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semester 2 (3rd year) > Case 20: fatigue and neck swelling > Flashcards

Case 20: fatigue and neck swelling Flashcards

1
Q

what is haematopoiesis

A

formation of all mature blood cells from haematopoietic stem cells

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2
Q

where does haematopoiesis occur

A

bone marrow

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3
Q

what are the two lineages of haematopoiesis

A

myeloid and lymphoid

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4
Q

examples of myeloid cells

A

thrombocytes (platelet)
basophils
neutrophils
eosinophils
macrophages (monocytes)
erythrocytes

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5
Q

examples of lymphoid cells

A

natural killer cells
T lymphocytes
B lymphocytes
plasma cells

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6
Q

process of haematopoiesis

A

a multipotential hematopoietic stem cell (hemocytoblast) either differentiates into a common myeloid progenitor or common lymphoid progenitor

the common myeloid progenitor can either differentiate into a megakaryocyte (which can then differentiate into thrombocytes), erythrocyte, mast cell or myeloblast (which can then differentiate into a basophil, neutrophil, eosinophil or monocyte)- the monocyte can then further differentiate into a macrophage

the common lymphoid progenitor can differentiate into a natural killer cell or small lymphocyte (this can then differentiate into a T or B lymphocyte)- the B lymphocyte can then further differentiate into a plasma cell

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7
Q

what does the term blast mean relative to cells

A

blasts refers to immature forms of cells

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8
Q

where are blasts usually seen

A

in the bone marrow

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9
Q

what does it mean when blasts are seen in the peripheral blood

A

suggest haematological malignancy

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10
Q

what us the lymphatic system comprised of

A

lymphatic vessels

bone marrow and thymus

lymph nodes, spleen and mucosa-associated lymphoid tissue (MALT)

organs and tissue where the immune cells collect and are stored

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11
Q

what happens in bone marrow and thymus

A

these are the organs in which the immune cells develop

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12
Q

what are the different areas of a lymph node

A

the cortex (surrounds)

medulla (middle)

mantle zone (areas in the medulla)

follicle (found in mantle zone)

germinal centre (the centre of the follicle)

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13
Q

other names for infectious mononucleosis

A

glandular fever
mono

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14
Q

causes of infectious mononucleosis

A

EBV aka HHV-4

also CMV, syphilis, HIV seroconversion, toxoplasma, brucella

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15
Q

signs and symptoms of infectious mononucleosis

A

generalised/cervical lymphadenopathy

systemic- low grade fever, fatigue/malaise, anorexia

pharyngitis

splenomegaly in 50%, hepatomegaly and jaundice suggests EBV in 10%

bilateral upper eyelid oedema

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16
Q

diagnosis of infectious mononucleosis

A

heterophil antibody tests (monospot/Paul-Bunnell) which looks for non-specific heterophilic IgM released by EBV-stimulated B cells

do EBV antibodies if the above is -ve

can also do EBV PCR

others- blood film shows atypical lymphocytes, throat swab should be -ve for strep A, US for splenomegaly, lumbar puncture if there is meningism

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17
Q

what should you do to avoid splenic rupture with infectious mononucleosis

A

avoid contact sports and alcohol

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17
Q

management of infectious mononucleosis

A

usually self resolving

paracetamol for fever and pain

prednisolone if airway obstruction or haemolytic anaemia

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18
Q

how is EBV spread

A

saliva/droplets (kissing is common)

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19
Q

what % of people have been exposed to EBV

A

90%

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20
Q

long term complications of EBV

A

cancer- Burkitts lymphoma, Hodgkins lymphoma, nasopharyngeal carcinoma

hairy leukoplakia (non-malignant warty lesion on lateral tongue in the immunosuppressed, can be scraped off)

