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yr 5 haem > haem malignancy > Flashcards

haem malignancy Flashcards

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 NHL

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
Q

bad prognostic factor for AML

A

age

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26
Q

phases of chemo treatment for a leukaemia

A

1) induction - high dose
2) consolidation
3) maintenance

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27
Q

what supportive therapies may be given in a leukaemia

A

blood cell transfusion, analgesia, antimicrobial prophylaxis, allopurinol or rasburiscase, fertility support, mental health support, nutritional support

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28
Q

genetic link to CML

A

9:22 translocation (philadelphia chromosome) which inhibits DNA repair

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29
Q

what are the phases of CML

A

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

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30
Q

what do bloods show in CML

A

anaemia and leucocytosis, thrombocytosis!!

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31
Q

what does a blood film show for CML

A

granulocytosis at different stages of maturation

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32
Q

what other investigation is important for leukaemia

A

FISH to look for the Philadelphia chromosome

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33
Q

CML is normally treated with targeted therapy, like imatinib. but is can be treated with hydroxycarbamide - what is this

A

it is an antimetabolite which suppresses all cell lines but suppress the highest first

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34
Q

what is the most common leukaemia in children

A

ALL (linked to T21)

35
Q

how can immunophenotyping be done

A

by flow cytometry to see what antigens Are on the cells

36
Q

how many blast cells are need on BM biopsy for a diagnosis of leukaemia to be given

A

20%!!!

37
Q

poor prognostic factors for ALL

A

age, higher WBC count, disease subtype with T cell being worse

38
Q

what is a good prognostic factor

A

hyperdiploid blast cells

39
Q

what is richters transformation

A

in CLL when it transforms into a DLCBL

40
Q

what is seen on blood smear of a CLL

A

smear and smudge cells

41
Q

what haemolytic anaemia is CLL associated with

A

warm

42
Q

what is overall survival like for CLL

A

long overall survival, its normally a slow proliferation. 1/3 static, 1/3 slow progressing and 1/3 rapidly progressing

43
Q

if anyone presents to the GP with a suspected leukaemia, what should happen next

A

they should have an FBC within 48 hours

44
Q

complications of leukaemia

A

hyperviscosity syndromes, secondary cancers and infection

45
Q

key investigations for a leukaemia

A

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
Q

what bacteria is most likely to cause neut sepsis from an infection on an indwelling line

A

staphylococcus epidermis (gram positive, coagulase negative)

47
Q

if not getting better on tazocin for neutropenic sepsis, what do you add

A

vancomycin

48
Q

if no improvement on abx after 4-6 days with neutropenic sepsis, what would you then consider

A

fungal infection

49
Q

side effect of GCSF

A

bone pain and nausea

50
Q

apart from chemo, what are some other causes of neutropenia

A

aplastic anaemia and hypersplenism

51
Q

how many of the bodies platelets are held in the spleen

A

90%

52
Q

what are the causes of hyposplenism

A

coeliac disease, graves, SCD, splenectomy

53
Q

potential indications for a splenectomy

A

ITP, hereditary spherocytosis, trauma

54
Q

what is mild splenomegaly classed as

A

spleen palpable 1-3cm below the costal margin

55
Q

what is moderate splenomegaly classed as

A

between umbilicus and midline

56
Q

what is massive splenomegaly classed as

A

crosses the midline and umbilicus

57
Q

what are some causes for massive splenomegaly

A

visceral leishmaniasis and CML

58
Q

what are some causes for a mild splenomegaly

A

haemolytic anaemias, EBV, portal hypertension

59
Q

risk factors for TLS (apart from type of cancer)

A

pre existing renal impairment, dehydration, age

60
Q

electrolyte abnormalities in TLS

A

hyperphosphataemia, hyperuricaemia, hyperkalaemia and hypocalcaemia (due to the excess P binding with it)

61
Q

how often do bloods need to be done in TLS and what are they

A

6 HOURLY
U+E, bone profile, uric acid, LDH (note a high LDH is a RF for TLS)

62
Q

complications of TLS

A

AKI (due to uric acid precipitates), arrhythmias, seizures

63
Q

how does TLS present

A

cramps, N+V, syncope, reduced urine output

64
Q

Mx of high risk TLS

A

rasburicase

65
Q

Mx of lower risk TLS

A

allopurinol

66
Q

definition of myeloma

A

clonal proliferation of abnormal B lymphocytes which then secrete non functioning immunoglobulins (known as paraproteins)

67
Q

CRABBI mneumonic for myeloma

A

Calcium - high due to increased osteoclast activity
Renal - SFLC deposition –> damage
Anaemia
Bleeding
Bone pain (lytic lesions)
Infection

68
Q

people over 60, who present with persistent bone pain or an explained fracture should have what tested

A

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

69
Q

investigations for myeloma

A

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 Hones
serum immunofixation - looks for the type of paraprotein

Bone marrow biopsy

whole body MRI

70
Q

for a diagnosis of myeloma, what should the Level of paraprotein be

A

> 30

71
Q

what criteria needs to be met for myeloma to be diagnosed

A

1) clonal plasma cells >10% on bone marrow biopsy
2) paraprotein
3) evidence of end organ damage

72
Q

options for drug Tx for myeloma

A

targeted drugs, steroids, chemo

73
Q

complications of myeloma

A

hyperviscosity syndromes, pain, pathological fractures, infection, fatigue

74
Q

what is MGUS

A

paraprotein found but <30g/L and no sign of end organ damage

75
Q

what is the chance MGUS will progress to myeloma

A

1-2%

76
Q

Rf of myeloma

A

old, fat, black, man

77
Q

what is amyloidosis

A

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
Q

what are the normal ways in which amyloidosis presents

A

renal disease, cardiac disease and neurological disease

complications –>ESRF, heart failure and Alzheimers

79
Q

definitive diagnosis of amyloidosis

A

biopsy from affected organ and stain with Congo red (see apple green birefringence)

80
Q

Mx of amyloidosis

A

antimyeloma therapy

81
Q

what paraprotein spike do you get in waldenstroms macroglobulinaemia

A

IgM (this is a rare condition which can affect older men. Presents with systemic upset and hyperviscosity syndromes)

82
Q

what is the course like for ITP

A

insidious onset and has a relapsing and remitting course

83
Q

Investigations for ITP

A

diagnosis of exclusion, FBC and bloods film should show isolated thrombocytopenia

84
Q

Mx of ITP

A

supportive but may need steroids if platelet count <30. Give IVIG + platelets if actively bleeding / life threatening.

yr 5 haem (5 decks)

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