Case 20: fatigue and neck swelling Flashcards
what is haematopoiesis
formation of all mature blood cells from haematopoietic stem cells
where does haematopoiesis occur
bone marrow
what are the two lineages of haematopoiesis
myeloid and lymphoid
examples of myeloid cells
thrombocytes (platelet)
basophils
neutrophils
eosinophils
macrophages (monocytes)
erythrocytes
examples of lymphoid cells
natural killer cells
T lymphocytes
B lymphocytes
plasma cells
process of haematopoiesis
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
what does the term blast mean relative to cells
blasts refers to immature forms of cells
where are blasts usually seen
in the bone marrow
what does it mean when blasts are seen in the peripheral blood
suggest haematological malignancy
what us the lymphatic system comprised of
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
what happens in bone marrow and thymus
these are the organs in which the immune cells develop
what are the different areas of a lymph node
the cortex (surrounds)
medulla (middle)
mantle zone (areas in the medulla)
follicle (found in mantle zone)
germinal centre (the centre of the follicle)
other names for infectious mononucleosis
glandular fever
mono
causes of infectious mononucleosis
EBV aka HHV-4
also CMV, syphilis, HIV seroconversion, toxoplasma, brucella
signs and symptoms of infectious mononucleosis
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
diagnosis of infectious mononucleosis
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
what should you do to avoid splenic rupture with infectious mononucleosis
avoid contact sports and alcohol
management of infectious mononucleosis
usually self resolving
paracetamol for fever and pain
prednisolone if airway obstruction or haemolytic anaemia
how is EBV spread
saliva/droplets (kissing is common)
what % of people have been exposed to EBV
90%
long term complications of EBV
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
what are Bs symptoms
fever
weight loss
might sweats
neck lump
what would you do if B symptoms were present
urgent 2 week wait referral to haematologist
does leukaemia usually present with lymphadenopathy
no not usually
does lymphoma usually present with lymphadenopathy
yes
might see bilateral hilar lymphadenopathy on CXR
if you suggest haematological malignancy which type of biopsy is suggested
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
lymphoma vs leukaemia
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
Hodgkins lymphoma is what % of all lymphomas
20%
what is seen on light microscopy with Hodgkins lymphoma
mirror-image binucleated Reed-Sternberg cells
which type of cancer cell is Hodgkins lymphoma
B cell cancer
subtypes of Hodgkins lymphom
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
ages most commonly affected by Hodgkins lymphoma
15-30 and 75-80
is Hodgkins lymphoma more common in men or women
men
lymphadenopathy symptoms of Hodgkins lymphoma
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
B symptoms of Hodgkins lymphoma
weight loss
fever (Pel-Ebstein fever every 2-4 weeks= rare)
night sweats
lethargy
pruritus
other features of Hodgkins lymphoma
anaemia of chronic disease
hepatosplenomegaly in advanced disease
risk factors for Hodgkins lymphoma
family history
EBV
increases socio-economic status
what staging is used for both Hodgkins lymphoma and non-Hodgkins lymphoma
ann arbor staging system
what does the ann arbor staging system take into account
location (lymph nodes and beyond)
presence/ absence of systemic symptoms
management of Hodgkins lymphoma
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
what % of all lymphomas is non-hodgkins lymphoma
80%
what is non-hodgkins lymphoma
it is a diverse group of conditions with proliferating cells potentially accumulating in lymph nodes, MALT, CNS and skin
what type of cell cancer is non-hodgkins lymphoma
90% is B cell proliferation
10% is T cell proliferation
2 sub-types of non-hodgkins lymphoma
low grade lymphoma
high grade lymphoma
is high or low grade non-hodgkins lymphoma easier to cure
low-grade= slow growing, good prognosis, but hard to cure
high-grade= more acute but easier to cure
examples of low grade non-hodgkins lymphoma
follicular lymphoma (CD20 +ve)
marginal zone lymphoma
lymphocytic lymphoma
waldenstroms macroglobulinaemia aka lymphoplasmacytoid (increased IgM)
examples of high grade non-hodgkins lymphoma
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
lymphadenopathy signs of symptoms of non-hodgkins lymphoma
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)
extra nodal manifestations of non-hodgkins lymphoma
can be anywhere meaning almost any symptom
gut- abdo pain, weight loss, dyspepsia
skin- T cell lymphoma, rash, discolouration
oropharynx- sore throat, obstructed breathing
are B symptoms present in non-hodgkins lymphoma
they are much more common in Hodgkins lymphoma rather than non-hodgkins lymphoma
can be quite frequent in high grade non-hodgkins lymphoma however
risk factors for non-hodgkins lymphoma