MS- 100% of MS patients have been exposed to virus

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21
Q

what are Bs symptoms

A

fever
weight loss
might sweats
neck lump

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22
Q

what would you do if B symptoms were present

A

urgent 2 week wait referral to haematologist

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23
Q

does leukaemia usually present with lymphadenopathy

A

no not usually

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24
Q

does lymphoma usually present with lymphadenopathy

A

yes
might see bilateral hilar lymphadenopathy on CXR

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25
Q

if you suggest haematological malignancy which type of biopsy is suggested

A

complete lymph node excision biopsy (leaves scar on neck)- better as the cells are far clearer when the entire node is biopsied

can do a needle biopsy which doesn’t leave scar however this is limited as is can give a false negative

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26
Q

lymphoma vs leukaemia

A

lymphoma= malignant proliferation of mature lymphocytes that accumulate in lymph nodes (and possibly other tissues), often as a solid tumour

leukaemia= arises in the bone marrow and is present in the blood

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27
Q

Hodgkins lymphoma is what % of all lymphomas

A

20%

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28
Q

what is seen on light microscopy with Hodgkins lymphoma

A

mirror-image binucleated Reed-Sternberg cells

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29
Q

which type of cancer cell is Hodgkins lymphoma

A

B cell cancer

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30
Q

subtypes of Hodgkins lymphom

A

common= nodular necrosis (70%) usually seen in young, and mixed cellularity (25%) usually seen in old- both have good prognosis

rare (5%)= lymphocyte-rich which has an excellent prognosis or lymphocyte-depleted which has a bag prognosis

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31
Q

ages most commonly affected by Hodgkins lymphoma

A

15-30 and 75-80

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32
Q

is Hodgkins lymphoma more common in men or women

A

men

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33
Q

lymphadenopathy symptoms of Hodgkins lymphoma

A

painless rubbery nodes which are usually cervical but can be inguinal/axillary

may be adherent to each other and move together (matted)

increase and decrease spontaneously

painful on alcohol consumption

mediastinal lymph nodes can cause SOB, dry cough, SVC obstruction

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34
Q

B symptoms of Hodgkins lymphoma

A

weight loss
fever (Pel-Ebstein fever every 2-4 weeks= rare)
night sweats
lethargy
pruritus

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35
Q

other features of Hodgkins lymphoma

A

anaemia of chronic disease
hepatosplenomegaly in advanced disease

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36
Q

risk factors for Hodgkins lymphoma

A

family history
EBV
increases socio-economic status

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37
Q

what staging is used for both Hodgkins lymphoma and non-Hodgkins lymphoma

A

ann arbor staging system

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38
Q

what does the ann arbor staging system take into account

A

location (lymph nodes and beyond)

presence/ absence of systemic symptoms

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39
Q

management of Hodgkins lymphoma

A

chemotherapy + radiotherapy

for relapsed/resistant disease can do autologous stem cell transplantation (bone marrow removed, chemo given and then marrow returned)

pneumococcal and flu vaccine

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40
Q

what % of all lymphomas is non-hodgkins lymphoma

A

80%

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41
Q

what is non-hodgkins lymphoma

A

it is a diverse group of conditions with proliferating cells potentially accumulating in lymph nodes, MALT, CNS and skin

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42
Q

what type of cell cancer is non-hodgkins lymphoma

A

90% is B cell proliferation
10% is T cell proliferation

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43
Q

2 sub-types of non-hodgkins lymphoma

A

low grade lymphoma
high grade lymphoma

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44
Q

is high or low grade non-hodgkins lymphoma easier to cure

A

low-grade= slow growing, good prognosis, but hard to cure

high-grade= more acute but easier to cure

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45
Q

examples of low grade non-hodgkins lymphoma

A

follicular lymphoma (CD20 +ve)
marginal zone lymphoma
lymphocytic lymphoma
waldenstroms macroglobulinaemia aka lymphoplasmacytoid (increased IgM)

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46
Q

examples of high grade non-hodgkins lymphoma

A

diffuse large B-cell lymphoma (CD20 +ve)
mantle cell lymphoma (usually incurable)
peripheral T cell lymphoma
burkitts lymphoma (commoner in children and characteristic jaw lymphadenopathy)
lymphoblastic lymphoma

