
>80 years

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86
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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87
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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88
Q

what is the diagnostic investigation for Hodgkin’s lymphoma?

A

exicisional lymph node biopsy

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89
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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90
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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91
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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92
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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93
Q

what staging is used in Hodgkin’s lymphoma?

A

Lugano classification

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94
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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95
Q

what is the most common type of anaemia?

A

iron deficiency

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96
Q

what percentage of menstruating women have iron deficiency anaemia?

A

14%

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97
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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98
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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99
Q

where is iron absorbed?

A

duodenum and jejunum

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100
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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101
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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102
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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103
Q

what are 5 side effects of iron supplementation?

A

Nausea
Abdo pain
constipation
diarrhoea
black stools

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104
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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105
Q

what is the top cause of iron deficiency worldwide?

A

hook worms

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106
Q

what is malaria?

A

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

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107
Q

what plasmid of malaria causes the most deaths?

A

Plasmodium falciparum

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108
Q

what are 6 manifestations of malaria infection?

A

fever and headache
myalgia/weakness
seizures
jaundice/pallor/anaemia
hepatosplenomegaly
influenza like resp symptoms

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109
Q

what are 4 investigations for malaria?

A

Giemsa stain blood film - gold
FBC, U+E, LFTs
rapid diagnostic test
thin and thick blood smear

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110
Q

what are 3 differentials for malaria?

A

Dengue fever (headache, retrobulbar pain worsening with eye movement)
Zika virus
Yellow fever

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111
Q

what is the management of uncomplicated malaria?

A

arthemether + lumefantrine
malarone
quinine sulphate
doxycycline

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112
Q

what are 3 complications of malaria?

A

AKI
cerebral malaria
severe haemolytic anaemia

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113
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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114
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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115
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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116
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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117
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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118
Q

what is the most common Ig produced in multiple myeloma?

A

IgG - 55%

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119
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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120
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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121
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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122
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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123
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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124
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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125
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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126
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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127
Q

what are 3 differentials for multiple myeloma?

A

MGUS
smouldering myeloma
amyloidosis

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128
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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129
Q

Is multiple myeloma curable?

A

NO - has chronic relapsing remitting course

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130
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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131
Q

what is the management of bone disease in multiple myeloma?

A

Bisphosphonates - Zoledronic acid 1st line

radiotherapy
ortho surgery

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132
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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133
Q

what are 6 complications of multiple myeloma?

A

recurrent infections
Fatigue
pathological fractures
renal failure
anaemia
hypercalcaemia

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134
Q

what is non-Hodgkin’s lymphoma?

A

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

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135
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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136
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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137
Q

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

A

B cells - 80-90%

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138
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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139
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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140
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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141
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

142
Q

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

A

Hodgkin’s lymphoma
ALL
infectious mononucleosis

143
Q

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

A

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

radiotherapy
Rituximab
stem cell transplant

144
Q

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

A

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

145
Q

what are 2 causes of relative polycythaemia?

A

Dehydration
Stress - Gaisbock syndrome

146
Q

what are 4 secondary causes of polycythaemia?

A

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

147
Q

what is Polycythaemia vera?

A

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

148
Q

what genetic mutation if associated with Polycythaemia vera?

A

JAK2 mutation - 95% of patients

149
Q

what are 7 presentations of Polycythaemia vera?

A

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

150
Q

what are 4 first line investigations of Polycythaemia vera?

A

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

151
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

152
Q

what are 5 complications of Polycythaemia vera?

A

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

153
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

154
Q

what is seen on FBC in myelofibrosis?

A

Anaemia
high WCC
High platelets

155
Q

what can be seen on blood film in myelofibrosis?

A

Tear drop poikilocytes

156
Q

what are 6 presentations of myelofibrosis?

A

Severe fatigue
Hepatosplenomegaly
B symptoms
Thromboembolic events
Unexplained bleeding

157
Q

what may be seen on bone marrow biopsy in myelofibrosis?

A

‘dry tap’ without aspirate

158
Q

what is the management of myelofibrosis?