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)
management of low grade non-hodgkins lymphoma
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
management of high grade non-hodgkins lymphoma
chemotherapy
autologous or allogenic stem cell transplant in refractory cases
what size of lymphadenopathy is concerning in adults
more than 1cm (if not related to infection)
any size if have persistent head and neck symptoms)
what size of lymphadenopathy is concerning in children
over 2cm
inflammatory causes of lymphadenopathy
secondary to infection- bacterial/viral (tonsils, teeth, ear, scalp)
TB (cervical)
HIV
how do the lumps feel in lymphoma
multiple often smooth and firm rather than hard
rubbery
how do the lumps feel when the lymph nodes are metastatic
fixed and irregular
vast majority come from head and neck cancer
most common primary cancers causing metastatic nodes
mucosal squamous carcinoma (oral, pharynx, larynx)
thyroid cancer
salivary gland cancer
skin cancer (squamous, melanoma- ear/scalp)
what does a fibreoptic endoscopy look at
mouth
nasal cavity
larynx
pharynx
risk factors for head and neck cancer
smoking
alcohol
HPV (for tonsilar cancer)
cannabis
how does a PETCT scan help diagnose in malignancies
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
stage I-IV lymphoma
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)
stage A and B lymphoma
A= no systemic symptoms
B= systemic symptoms present (weight loss, fever, night sweats)
chemotherapy for lymphoma increases your risk of what
infection due to reduced white cells (neutropenia)
cure rate for Hodgkins lymphoma
over 80% are cured
relapse after 5 years is rare
what blood is a key prognostic factor in diffuse large B cell non-hodgkins lymphoma
lactate dehydrogenase
how to treat neutropenia + pyrexia following chemotherapy for lymphoma
need immediate IV antibiotics (can be deadly due to neutropenic sepsis)
what is R-CHOP treatment
used to treat NHL
chemoimmmuntherapy plus steroid
what does R-CHOP treatment target
CD20 antibody
rituximab
cure rate for diffuse large B cell non-hodgkins lymphoma
50% are cured
relapse after 5 years is rare
two most common types of B cell non-hodgkins lymphoma
diffuse large B cell (most common)
follicular (second most common)
features of follicular non-hodgkins lymphoma
typically no symptoms
responds well to treatment but recurrent relapses- incurable
median survival of follicular non-hodgkins lymphoma
10 years
treatment of follicular non-hodgkins lymphoma
radiotherapy (targets specific nodes affected)
often daily for a short course to manage disease progression
when considering lymphoma which part of the abdominal examination is important
palpation of liver and spleen as can see hepatic-splenomegaly
what is seen histologically with non-hodgkins lymphoma
diffuse or nodular abnormal lymphocytes
features of low grade NHL
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
features of high grade NHL
quicker growing and symptomatic
more likely to be completely cured with chemotherapy
examples= diffuse large B cell and Burkitt lymphomas
womens fertility and Hodgkins lymphoma
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
long term risks following HL
second cancers
cardiac problems
hormonal problems
patients with HL who have received chemotherapy and radiotherapy are at a greater risk of developing which cancer
lung (smoking cessation necessary)
which age is typically affected by NHL
median age is 55
is HL or NHL more common
NHL
what is a non blanching rash
a rash which does not disappear with pressure
what is purpura
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
petechiae vs ecchymosis
petechiae= purpura less than 1cm
ecchymosis= purpura greater than 1cm
main pathophysiological causes of petechiae
thrombocytopenia
platelet dysfunction
disorders of coagulation
loss of vascular integrity
vitamin cde causes of petechial rash
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
causes of non-thrombocytopenic purpura
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
causes of thrombocytopenic purpura
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
how does a blood clot form
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
what is ADAMTS13
an enzyme which breaks down vWF when it is no longer needed
what is thrombotic thrombocytopenic purpura
rare serious blood disease causing many small blood clots throughout the body
severe deficiency of ADAMTS13
clinical findings of thrombotic thrombocytopenic purpura in blood
low platelets
increased RBC destruction
signs and symptoms of thrombotic thrombocytopenic purpura
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
complication of thrombotic thrombocytopenic purpura
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
types of thrombotic thrombocytopenic purpura
immune mediated/ acquired thrombotic thrombocytopenic purpura
and
congential/familial thrombotic thrombocytopenic purpura
features of immune mediated/ acquired thrombotic thrombocytopenic purpura
more common and autoimmune
develops late childhood/adulthood