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47
Q

lymphadenopathy signs of symptoms of non-hodgkins lymphoma

A

lymphadenopathy is presenting complaint in 2/3s

in high grade there is a short history of rapid growth

splenomegaly often comes with lymphadenopathy (especially common in marginal zone lymphoma)

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48
Q

extra nodal manifestations of non-hodgkins lymphoma

A

can be anywhere meaning almost any symptom

gut- abdo pain, weight loss, dyspepsia

skin- T cell lymphoma, rash, discolouration

oropharynx- sore throat, obstructed breathing

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49
Q

are B symptoms present in non-hodgkins lymphoma

A

they are much more common in Hodgkins lymphoma rather than non-hodgkins lymphoma

can be quite frequent in high grade non-hodgkins lymphoma however

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50
Q

risk factors for non-hodgkins lymphoma

A

immunodeficiency:
congenital like Wiskott-Aldrich
drugs
HIV (usually high grade lymphomas)

infection:
HTLV1
EBV
Hep C
H.pylori

autoimmune:
hashimotos (thyroid MALT lymphoma)
Sjogrens (salivary MALT lymphoma)

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51
Q

management of low grade non-hodgkins lymphoma

A

treatment may not be needed if asymptomatic (in follicular lymphoma)

chemo + radiotherapy

maintain remission with alpha-interferon or rituximab (especially if CD20 +ve)

H.pylori eradication can sometimes cure gastric MALT

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52
Q

management of high grade non-hodgkins lymphoma

A

chemotherapy

autologous or allogenic stem cell transplant in refractory cases

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53
Q

what size of lymphadenopathy is concerning in adults

A

more than 1cm (if not related to infection)
any size if have persistent head and neck symptoms)

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54
Q

what size of lymphadenopathy is concerning in children

A

over 2cm

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55
Q

inflammatory causes of lymphadenopathy

A

secondary to infection- bacterial/viral (tonsils, teeth, ear, scalp)

TB (cervical)

HIV

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56
Q

how do the lumps feel in lymphoma

A

multiple often smooth and firm rather than hard
rubbery

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57
Q

how do the lumps feel when the lymph nodes are metastatic

A

fixed and irregular
vast majority come from head and neck cancer

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58
Q

most common primary cancers causing metastatic nodes

A

mucosal squamous carcinoma (oral, pharynx, larynx)
thyroid cancer
salivary gland cancer
skin cancer (squamous, melanoma- ear/scalp)

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59
Q

what does a fibreoptic endoscopy look at

A

mouth
nasal cavity
larynx
pharynx

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60
Q

risk factors for head and neck cancer

A

smoking
alcohol
HPV (for tonsilar cancer)
cannabis

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61
Q

how does a PETCT scan help diagnose in malignancies

A

it helps show the difference between a reactive node and a malignant node (uptake in malignant is higher)

the radioactive sugar is taken up by metabolically active cells and so shows how widespread a cancer or lymphoma is

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62
Q

stage I-IV lymphoma

A

I- one group of nodes affected
II= two or more groups nodes affected on same side of the diaphragm
III= both sides of diaphragm affected (north and south)
IV= involvement of extra-nodal tissue (bone marrow, liver, lung, spleen)

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63
Q

stage A and B lymphoma

A

A= no systemic symptoms
B= systemic symptoms present (weight loss, fever, night sweats)

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64
Q

chemotherapy for lymphoma increases your risk of what

A

infection due to reduced white cells (neutropenia)

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65
Q

cure rate for Hodgkins lymphoma

A

over 80% are cured
relapse after 5 years is rare

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66
Q

what blood is a key prognostic factor in diffuse large B cell non-hodgkins lymphoma

A

lactate dehydrogenase

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67
Q

how to treat neutropenia + pyrexia following chemotherapy for lymphoma

A

need immediate IV antibiotics (can be deadly due to neutropenic sepsis)