A

Ruxolitinib - JAK 2 inhibitor

Hydroxyurea

159
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

160
Q

what is essential thrombocytosis?

A

myeloproliferative disorder of excessive mature platelet production

161
Q

what is the presentation of essential thrombocytosis?

A

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

162
Q

what is seen on peripheral blood smear in essential thrombocytosis?

A

platelet anisocytosis (different sizes)

163
Q

what is the management of essential thrombocytosis?

A

Aspirin - thrombosis prophylaxis

Hydroxycarbamide

Interferon alpha - in younger patients and women of childbearing age

164
Q

what is the inheritance of sickle cell anaemia?

A

Autosomal recessive

point mutation substituting hydrophilic glutamic acid for hydrophobic valine

165
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

166
Q

what chromosome if affected in sickle cell anaemia?

A

chromosome 11

167
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

168
Q

what triggers sickling in sickle cell disease?

A

hypoxia
acidosis
dehydration
Cold temperatures
extreme exercise
stress
Infections

169
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

170
Q

what haemoglobin chain is affected in sickle cell anaemia?

A

Beta haemoglobin

171
Q

what is foetal haemoglobin made up of?

A

2 alpha chains and 2 gamma chains

172
Q

what is adult haemoglobin made up of?

A

2 alpha chains
2 beta chains

173
Q

what is the gold standard investigation for sickle cell anaemia?

A

haemoglobin electrophoresis

174
Q

what are 3 investigations for sickle cell?

A

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

175
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

176
Q

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

A

Hydroxycarbamide (usually 15mg/kg)

177
Q

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

A

pneumococcal

178
Q

what is the management for sickle cell crisis?

A

Supportive

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

179
Q

what are 4 sickle cell crises?

A

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

180
Q

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

A

blood transfusion
IV fluids

Splenectomy if recurrent

181
Q

what can be seen on FBC in splenic sequestration crisis?

A

increased reticulocyte count

182
Q

what virus can trigger aplastic crisis in sickle cell anaemia?

A

Parvovirus B19

183
Q

what is the presentation of acute chest syndrome?

A

Hx of sickle cell

Chest pain
cough
SOB
fever
hypoxia
Pulmonary infiltrates on CXR

184
Q

what is the management of Acute chest syndrome?

A

Analgesia
Resp support
Abx
Transfusion

185
Q

what is the presentation of an aplastic crisis?

A

Sudden fall in Hb

Bone marrow suppression causes reduced reticulocyte count

186
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

187
Q

what are the vitamin K dependant clotting factors?

A

X, IX, VII, II - 1972

188
Q

can warfarin be used in pregnancy?

A

NO it’s teratogenic

189
Q

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

A

Rouleaux formation

190
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

191
Q

what are 5 generic signs of anaemia?

A

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

192
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

193
Q

what is a sign of haemolytic anaemias?

A

jaundice

194
Q

what is a sign of thalassaemia?

A

bone deformities

195
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

196
Q

what are the 4 iron studies?

A

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

197
Q

what is total iron binding capacity?

A

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

198
Q

what is the formula for transferrin saturation?

A

serum iron / total iron binding capacity

199
Q

what is normal transferrin saturation?

A

30%

200
Q

what affects ferritin levels?

A

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

201
Q

what is the inheritance pattern of hereditary spherocytosis?

A

majority autosomal dominant

202
Q

what is the management of hereditary spherocytosis?

A

phototherapy or exchange transfusion - neonates with jaundice

folic acid supplementation

Splenectomy

phenoxymethylpenicillin

203
Q

what are 3 complications of hereditary spherocytosis?

A

gallstones
aplastic crisis
bone marrow expansion

204
Q

what are 4 precipitators of G6PD deficiency?

A

Fava beans
soy
red wine
infections

205
Q

what does the urine look like in G6PD deficiency?

A

tea coloured

206
Q

what is aplastic anaemia?

A

stem cell disorder characterised by pancytopenia

207
Q

what are 4 causes of aplastic anaemia?

A

radiation and toxins
drugs
infections
fanconi’s anaemia

208
Q

what condition are Howell-jolly bodies seen in?