antibodies against ADAMTS13
features of congential/familial thrombotic thrombocytopenic purpura
autosomal recessive
very low levels of ADAMTS13
diagnosis of thrombotic thrombocytopenic purpura
needs to be urgent
deficiency of less than 10% ADAMTS13 activity on bloods
for autoimmune need anti-ADAMTS13 antibodies in blood
treatment of thrombotic thrombocytopenic purpura
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
what is leukaemia
malignant neoplastic process involving one of the WBC lines (neutrophils, lymphocytes, monocytes, etc)
main classifications of leukaemia
is either myeloid or lymphoid
then further classified as acute or chronic
what is seen on blood film with acute leukaemia
peripheral blood film is dominated by immature (blast) cells therefore is referred to as acute myeloblastic or lymphoblastic leukaemia
issues with blast cells crowding the bone marrow
means the marrow is unable to produce healthy blood cells- can result in patient being anaemic and/or thrombocytopenic
which is the most common childhood cancer
acute lymphocytic leukaemia
pathology of acute lymphocytic leukaemia
malignant clonal disease that develops when B/T precursor stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergo uncontrolled proliferation
age commonly affected by acute lymphocytic leukaemia
under 20s
accounts for 80% of leukaemia in paediatrics and 20% in adults
pathology of acute myeloid leukaemia
clinical expansion of myeloid blasts in the bone marrow, peripheral blood, or extra-medullary tissues
ages affected by acute myeloid leukaemia
risk increases with age
which sex is more commonly affected by acute leukaemia
male
signs and symptoms of acute leukaemia
anaemia- SOB, fatigue, pallor
infection- there is increased WBCs but low neutrophils (neutropenia), low grade fever
bleeding- bruising, menorrhagia, internal
signs and symptoms of acute leukaemia causing organ infiltration
hepatosplenomegaly
lymphadenopathy
CNS (especially in ALL)- CN palsy, papilloedma, meningism unilateral swelling of testes (especially in ALL)
gum hypertrophy, skin nodules
mediastinal mass (thymus)
risk factors of acute leukaemia
chemotherapy/radiation exposure
previous haematological disease (for AML) such as myelodysplastic syndrome, paroxysmal nocturnal haemoglobinuria
family history
genetic- downs syndrome, fanconis anaemia, ataxia telangiectasia
blood findings in acute leukaemia
pancytopenia (low red, white and platelets), but neutropenia
may have disseminated intravascular coagulation
may have raised urea and creatinine
increased LFTs
increased lactate dehydrogenase
what is seen on blood film with acute leukemias
lymphoblasts seen in ALL
myeloblasts containing Auer rods in AML
diagnosis of acute leukemia
bone marrow biopsy and/or aspiration, plus biopsy of infiltrated organs
blast cells above 20% confirm diagnosis
which test identifies the subtype of acute leukaemia
immunophenotyping
what test provides prognostic and therapeutic info about acute leukaemia
cytogenics
translocations:
philadelphia chromsome in adults with ALL
t(12;21) in kids with ALL
t(15;17) in acute promyeloid leukaemia (AML variant)
supportive management of acute leukemias
for pancytopenia- RBC and platelet transfusion, antibiotics
allopurinol if increased uric acid from tumour lysis
chemotherapy duration for AML
6-8 months
chemotherapy duration for ALL
up to 3 years
iatrogenic complications of acute leukaemia
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
prognosis of ALL
5 year survival:
90% in children
50% in adults
prognosis of AML
5 year survival:
20% overall
75% if under 60
pathophysiology of chronic lymphocytic leukaemia
there is proliferation of mature B cells and accumulation in the blood and bone marrow
which is the commonest adult leukaemia
CLL
which sex is most affected by chronic lymphocytic leukaemia
male
most common presentation of chronic lymphocytic leukaemia
90% are asymptomatic
usually incidental findings of increased WBCs
can see lymphadenopathy, splenomegaly and hepatomegaly
symptomatic presentation of chronic lymphocytic leukaemia
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
FBC investigation for chronic lymphocytic leukaemia
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)
what is seen on blood film in chronic lymphocytic leukaemia
lymphocytosis
smudge cells (lymphocytes damaged in slide preparation)
staging system for chronic lymphocytic leukaemia
binet system (A-C)
what does the Binet system take into account
based off of bloods (WBCs, Hb) and clinical findings (lymphadenopathy)
do you do a CT for chronic lymphocytic leukaemia
not usually needed
management of chronic lymphocytic leukaemia
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
is chemotherapy effective for chronic lymphocytic leukaemia
up to 50% remission but most relapse
prognosis for binet A chronic lymphocytic leukaemia
median survival of over 10 years and is unlikely to markedly limit life expectancy
prognosis for binet C chronic lymphocytic leukaemia
median survival 2-3 years