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68
Q

what is R-CHOP treatment

A

used to treat NHL
chemoimmmuntherapy plus steroid

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69
Q

what does R-CHOP treatment target

A

CD20 antibody
rituximab

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70
Q

cure rate for diffuse large B cell non-hodgkins lymphoma

A

50% are cured
relapse after 5 years is rare

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71
Q

two most common types of B cell non-hodgkins lymphoma

A

diffuse large B cell (most common)
follicular (second most common)

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72
Q

features of follicular non-hodgkins lymphoma

A

typically no symptoms
responds well to treatment but recurrent relapses- incurable

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73
Q

median survival of follicular non-hodgkins lymphoma

A

10 years

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74
Q

treatment of follicular non-hodgkins lymphoma

A

radiotherapy (targets specific nodes affected)
often daily for a short course to manage disease progression

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75
Q

when considering lymphoma which part of the abdominal examination is important

A

palpation of liver and spleen as can see hepatic-splenomegaly

76
Q

what is seen histologically with non-hodgkins lymphoma

A

diffuse or nodular abnormal lymphocytes

77
Q

features of low grade NHL

A

tumours grow slowly and may not require treatment for long periods

when treatment needed they respond well, but are very rarely cured

example= follicular NHL

78
Q

features of high grade NHL

A

quicker growing and symptomatic

more likely to be completely cured with chemotherapy

examples= diffuse large B cell and Burkitt lymphomas

79
Q

womens fertility and Hodgkins lymphoma

A

for young women undergoing standard chemotherapy their fertility should recover

pregnancy should be prevented during chemotherapy and for at least 6 months afterwards (relapse is highest in first 2 years following)

in older women/those undergoing gonadotoxic chemotherapy can do egg retrieval/embryo storage

80
Q

long term risks following HL

A

second cancers
cardiac problems
hormonal problems

81
Q

patients with HL who have received chemotherapy and radiotherapy are at a greater risk of developing which cancer

A

lung (smoking cessation necessary)

82
Q

which age is typically affected by NHL

A

median age is 55

83
Q

is HL or NHL more common

A

NHL

84
Q

what is a non blanching rash

A

a rash which does not disappear with pressure

85
Q

what is purpura

A

the appearance of non-blanching purple red spots of the skin
it signifies bleeding vessels near the surface and can also occur in the mucous membrane

86
Q

petechiae vs ecchymosis

A

petechiae= purpura less than 1cm
ecchymosis= purpura greater than 1cm

87
Q

main pathophysiological causes of petechiae

A

thrombocytopenia
platelet dysfunction
disorders of coagulation
loss of vascular integrity

88
Q

vitamin cde causes of petechial rash

A

vascular= henoch-schonlein Purpur (HSP)

infective= viral (EBV, mononucleosis) which would come with fever sore throat and fatigue, sepsis particularly meningococcal sepsis whenever patient has fever and purpuric rash, fungi such as rickettsial

trauma= any

autoimmune= systemic lupus erythematous (SLE), thrombotic thrombocytopenia purpura (TTP) and haemolytic uraemia syndrome (HUS), for TTP and HUS will have urinary symptoms

metabolic= n/a

iatrogenic= drugs such as chemotherapy which can cause thrombocytopenia

neoplastic= leukaemia

congential= wischt-aldrich syndrome which is characterised by immune deficiency, eczema, and reduced ability to form blood clots (primary affects males

degenerative= chronic liver disease

endocrine= n/a

89
Q

causes of non-thrombocytopenic purpura

A

congenital-hereditary haemorrhagic telangiectasia, Ehlers-Danlos syndrome (connective tissue disease)

acquired- infections secondary to septicaemia, meningococcal infections or measles

vasculitis- Henoch-Schoelein purpura (HSP), SLE

drugs- steroids, sulphonamides, clopidogrel, aspirin, SSRIs, fish oils

trauma

90
Q

causes of thrombocytopenic purpura

A

impaired platelet production- bone marrow failure such as leukaemia, aplastic anaemia, myeloma, marrow infiltrations, drugs such as co-trimoxazole