A

Sickle cell

209
Q

what is a histological sign go sickle cell disease?

A

Howell-jolly bodies

210
Q

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

A

salmonella

211
Q

what do Reed-Sternberg cells look like?

A

owl eye nuclei

212
Q

what is found in the urine in multiple myeloma?

A

bench-jones proteins - Ig light chains

213
Q

what is found on the serum electrophoresis in multiple myeloma?

A

monoclonal paraprotein band

214
Q

what is the treatment of severe malaria?

A

Artemisinin combination therapy (ACT)

quinine

215
Q

what is the inheritance pattern for haemophilia?

A

X-linked recessive

216
Q

which factor is there a deficiency in in haemophilia A?

A

VIII - 8

217
Q

which factor is there a deficiency in in haemophilia B?

A

IX - 9

218
Q

what are 7 clinical manifestations of haemophilia?

A

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

219
Q

what is needed for diagnosis of haemophilia?

A

Bleeding scores
coagulation factor assays
genetic testing

220
Q

what clotting time will be prolonged in haemophilia?

A

activated partial thromboplastic time - aPTT

Prothrombin time (PT) will be normal

221
Q

what are 3 differentials for haemophilia?

A

Von Willebrand disease
Platelet dysfunction disorders
Scurvy

222
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

223
Q

what medications should be avoided in haemophilia?

A

Aspirin and NSAIDs - increase bleeding risk

224
Q

what are 3 complications of haemophilia?

A

Intracranial haemorrhage
Compartment syndrome
arthropathy from haemarthrosis

225
Q

what chromosome codes for von willebrand factor?

A

chromosome 12

226
Q

what are the 3 different types of von willebrand disease?

A

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

227
Q

what does von willebrand factor do?

A

Mediated platelet adhesion

Stabilises factor VIII

228
Q

what is the presentation of von Willebrand disease?

A

Bleeding gums
epistaxis
easy bruising
menorrhoagia
heavy bleeding

FAMILY HISTORY

229
Q

what is the most common bleeding disorder?

A

Von Willebrand disease

230
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

231
Q

what is thalassaemia

A

autosomal recessive haemoglobinopathy which causes microcytic anaemia

232
Q

what demographic is alpha thalassaemia most common in?

A

asian and African descent

233
Q

what demographic is beta thalassaemia most common in?

A

SE asian, Mediterranean and Middle Eastern descent

234
Q

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

A

deferoxamine

235
Q

what can reverse heparin overdose?

A

protamine sulphate

236
Q

what is the antidote to warfarin?

A

vitamin K1

237
Q

What is the Philadelphia chromosome?

A

Translocation of a part of chromosome 9 to chromosome 22

238
Q

is primaquine safe in pregnancy?

A

NO

239
Q

what is given as tumour lysis prophylaxis in AML?

A

allopurinol

240
Q

what are the histological signs of G6PD?

A

bite cells
Heinz bodies
reticulocytes

241
Q

what antibiotic is contraindicated in G6PD?

A

trimethoprim

242
Q

What leukaemia is tumour lysis syndrome most common in?

A

AML

243
Q

what cells are seen in CLL?

A

smudge cells

244
Q

what cells are seen in ALL?

A

blast cells

245
Q

what is antiphospholipid syndrome?

A

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

246
Q

what antibodies cause antiphospholipid syndrome?

A

antiphospholipid antibodies

247
Q

what is the inheritance pattern of haemochromatosis?

A

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

248
Q

what is haemochromatosis?

A

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

249
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

250
Q

what are 6 causes of raised serum ferritin?

A

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

251
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

252
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

253
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

254
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

255
Q

what stem cell do all blood cells differentiate from?

A

Multipotential haematopoietic stell cell - hemocytoblast

256
Q

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

A

Common myeloid progenitor

Common lymphoid progenitor

257
Q

what 2 cells does the common lymphoid progenitor differentiate into?

A

Natural killer cell - large granular lymphocyte

small lymphocyte

258
Q

what 2 cells does the small lymphocyte cell differentiate into?