pathophysiology of chronic myeloid leukaemia
the clinical proliferation of myeloid stem cells which differentiate into granulocytes
mostly due to chromosome 9-22 reciprocal translocation, creating Philadelphia chromosome
ages most commonly affected by chronic myeloid leukaemia
40-60
which sex is chronic myeloid leukaemia more common in
males
signs and symptoms of chronic myeloid leukaemia
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)
FBC findings in chronic myeloid leukaemia
increased WBCs (can be over 100)
anaemia in 50%
increased platelets in chronic/accelerated phase, decreased platelets in blast phase
what can be seen on blood film with chronic myeloid leukaemia
increased granulocytes especially neutrophils
diagnosis of chronic myeloid leukaemia
bone marrow biopsy- will see granulocytic hyperplasia
cytogenetics- Philadelphia chromosome
1st line management of chronic myeloid leukaemia
imatinib
what is imatinib
tyrosine kinase inhibitor
inhibits BCR-ABL p210 tyrosine kinase and induces long term remission
side effects of imatinib
cramps
oedema
rash
diarrhoea
what to do if imatinib doesn’t work
try other tyrosine kinase inhibitors (end in nib)
if no remission then/if relapse can do stem cell transplantation
prognosis of chronic myeloid leukaemia
worse if Philadelphia -ve
5 year survival is 90% but complete BCR/ABL eradication is rare
what type of sepsis can chemotherapy cause
neutropenic sepsis
must be recognised early and given IV antibiotics within 1hr
give blood transfusion
what is filgrastim
recombinant human granulocyte colony stimulating factor (G-CSF)
it stimulates production of neutrophils
reduces duration of neutropenia and neutropenic sepsis
vitamin CDE causes of back pain
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
signs and symptoms of hypercalcaemia
renal stones
confusion
nausea and vomiting
depression
polyuria
polydipsia
abdominal pain
back pain, normocytic anaemia and hypercalcaemia suggest what
malignant cause- spinal metastases or multiple myeloma
what may hypercalcaemia look like on ECG
shortened QT interval
which medications can worsen AKI and need to be stopped when AKI present
NSAIDs
diuretics
ACE inhibitors
ARBs
what is adcal d3
tablet used for low calcium and vitamin d
need to stop when hypercalcaemia
initial management for hypercalcaemia
IV fluids (0.9% sodium chloride)
what is pamidronate
bisphosphonate
used to treat hypercalcaemia
other name for myeloma
multiple myeloma
pathophysiology of multiple myeloma
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)
which age is most commonly affected by multiple myeloma
incidence increases with age
rare under 55
which sex is more commonly affected by multiple myeloma
male
which race is more commonly affected by multiple myeloma
2 times more common in black people compared to white people
signs and symptoms of multiple myeloma
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
multiple myeloma and kidney damage
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
blood results in multiple myeloma
normocytic anaemia
raised ESR
raised urea and creatinine
increased Ca2+
prognostic tests for multiple myeloma
beta2-microglobulin and albumin
what can be seen on blood film in multiple myeloma
rouleaux formation (stacked RBCs)
imaging done for multiple myeloma
1st line= full body MRI
2nd line= CT
3rd line= skeletal survey
screening for multiple myeloma
serum electrophoresis and ESR on all patients over 50 with new back pain
diagnosis of multiple myeloma
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
specific treatment for multiple myeloma
haematopoietic stem cell transplantation (clean stem cells and put back in) + induction chemotherapy
chemotherapy if unsuitable for above
2 complications of multiple myeloma
hyperviscosity syndrome
spinal cord compression
prognosis of multiple myeloma
median survival 3-4 years
death usually from infection/kidney failure
what is hyperviscosity syndrome
increase in plasma viscosity usually due to elevated immunoglobulins, RBCs, WBCs
sign and symptoms of hyperviscosity syndrome
triad of:
neurological symptoms- impaired cognition, headaches, seizures
visual changes from retinopathy
mucosal bleeding
management of hyperviscosity syndrome
plasmapheresis
what happens to parathyroid hormone in multiple myeloma
it is suppressed due to increased calcium
what proteins may be detected in people with multiple myeloma
bence jones proteins which are monoclonal globulins which can be detected in the urine
what is the second most common haematological cancer
multiple myeloma
genetic and environmental factors affecting multiple myeloma
there are no known factors which increase your risk of multiple myeloma
main antibodies associated with multiple myeloma
IgG
IgA
do most people with lymphoma/leukeamia have family history of the disease
no, there is a very small increased risk if there is a family history but most do not have
which haematological cancers can be cured
acute myeloid leukaemia
diffuse large B cell lymphoma
classical Hodgkins lymphoma (best survival rate)