excessive platelet destruction- immune thrombocytopenic purpura (ITP) and coagulation problems such as disseminated intravascular coagulation (DIC), HUS, TTP

sequestration of platelets in splenomegaly

91
Q

how does a blood clot form

A

cells release von willebrand factor

vWF sticks to fibres in the torn tissue and becomes like glue

this allows platelets to stick to the site

platelets link forming a mesh- clot

92
Q

what is ADAMTS13

A

an enzyme which breaks down vWF when it is no longer needed

93
Q

what is thrombotic thrombocytopenic purpura

A

rare serious blood disease causing many small blood clots throughout the body

severe deficiency of ADAMTS13

94
Q

clinical findings of thrombotic thrombocytopenic purpura in blood

A

low platelets

increased RBC destruction

95
Q

signs and symptoms of thrombotic thrombocytopenic purpura

A

headaches
confusion
mental changes
speech abnormalities
partial paralysis
seizures
coma
abnormal bleeding in the skin- purpura
fever
weakness
fatigue
extreme paleness
heavy bleeding
abdominal pain
nausea and vomiting

96
Q

complication of thrombotic thrombocytopenic purpura

A

AKI- occurs in less than 10% and requires dialysis

this is due to the lack of blood and urine filtration which increases water salt and protein retention

can result in SOB, pedal oedema, headaches and irregular heartbeat

97
Q

types of thrombotic thrombocytopenic purpura

A

immune mediated/ acquired thrombotic thrombocytopenic purpura

and

congential/familial thrombotic thrombocytopenic purpura

98
Q

features of immune mediated/ acquired thrombotic thrombocytopenic purpura

A

more common and autoimmune

develops late childhood/adulthood

antibodies against ADAMTS13

99
Q

features of congential/familial thrombotic thrombocytopenic purpura

A

autosomal recessive

very low levels of ADAMTS13

100
Q

diagnosis of thrombotic thrombocytopenic purpura

A

needs to be urgent

deficiency of less than 10% ADAMTS13 activity on bloods

for autoimmune need anti-ADAMTS13 antibodies in blood

101
Q

treatment of thrombotic thrombocytopenic purpura

A

plasmapheresis- blood is removed, plasma is separated from their blood and replaced with healthy plasma (removes antibodies and adds back enzyme)

steroids

rituximab- decreases antibodies and given via IV infsuion

102
Q

what is leukaemia

A

malignant neoplastic process involving one of the WBC lines (neutrophils, lymphocytes, monocytes, etc)

103
Q

main classifications of leukaemia

A

is either myeloid or lymphoid

then further classified as acute or chronic

104
Q

what is seen on blood film with acute leukaemia

A

peripheral blood film is dominated by immature (blast) cells therefore is referred to as acute myeloblastic or lymphoblastic leukaemia

105
Q

issues with blast cells crowding the bone marrow

A

means the marrow is unable to produce healthy blood cells- can result in patient being anaemic and/or thrombocytopenic

106
Q

which is the most common childhood cancer

A

acute lymphocytic leukaemia

107
Q

pathology of acute lymphocytic leukaemia

A

malignant clonal disease that develops when B/T precursor stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergo uncontrolled proliferation

108
Q

age commonly affected by acute lymphocytic leukaemia

A

under 20s
accounts for 80% of leukaemia in paediatrics and 20% in adults

109
Q

pathology of acute myeloid leukaemia

A

clinical expansion of myeloid blasts in the bone marrow, peripheral blood, or extra-medullary tissues