A

T lymphocyte

b lymphocyte

259
Q

what do B lymphocytes differentiate into?

A

Plasma cells

Memory b lymphocytes

260
Q

what is the role of plasma cells?

A

Produce antibodies against specific antigens

261
Q

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

A

Megakaryocyte
Erythrocyte
Mast cell
Myeloblast

262
Q

what do megakaryocytes differentiate into?

A

Platelets

263
Q

what 4 cells do myeloblasts differentiate into?

A

Basophils
Neutrophils
Eosinophils
Monocytes

264
Q

what do monocytes differentiate into?

A

Macrophages

265
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)

266
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

267
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

268
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

269
Q

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

A

Temporarily stop transfusion
Antihistamines
monitor

270
Q

what causes anaphylaxis in blood transfusion?

A

patient with IgA deficiency who have anti-IgA antibodies

271
Q

what is transfusion associated circulatory overload (TACO)?

A

Due to fluid overloading - causes pulmonary oedema

may also be hypertensive

272
Q

What is the management of transfusion associated circulatory overload?

A

Slow/stop transfusion
Consider IV loop diuretics - furosemide
O2

273
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

274
Q

what is the management of transfusion related acute lung injury?

A

Stop transfusion
Oxygen and supportive care

275
Q

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

A

Platelets as stored at room temp

276
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

277
Q

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

A

intrinsic pathway

aPTT = Play Table Tennis inside = intrinsic

278
Q

what factors are involved in the intrinsic coagulation pathway?

A

XII
XI
IX
VIII

12, 11, 9, 8

279
Q

what factors are involved in the extrinsic coagulation pathway?

A

III
VII

5 + 7

280
Q

what factors are involved in the common coagulation pathway?

A

X
V
II
I
XIII

10, 5, 2, 1, 13

281
Q

what triggers the intrinsic coagulation pathway?

A

Contact with damaged endothelial surfaces

282
Q

what triggers the extrinsic coagulation pathway?

A

Tissue factor III activation via endothelial tissue damage to IIIa

283
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

284
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

285
Q

what is seen on bloods in DIC?

A

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

286
Q

what is the management of DIC?

A

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

Blood product transfusion

287
Q

what are 4 complications of DIC?

A

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

288
Q

what is the most common cause of thrombocytopenia?

A

Immune thrombocytopenia

289
Q

what is thrombocytopenia?

A

Platelets <150 x10^9/L

290
Q

what is the normal range for platelets?

A

150-450 x10^9 /L

291
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

292
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

293
Q

What low platelet count is usually symptomatic?

A

<50 x10^9/L

294
Q

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

A

<10 x10^9/L

295
Q

what are the top 4 differentials for abnormal bleeding?

A

thrombocytopenia
von willebran disease
Haemophillia
Disseminated intravascular coagulation

296
Q

what is wiskott-aldrich syndrome?

A

X-linked condition - eczema, thrombocytopenia, immunodeficiency

297
Q

what is faconi aanemia?

A

inherited bone marrow failure syndrome, characterised by pancytopenia

298
Q

what are 4 drugs that can induce thrombocytopenia?

A

Heparin
Quinine
Sulfonamides
Naproxen

299
Q

what is immune thrombocytopenia purpura?

A

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

300
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

301
Q

what is primary versus secondary ITP?

A

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

302
Q

How is immune thrombocytopenia purpura diagnosed?

A

Diagnosis of exclusion

1 - FBC, peripheral blood smear

Bone marrow aspiration, blood borne virus serology

303
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

304
Q

what is thrombotic thrombocytopenic purpura?

A

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

305
Q

deficiency of what protein causes thrombotic thrombocytopenic purpura?

A

ADAMTS13

306
Q

what is the role of ADAMTS13?

A

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

307
Q

what can cause ADAMTS13 deficiency?

A

Inherited genetic mutation
Autoimmune disease against protein

308
Q

what is the classic pentad of thrombotic thrombocytopenic purpura ?

