haem malignancy Flashcards
what is burkitts lymphoma
-a high grade B cell lymphoma
-presents with a large abdominal mass, SVC and symptoms of bowel obstruction
-linked to EBV
-starry sky appearance
Rf for NHL
HIV, EBV, H pylori, Hep B/C
what cancer can hepB/C turn into
diffuse large B cell
what is the spread like in NHL
non contiguous
is extranodal involvement more common in HL or NHL
NHL
gold standard Ix for lymphoma
excisional LN biopsy (use CT to find LN) and the PET
bloods for lymphoma
FBC (anaemia, thrombocytopenia, lymphocytosis), LDH (higher this is, the worse the prognosis), U+E, ESR (prognostic value), also can do viral screen for HIV and HepB/C
worse prognostic factor for DLBCL
MYC arrangement
what’s another important management consideration for lymphoma
must receive irradiated blood products
complications of lymphoma
SVC obstruction, pericardial effusion, anaemia, hyperviscosity
in what kind of lymphoma does a malignant transformation most commonly occur in?
a low grade (FOLLICULAR) into high grade - DLBCL
NHL can be B, T or NKC lymphoma, which is worse
T cell
what is the definition of a lymphoma
uncontrolled proliferation of lymphocytes which are mature and originate outside the bone marrow (unlike in a leukaemia)
is NHL or HL more common
NHL
what is the distribution of HL
bimodal
aetiology of NHL
EBV, immunosuppression, FHx
B symptoms in lymphoma (much more common in HL)
night sweats, fever, weight loss
what is the 2WW for lymphoma
unexplained lymphadenopathy and symptoms - 2WW in adults and 48 hours in children/adolscents
what staging classification is used for lymphoma
lugano
poor prognostic factors of a HL
lymphocyte deplete, men, B symptoms, increasing age
poor prognostic factors of NHL
increasing age, type of lymphoma eg High grade vs low grade, performance status, LDH
what is seen on a excision LN biopsy of HL
reed steinberg cells
what is seen on blood film of AML
Auer rods
when is a diagnosis of AML given
when there are >20% of blast cells seen on BM
bad prognostic factor for AML
age
phases of chemo treatment for a leukaemia
1) induction - high dose
2) consolidation
3) maintenance
what supportive therapies may be given in a leukaemia
blood cell transfusion, analgesia, antimicrobial prophylaxis, allopurinol or rasburiscase, fertility support, mental health support, nutritional support
genetic link to CML
9:22 translocation (philadelphia chromosome) which inhibits DNA repair
what are the phases of CML
1) chronic - often asymptomatic and people get diagnosed incidentally by a high WCC
2) accelerated - myeloid blasts take up 10-20% of bone marrow and people become symptomatic - splenomegaly / abdominal discomfort/ infection / bleeding
2) blast stage - behaves like an acute leukaemia
what do bloods show in CML
anaemia and leucocytosis, thrombocytosis!!
what does a blood film show for CML
granulocytosis at different stages of maturation
what other investigation is important for leukaemia
FISH to look for the Philadelphia chromosome
CML is normally treated with targeted therapy, like imatinib. but is can be treated with hydroxycarbamide - what is this
it is an antimetabolite which suppresses all cell lines but suppress the highest first
what is the most common leukaemia in children
ALL (linked to T21)
how can immunophenotyping be done
by flow cytometry to see what antigens Are on the cells
how many blast cells are need on BM biopsy for a diagnosis of leukaemia to be given
20%!!!