110
Q

ages affected by acute myeloid leukaemia

A

risk increases with age

111
Q

which sex is more commonly affected by acute leukaemia

A

male

112
Q

signs and symptoms of acute leukaemia

A

anaemia- SOB, fatigue, pallor

infection- there is increased WBCs but low neutrophils (neutropenia), low grade fever

bleeding- bruising, menorrhagia, internal

113
Q

signs and symptoms of acute leukaemia causing organ infiltration

A

hepatosplenomegaly
lymphadenopathy
CNS (especially in ALL)- CN palsy, papilloedma, meningism unilateral swelling of testes (especially in ALL)
gum hypertrophy, skin nodules
mediastinal mass (thymus)

114
Q

risk factors of acute leukaemia

A

chemotherapy/radiation exposure

previous haematological disease (for AML) such as myelodysplastic syndrome, paroxysmal nocturnal haemoglobinuria

family history

genetic- downs syndrome, fanconis anaemia, ataxia telangiectasia

115
Q

blood findings in acute leukaemia

A

pancytopenia (low red, white and platelets), but neutropenia

may have disseminated intravascular coagulation

may have raised urea and creatinine

increased LFTs

increased lactate dehydrogenase

116
Q

what is seen on blood film with acute leukemias

A

lymphoblasts seen in ALL
myeloblasts containing Auer rods in AML

117
Q

diagnosis of acute leukemia

A

bone marrow biopsy and/or aspiration, plus biopsy of infiltrated organs

blast cells above 20% confirm diagnosis

118
Q

which test identifies the subtype of acute leukaemia

A

immunophenotyping

119
Q

what test provides prognostic and therapeutic info about acute leukaemia

A

cytogenics

translocations:
philadelphia chromsome in adults with ALL
t(12;21) in kids with ALL
t(15;17) in acute promyeloid leukaemia (AML variant)

120
Q

supportive management of acute leukemias

A

for pancytopenia- RBC and platelet transfusion, antibiotics

allopurinol if increased uric acid from tumour lysis

121
Q

chemotherapy duration for AML

A

6-8 months

122
Q

chemotherapy duration for ALL

A

up to 3 years

123
Q

iatrogenic complications of acute leukaemia

A

infertility (especially in males so sperm bank)
nausea and vomiting
bone marrow failure (transplant)
tumour lysis syndrome
long term risks= cancer, hypothyroidism, pulmonary fibrosis, heart failure
kids= short stature and decreased IQ

124
Q

prognosis of ALL

A

5 year survival:
90% in children
50% in adults

125
Q

prognosis of AML

A

5 year survival:
20% overall
75% if under 60

126
Q

pathophysiology of chronic lymphocytic leukaemia

A

there is proliferation of mature B cells and accumulation in the blood and bone marrow

127
Q

which is the commonest adult leukaemia

A

CLL

128
Q

which sex is most affected by chronic lymphocytic leukaemia

A

male

129
Q

most common presentation of chronic lymphocytic leukaemia

A

90% are asymptomatic
usually incidental findings of increased WBCs

can see lymphadenopathy, splenomegaly and hepatomegaly

130
Q

symptomatic presentation of chronic lymphocytic leukaemia

A

can have cytopenia from bone marrow infiltration- anaemia (SOB, fatigue), thrombocytopenia can cause petechiae

systemic- weight loss, sweats, anorexia

recurent infection due to dysfunctional lymphocytes causing decreased immunoglobulins can cause pneumonia

131
Q

FBC investigation for chronic lymphocytic leukaemia

A

increased WBCs, lymphocytes over 5000, must be persistent for over 3 months to make a diagnosis

decreased platelets

anaemia (may be haemolytic- do DAT test to see)

132
Q

what is seen on blood film in chronic lymphocytic leukaemia

A

lymphocytosis
smudge cells (lymphocytes damaged in slide preparation)

133
Q

staging system for chronic lymphocytic leukaemia

A

binet system (A-C)

134
Q

what does the Binet system take into account

A

based off of bloods (WBCs, Hb) and clinical findings (lymphadenopathy)

