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yr 5 haem > haem malignancy > Flashcards

haem malignancy Flashcards

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1
Q

what is burkitts lymphoma

A

-a high grade B cell lymphoma
-presents with a large abdominal mass, SVC and symptoms of bowel obstruction
-linked to EBV
-starry sky appearance

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2
Q

Rf for NHL

A

HIV, EBV, H pylori, Hep B/C

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3
Q

what cancer can hepB/C turn into

A

diffuse large B cell

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4
Q

what is the spread like in NHL

A

non contiguous

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5
Q

is extranodal involvement more common in HL or NHL

A

NHL

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6
Q

gold standard Ix for lymphoma

A

excisional LN biopsy (use CT to find LN) and the PET

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7
Q

bloods for lymphoma

A

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

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8
Q

worse prognostic factor for DLBCL

A

MYC arrangement

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9
Q

what’s another important management consideration for lymphoma

A

must receive irradiated blood products

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10
Q

complications of lymphoma

A

SVC obstruction, pericardial effusion, anaemia, hyperviscosity

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11
Q

in what kind of lymphoma does a malignant transformation most commonly occur in?

A

a low grade (FOLLICULAR) into high grade - DLBCL

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12
Q

NHL can be B, T or NKC lymphoma, which is worse

A

T cell

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13
Q

what is the definition of a lymphoma

A

uncontrolled proliferation of lymphocytes which are mature and originate outside the bone marrow (unlike in a leukaemia)

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14
Q

is NHL or HL more common

A

NHL

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15
Q

what is the distribution of HL

A

bimodal

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16
Q

aetiology of NHL

A

EBV, immunosuppression, FHx

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17
Q

B symptoms in lymphoma (much more common in HL)

A

night sweats, fever, weight loss

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18
Q

what is the 2WW for lymphoma

A

unexplained lymphadenopathy and symptoms - 2WW in adults and 48 hours in children/adolscents

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19
Q

what staging classification is used for lymphoma

A

lugano

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20
Q

poor prognostic factors of a HL

A

lymphocyte deplete, men, B symptoms, increasing age

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21
Q

poor prognostic factors of NHL

A

increasing age, type of lymphoma eg High grade vs low grade, performance status, LDH

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22
Q

what is seen on a excision LN biopsy of HL

A

reed steinberg cells

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23
Q

what is seen on blood film of AML

A

Auer rods

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24
Q

when is a diagnosis of AML given

A

when there are >20% of blast cells seen on BM

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25
bad prognostic factor for AML
age
26
phases of chemo treatment for a leukaemia
1) induction - high dose 2) consolidation 3) maintenance
27
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
28
genetic link to CML
9:22 translocation (philadelphia chromosome) which inhibits DNA repair
29
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
30
what do bloods show in CML
anaemia and leucocytosis, thrombocytosis!!
31
what does a blood film show for CML
granulocytosis at different stages of maturation
32
what other investigation is important for leukaemia
FISH to look for the Philadelphia chromosome
33
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
34
what is the most common leukaemia in children
ALL (linked to T21)
35
how can immunophenotyping be done
by flow cytometry to see what antigens Are on the cells
36
how many blast cells are need on BM biopsy for a diagnosis of leukaemia to be given
20%!!!
37
poor prognostic factors for ALL
age, higher WBC count, disease subtype with T cell being worse
38
what is a good prognostic factorof ALL
hyperdiploid blast cells
39
what is richters transformation
in CLL when it transforms into a DLCBL
40
what is seen on blood smear of a CLL
smear and smudge cells
41
what haemolytic anaemia is CLL associated with
warm
42
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
43
if anyone presents to the GP with a suspected leukaemia, what should happen next
they should have an FBC within 48 hours
44
complications of leukaemia
hyperviscosity syndromes, secondary cancers and infection
45
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
46
what bacteria is most likely to cause neut sepsis from an infection on an indwelling line
staphylococcus epidermis (gram positive, coagulase negative)
47
if not getting better on tazocin for neutropenic sepsis, what do you add
vancomycin
48
if no improvement on abx after 4-6 days with neutropenic sepsis, what would you then consider
fungal infection
49
side effect of GCSF
bone pain and nausea
50
apart from chemo, what are some other causes of neutropenia
aplastic anaemia and hypersplenism
51
how many of the bodies platelets are held in the spleen
90%
52
what are the causes of hyposplenism
coeliac disease, graves, SCD, splenectomy
53
potential indications for a splenectomy
ITP, hereditary spherocytosis, trauma
54
what is mild splenomegaly classed as
spleen palpable 1-3cm below the costal margin
55
what is moderate splenomegaly classed as
between umbilicus and midline
56
what is massive splenomegaly classed as
crosses the midline and umbilicus
57
what are some causes for massive splenomegaly
visceral leishmaniasis and CML
58
what are some causes for a mild splenomegaly
haemolytic anaemias, EBV, portal hypertension
59
risk factors for TLS (apart from type of cancer)
pre existing renal impairment, dehydration, age
60
electrolyte abnormalities in TLS
hyperphosphataemia, hyperuricaemia, hyperkalaemia and hypocalcaemia (due to the excess P binding with it)
61
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)
62
complications of TLS
AKI (due to uric acid precipitates), arrhythmias, seizures
63
how does TLS present
cramps, N+V, syncope, reduced urine output
64
Mx of high risk TLS
rasburicase
65
Mx of lower risk TLS
allopurinol
66
definition of myeloma
clonal proliferation of abnormal B lymphocytes which then secrete non functioning immunoglobulins (known as paraproteins)
67
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
68
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
69
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
70
for a diagnosis of myeloma, what should the Level of paraprotein be
>30
71
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
72
options for drug Tx for myeloma
targeted drugs, steroids, chemo (cyclophosphamide), transfusions, bisphosphonates for bone pain
73
complications of myeloma
hyperviscosity syndromes, pain, pathological fractures, infection, fatigue
74
what is MGUS
paraprotein found but <30g/L and no sign of end organ damage
75
what is the chance MGUS will progress to myeloma
1-2%
76
Rf of myeloma
old, fat, black, man
77
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.
78
what are the normal ways in which amyloidosis presents
renal disease, cardiac disease and neurological disease complications -->ESRF, heart failure and Alzheimers
79
definitive diagnosis of amyloidosis
biopsy from affected organ and stain with Congo red (see apple green birefringence)
80
Mx of amyloidosis
antimyeloma therapy
81
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)
82
what is the course like for ITP
insidious onset and has a relapsing and remitting course
83
Investigations for ITP
diagnosis of exclusion, FBC and bloods film should show isolated thrombocytopenia
84
Mx of ITP
supportive but may need steroids if platelet count <30. Give IVIG + platelets if actively bleeding / life threatening.
85
what does polychromasia mean
more immature red blood cells in the blood film than you would expect
86
chemo regime for NHL (DLBCL)
R-CHOP (rituximab, doxorubicin, pred, vincristine)
87
most common type of paraprotein in myeloma
IgG (then IgA)
88
poor prognosis of myeloma
1) renal involvement 2) multiple bone lesions 3) very high level of paraprotein

yr 5 haem (5 decks)

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