poor prognostic factors for ALL
age, higher WBC count, disease subtype with T cell being worse
what is a good prognostic factorof ALL
hyperdiploid blast cells
what is richters transformation
in CLL when it transforms into a DLCBL
what is seen on blood smear of a CLL
smear and smudge cells
what haemolytic anaemia is CLL associated with
warm
what is overall survival like for CLL
long overall survival, its normally a slow proliferation. 1/3 static, 1/3 slow progressing and 1/3 rapidly progressing
if anyone presents to the GP with a suspected leukaemia, what should happen next
they should have an FBC within 48 hours
complications of leukaemia
hyperviscosity syndromes, secondary cancers and infection
key investigations for a leukaemia
1) FBC
2) blood film
3) bone marrow - aspiration and trephine
4) cytogenetics / molecular tests - FISH
5) immunophenotyping - flow cytometry - looks at the antigens on the surface
6) may do a LP to investigate for CNS involvement in ALL
7) may do imaging to look for mediastinal mass
what bacteria is most likely to cause neut sepsis from an infection on an indwelling line
staphylococcus epidermis (gram positive, coagulase negative)
if not getting better on tazocin for neutropenic sepsis, what do you add
vancomycin
if no improvement on abx after 4-6 days with neutropenic sepsis, what would you then consider
fungal infection
side effect of GCSF
bone pain and nausea
apart from chemo, what are some other causes of neutropenia
aplastic anaemia and hypersplenism
how many of the bodies platelets are held in the spleen
90%
what are the causes of hyposplenism
coeliac disease, graves, SCD, splenectomy
potential indications for a splenectomy
ITP, hereditary spherocytosis, trauma
what is mild splenomegaly classed as
spleen palpable 1-3cm below the costal margin
what is moderate splenomegaly classed as
between umbilicus and midline
what is massive splenomegaly classed as
crosses the midline and umbilicus
what are some causes for massive splenomegaly
visceral leishmaniasis and CML
what are some causes for a mild splenomegaly
haemolytic anaemias, EBV, portal hypertension
risk factors for TLS (apart from type of cancer)
pre existing renal impairment, dehydration, age
electrolyte abnormalities in TLS
hyperphosphataemia, hyperuricaemia, hyperkalaemia and hypocalcaemia (due to the excess P binding with it)
how often do bloods need to be done in TLS and what are they
6 HOURLY
U+E, bone profile, uric acid, LDH (note a high LDH is a RF for TLS)
complications of TLS
AKI (due to uric acid precipitates), arrhythmias, seizures
how does TLS present
cramps, N+V, syncope, reduced urine output
Mx of high risk TLS
rasburicase
Mx of lower risk TLS
allopurinol
definition of myeloma
clonal proliferation of abnormal B lymphocytes which then secrete non functioning immunoglobulins (known as paraproteins)
CRABBI mneumonic for myeloma
Calcium - high due to increased osteoclast activity and renal dysfunction
Renal - SFLC deposition –> damage + stones
Anaemia
Bleeding
Bone pain (lytic lesions)
Infection
people over 60, who present with persistent bone pain or an explained fracture should have what tested
FBC, Ca, PV and ESR. If anyone then has features suggested of myeloma, they should have a myeloma SPE and bence jones within 48 hours
-if someone presents to GP with hypercalcaemia and features suggestive myeloma do a SPE and bench jones
-if any of the following suggest myeloma they need a 2WW
investigations for myeloma
bloods - anaemia, U+E - renal failure, bone profile - hypercalcaemia, normal P and normal ALP, ESR - raised
Blood film - rouleax formation
SPE - looks for the amount of immunoglobulins in blood
SFLC assay
bence jones
serum immunofixation - looks for the type of paraprotein
Bone marrow biopsy (>10% clonal plasma cells for diagnosis)
whole body MRI
for a diagnosis of myeloma, what should the Level of paraprotein be
> 30
what criteria needs to be met for myeloma to be diagnosed
1) clonal plasma cells >10% on bone marrow biopsy
2) paraprotein
3) evidence of end organ damage
options for drug Tx for myeloma
targeted drugs, steroids, chemo (cyclophosphamide), transfusions, bisphosphonates for bone pain
complications of myeloma
hyperviscosity syndromes, pain, pathological fractures, infection, fatigue
what is MGUS
paraprotein found but <30g/L and no sign of end organ damage
what is the chance MGUS will progress to myeloma
1-2%
Rf of myeloma
old, fat, black, man
what is amyloidosis
deposition of abnormal protein (beta pleated sheets) in various organs and tissues. It can be caused by myeloma but there are other causes too.
what are the normal ways in which amyloidosis presents
renal disease, cardiac disease and neurological disease
complications –>ESRF, heart failure and Alzheimers
definitive diagnosis of amyloidosis
biopsy from affected organ and stain with Congo red (see apple green birefringence)
Mx of amyloidosis
antimyeloma therapy
what paraprotein spike do you get in waldenstroms macroglobulinaemia
IgM (this is a rare condition which can affect older men. Presents with systemic upset and hyperviscosity syndromes)
what is the course like for ITP
insidious onset and has a relapsing and remitting course
Investigations for ITP
diagnosis of exclusion, FBC and bloods film should show isolated thrombocytopenia
Mx of ITP
supportive but may need steroids if platelet count <30. Give IVIG + platelets if actively bleeding / life threatening.
what does polychromasia mean
more immature red blood cells in the blood film than you would expect
chemo regime for NHL (DLBCL)
R-CHOP
(rituximab, doxorubicin, pred, vincristine)
most common type of paraprotein in myeloma
IgG (then IgA)
poor prognosis of myeloma
1) renal involvement
2) multiple bone lesions
3) very high level of paraprotein