135
Q

do you do a CT for chronic lymphocytic leukaemia

A

not usually needed

136
Q

management of chronic lymphocytic leukaemia

A

if binet A-B (asymptomatic)- watchful waiting, 3 monthly FBC, flow cytometry and examination

if binet C (symptomatic)- chemotherapy

stem cell transplantation for refractory disease

137
Q

is chemotherapy effective for chronic lymphocytic leukaemia

A

up to 50% remission but most relapse

138
Q

prognosis for binet A chronic lymphocytic leukaemia

A

median survival of over 10 years and is unlikely to markedly limit life expectancy

139
Q

prognosis for binet C chronic lymphocytic leukaemia

A

median survival 2-3 years

140
Q

pathophysiology of chronic myeloid leukaemia

A

the clinical proliferation of myeloid stem cells which differentiate into granulocytes

mostly due to chromosome 9-22 reciprocal translocation, creating Philadelphia chromosome

141
Q

ages most commonly affected by chronic myeloid leukaemia

A

40-60

142
Q

which sex is chronic myeloid leukaemia more common in

A

males

143
Q

signs and symptoms of chronic myeloid leukaemia

A

often asymptomatic

systemtic= tired, malaise, weight, loss, fever, night sweats

splenomegaly, LLQ discomfort and satiety

cytopenia- anaemia (pallor), bleeding or bruising including epistaxis (nose bleeds)

gout/arthlagia (due to increased urate)

144
Q

FBC findings in chronic myeloid leukaemia

A

increased WBCs (can be over 100)

anaemia in 50%

increased platelets in chronic/accelerated phase, decreased platelets in blast phase

145
Q

what can be seen on blood film with chronic myeloid leukaemia

A

increased granulocytes especially neutrophils

146
Q

diagnosis of chronic myeloid leukaemia

A

bone marrow biopsy- will see granulocytic hyperplasia
cytogenetics- Philadelphia chromosome

147
Q

1st line management of chronic myeloid leukaemia

A

imatinib

148
Q

what is imatinib

A

tyrosine kinase inhibitor
inhibits BCR-ABL p210 tyrosine kinase and induces long term remission

149
Q

side effects of imatinib

A

cramps
oedema
rash
diarrhoea

150
Q

what to do if imatinib doesn’t work

A

try other tyrosine kinase inhibitors (end in nib)
if no remission then/if relapse can do stem cell transplantation

151
Q

prognosis of chronic myeloid leukaemia

A

worse if Philadelphia -ve

5 year survival is 90% but complete BCR/ABL eradication is rare

152
Q

what type of sepsis can chemotherapy cause

A

neutropenic sepsis
must be recognised early and given IV antibiotics within 1hr
give blood transfusion

153
Q

what is filgrastim

A

recombinant human granulocyte colony stimulating factor (G-CSF)

it stimulates production of neutrophils

reduces duration of neutropenia and neutropenic sepsis

154
Q

vitamin CDE causes of back pain

A

vascular= aortic aneurysm
infective/inflammatory= oestomyelitis, spinal abscess
trauma= injury resulting in fracture/muscle pain
autoimmune= ankylosing spondylitis, inflammatory arthritis
metabolic= renal stones (referred pain)
iatrogenic= n/a
neoplastic= spinal mets, multiple myeloma

congenital= spina bifida
degenerative= oesteoarthritis, disc herniation
endocrine= osteoporotic fracture

155
Q

signs and symptoms of hypercalcaemia

A

renal stones
confusion
nausea and vomiting
depression
polyuria
polydipsia
abdominal pain

156
Q

back pain, normocytic anaemia and hypercalcaemia suggest what

A

malignant cause- spinal metastases or multiple myeloma

157
Q

what may hypercalcaemia look like on ECG

A

shortened QT interval

158
Q

which medications can worsen AKI and need to be stopped when AKI present

A

NSAIDs
diuretics
ACE inhibitors
ARBs

159
Q

what is adcal d3

A

tablet used for low calcium and vitamin d

need to stop when hypercalcaemia

160
Q

initial management for hypercalcaemia

A

IV fluids (0.9% sodium chloride)