A

Thrombocytopaenia purpura
Microangiopathic haemolytic anaemia
Neurological dysfunction
Renal dysfunction
Fever

309
Q

what is the management of thrombotic thrombocytopenic purpura ?

A

1 - Plasma exchange
1 - orticosteroids
1 - Caplacizumab

2 - Rituximab

310
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

311
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

312
Q

How is heparin induced thrombocytopenia diagnosed?

A

Testing serum heparin induced thrombocytopenia antibodies

313
Q

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

A

Fondaparinux or argatroban

314
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

315
Q

what is the secondary prevention dose of aspirin?

A

75mg OD

316
Q

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

A

300mg

317
Q

what are 4 examples of P2Y12 inhibitors?

A

Clopidogrel
Parasugrel
Ticagrelor
Ticlopidine

318
Q

what medications can reduce the efficacy of clopidogrel?

A

PPIs - especially omeprazole and esomeprazole

319
Q

what is the reversal agent for ticagrelor?

A

Bentracimab

320
Q

what is the MOA of warfarin?

A

Vitamin K antagonist

321
Q

what is the normal warfarin target?

A

Between 2-3

Aim 2.5

322
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

323
Q

what are 7 cytochrome P450 inducers?

A

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

324
Q

what are 11 p450 inhibitors?

A

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

325
Q

what is the MOA of LMWH and heparin?

A

Activates antithrombin III - forms complex that inhibits factor Xa

326
Q

what are 3 adverse effects of LMWH and heparin?

A

Thrombocytopenia
osteoporosis
hyperkalaemia

327
Q

what is the antidose for LMWH and heparin?

A

Protamine sulphate

328
Q

how is anticoagulation with heparin monitored?

A

activated partial thromboplastin time - APTT

329
Q

what is the reversal agent for dabigatran?

A

Idarucizumab

330
Q

what is the reversal agent for DOACs?

A

AdeXanet alfa

Xa - what DOACs inhibits

331
Q

what is the MOA of fondaparinux?

A

Activates antithrombin III which inhibits coagulation factor Xa

332
Q

what is the MOA of DOACs?

A

Direct factor Xa inhibitors

333
Q

What is the MOA of Dabigatran?

A

direct thrombin inhibitor

334
Q

what increases the efficacy of riveroxaban?

A

taking it with food

335
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

336
Q

what is pancytopenia?

A

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

337
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

338
Q

what are 2 causes of pancytopenia due to increased destruction?

A

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

339
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

340
Q

what are 5 presentations of pancytopenia?

A

Fatigue
B symptoms
recurrent infections
epistaxis
Pallor
petechia
splenomegaly

341
Q

what is aplastic anaemia?

A

Bone marrow hypocellularity secondary to primary haematopoietic failure

Causes pancytopenia -
Normochromic normocytic anaemia
Thrombocytopenia
leukopenia

342
Q

what is the most common hereditary cause of aplastic anaemia?

A

Fanconi anaemia

343
Q

what is the inheritance of fanconi anaemia?

A

autosomal recessive

344
Q

what is the most common cause of aplastic anaemia?

A

idiopathic

345
Q

what are 6 drugs that can cause aplastic anaemia?

A

Carbimazole
Carbamezapine and phenytoin
Chloramphenicol
Chemo
Benze chemicals
Radiation

346
Q

what is seen on bone marrow biopsy in aplastic anaemia?

A

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

347
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

348
Q

what is the difference between petechiae, purpura and ecchymosis?

A

petechiae = <3mm - burst capillaries

Purpura = 3-10mm

Ecchymosis = >1cm

349
Q

what 4 electrolyte disturbances are seen in tumour lysis sydrome?

A

High uric acid
Hyperkalaemia
Hyperphosphatemia
Hypocalcaemia

350
Q

Treatment of what cancers can cause tumour lysis syndrome?

A

Leukaemia
Lymphomas

351
Q

what can be used as prevention for tumour lysis syndrome?

A

IV fluids
Allopurinol or rasburicase

352
Q

what is clinical tumour lysis syndrome?

A

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

Phase 4 (22 decks)

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