161
Q

what is pamidronate

A

bisphosphonate
used to treat hypercalcaemia

162
Q

other name for myeloma

A

multiple myeloma

163
Q

pathophysiology of multiple myeloma

A

malignant proliferation of plasma cells in bone marrow

causes bone marrow destruction via infiltration and bone destruction via increased RANKL activity (means increased osteoclast activity)

164
Q

which age is most commonly affected by multiple myeloma

A

incidence increases with age
rare under 55

165
Q

which sex is more commonly affected by multiple myeloma

A

male

166
Q

which race is more commonly affected by multiple myeloma

A

2 times more common in black people compared to white people

167
Q

signs and symptoms of multiple myeloma

A

osteolytic bone lesions can cause fractures causing back bone pain (and raised calcium)

marrow infiltration can cause pancytopenia which leads to anaemia, infection, bruising and bleeding

immunoparesis can lead to infection

168
Q

multiple myeloma and kidney damage

A

50% of people have renal disease at presentation

cast nephropathy= light chains (pence-jones protein) deposit in the kidneys and aggregate with Tamm-horsfall proteins in the loop of henle causing tubular obstruction and kidney failure

can also damage kidneys via monoclonal immunoglobulin deposition disease (affecting the glomerular basement membrane) or light chain (AL) amyloidosis

169
Q

blood results in multiple myeloma

A

normocytic anaemia

raised ESR

raised urea and creatinine

increased Ca2+

170
Q

prognostic tests for multiple myeloma

A

beta2-microglobulin and albumin

171
Q

what can be seen on blood film in multiple myeloma

A

rouleaux formation (stacked RBCs)

172
Q

imaging done for multiple myeloma

A

1st line= full body MRI
2nd line= CT
3rd line= skeletal survey

173
Q

screening for multiple myeloma

A

serum electrophoresis and ESR on all patients over 50 with new back pain

174
Q

diagnosis of multiple myeloma

A

need both:

bone marrow aspirate and/or biopsy showing clonal plasma cells 10% or over (should be less than 5% normally) or biopsy proven plasmacytoma

and

one or more of myeloma related organ dysfunction (increased Ca2+, renal insufficiency, anaemia, lytic bone lesions) or biomarkers suggesting very high risk progression to organ dysfunction

serum and urine protein electrophoresis, bone marrow examination and whole body low dose CT

175
Q

specific treatment for multiple myeloma

A

haematopoietic stem cell transplantation (clean stem cells and put back in) + induction chemotherapy

chemotherapy if unsuitable for above

176
Q

2 complications of multiple myeloma

A

hyperviscosity syndrome
spinal cord compression

177
Q

prognosis of multiple myeloma

A

median survival 3-4 years
death usually from infection/kidney failure

178
Q

what is hyperviscosity syndrome

A

increase in plasma viscosity usually due to elevated immunoglobulins, RBCs, WBCs

179
Q

sign and symptoms of hyperviscosity syndrome

A

triad of:
neurological symptoms- impaired cognition, headaches, seizures

visual changes from retinopathy

mucosal bleeding

180
Q

management of hyperviscosity syndrome

A

plasmapheresis

181
Q

what happens to parathyroid hormone in multiple myeloma

A

it is suppressed due to increased calcium

182
Q

what proteins may be detected in people with multiple myeloma

A

bence jones proteins which are monoclonal globulins which can be detected in the urine

183
Q

what is the second most common haematological cancer

A

multiple myeloma

184
Q

genetic and environmental factors affecting multiple myeloma

A

there are no known factors which increase your risk of multiple myeloma

185
Q

main antibodies associated with multiple myeloma

A

IgG
IgA

186
Q

do most people with lymphoma/leukeamia have family history of the disease

A

no, there is a very small increased risk if there is a family history but most do not have

187
Q

which haematological cancers can be cured

A

acute myeloid leukaemia

diffuse large B cell lymphoma

classical Hodgkins lymphoma (best survival rate)

semester 2 (3rd year) (12 decks)

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