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HAEM ONC Flashcards

1
Q

CML treatment= imatinib– MOA

A

imatinib MOA= tyrosine kinase inhibitor

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2
Q

CML- chromosome problem

A

translocation chr 9 and 22– philadelphia chromosome

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3
Q

TRALI vs TACO

A

TRALI– causes hypotension and overload signs and RAPID

TACO- slower, hypertension, overload

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4
Q

heparin affects which part of coagulation?

A

antithrombin

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5
Q

acute chest syndrome == which ocndition?

A

sickle cell crisis

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6
Q

most common type oh hodgkins lymphona

A

nodular sclerosing

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7
Q

monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells

A

CLL

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8
Q

combined B and T cell disorders ? (4)

A

WASH your b cells and t cells
Wisckott
Ataxic telangiectasia
severe combined immunodeficiency
Hyper IgM

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9
Q

neutrophil disorders (3)

A

Chronic granulomatous disease
Chediak-Higashi syndrome
Leukocyte adhesion deficiency

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10
Q

B cell only disorders (3)

A

Brutons X linked
(selective) IgA deficiency
Common variable immunodeficiency

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11
Q

T cell only disorder (1)

A

digeorge

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12
Q

draw toxicity bear

A

see photo

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13
Q

hereditary angioedma- inheritance and best screening test

A

autosomal dominant
C4 screening in between attacks

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14
Q

Basophilic stippling typical of?

A

lead poisoning

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15
Q

antiphospholipid syndrome management

A

LMWH and aspirin

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16
Q

most common type of inherited thrombp[hilia

A

factor 5 leiden heterozygous= activated factor C resistance (c for COMMON)

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17
Q

what is ITP
what antibodies against

Ix

A

reduction in the platelet count.

Antibodiesagainst the glycoprotein IIb-IIIa or Ib complex

Ix :
IgG antiplatelet antibodies
bone marrow asp- megakaryocytes

Mx:
PO prednisolone
plasma exchange IvIG
splenectomy if does not respond to steroids after3 motnhs

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18
Q

what is most likely to precipitate haemolysis in a patient with G6PD deficiency?

A

penicillin

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19
Q

Raynaud’s
type of cryoglobinuaemia

A

Type 1 cryoglobinuamiea

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20
Q

3 types of cryoglobinaemia and conditions associated with each type

A

type 1 - monoclonal- IgG, IgM
raynayds, multiple myeloma, waldenstrom
type 2 - mixed mono and polyclonal rheumatoid factor: hep c, RA, sjogrens
type 3 : polyclonal, RA,Sjogrens

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21
Q

Mx for cyoglobinaemia and tx

A

Low complement levels
high ESR

Tx:
plasma phoresis
immunosuppression

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22
Q

methaemoglobinaemia affects o2 and co2?

A

ABG:
high o2
low co2

O2 sats LOW, HIGH pao2

symptoms: headache
SOB cyanosis

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23
Q

medication (ABx) most likely to cause methaemoglobinaemia

A

co-trimoxazole

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24
Q

what is methamoglobinaemia

how does it affect o2 dissociation curve

A

Hb oxidsied to Fe2+ to Fe3+

shifts curve to the LEFT

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25
Q

causes of methaemoglobinaemia

A

MNEUMONIC CAUSES methaemoglobinaemia:

Sulphur, Nitrates and Poppers means Dappy’s Prime Pussy Dyes

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26
Q

treatment Methaemoglobinaemia

A

NADH methaemoglobinaemia reductase deficiency: ascorbic acid
IV methylthioninium chloride (methylene blue) if acquired

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27
Q

what type of infection associated with hereditary spherocytosis causes aplastic crisis

A

parvovirus B19 infection

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28
Q

what is seen on blood film for G6PD?
inheritance pattern?
Diagnostic test?

A

heinz bodies on blood film (6yo like heinz)

X LINKED recessive
Diagnostic test= Measure enzyme activity of G6PD

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29
Q

hereditary spherocytosis
Diagnostic test
inheritance
what is seen on bloodfilm

A

diagnostic test= EMA binding
aut dominant
blood film- spherocytes

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30
Q

adverse effects of aromatase inhibitors eg letrozole

and MOA

A

MOA= reduce production of oestrogen in peripheral tissues

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31
Q

features of TTP : ask the FAT RN

A

Fever
Anaemia
Thrombocytopaenia

Renal impairment
Neuro involvement

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32
Q

Tx TTP

A

Plasma exchagne
steroids
imunosuppressants
vincristine

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33
Q

alpha thalassemia affects ‘? chr

A

chromosome 16 (16 sort of looks like alpha)

if only 1 or 2 alpha chains affected- hypochromic and microcytic

IF severe= all 4 chains are affected =death in utero hydrops fetalis

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34
Q

how to Differentiate CML from leukaemoid reactions:

A

CML: leukocyte alkaline phosphatase score is low

Leukaemoid reaction; alk phos score is HIGH
high leucocyte alkaline phosphatase score
toxic granulation (Dohle bodies) in the white cells
‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus

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35
Q

causes of leakaemoid reaction

A

severe infection
metastatic Ca with BM infiltration
severe haemolysis
massive haemorrhage

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36
Q

most common inherited bleeding disorder– inheritance pattern

A

von willebrands

AUT DOM

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37
Q

Treatment von willebrand disease

A

factor 8 concentrate
tranexamic acid for mild bleeding
desmopressin_ raises WVB factor

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38
Q

sickle cell

cause
- inheritance
- diagnosis

A

Inheritance= autosomal recessive
diagnosis- electrophoresis

valine substituted at position 6 of beta globin chain instead of glutamic acid

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39
Q

Tranplant vs graft disease: time frame
CFs
skin biopsy will show?
Tx?

A

2-6 weeks after transfusion
CFs diarrhoea, rash, liver damage
-skin biopsy will show abundant necrotic keratinocytes
There is no treatment, so prevention by using gamma-irradiated blood products in high-risk patients is the key.

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40
Q

tumour marker CA 125

A

ovarian

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41
Q

CA 19-9 tumour marker

A

pancreatic

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42
Q

CA 15-3 tumour marker

A

breast cancer

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43
Q

Alpha-feto protein (AFP) tumour marker

A

hepatocellular
teratoma

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44
Q

CEA

A

colorectal

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45
Q

S-100 tumour marker

A

S-100 Melanoma, schwannomas

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46
Q

Bombesin tumour marker

A

SCLC, gastric , neuroblastoma

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47
Q

what type of leukaemia associated with polycythaemia rubr vera

A

AML

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48
Q

most common inherited BLEEDING disorder

A

VWB disease

most common inherited thrombophilia= anti protein c resistance

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49
Q

follicular lymphona translocation?

A

14:18

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50
Q

c-myc gene translocation

A

Burkitts lymphoma

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51
Q

Burkitts lymphoma transloccation

A

YMCA= YMC8
8 for translocation t(8:14)
myc has the same letters (c myc gene translocation)

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52
Q

TLS management

A

HIGH VOL IV fluids
prophylactic allopurinol and also rasburicase can be used
Tx- rasburicase

DO NOT give rasburicase and allopurinol together as this red effect of rasburicase

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53
Q

hereditary spherocytosis abdo pain cause

A

likely gallstones

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54
Q

what is likely to develop from polycythaemia rubra vera

A

myelofibrosis
AML

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55
Q

acute intermittent porphyria vs porphyria cutaena tarda differences

A

porphyria cutaena tarda = photosensitive bullae

acute intermittent porphyria= presents with psych, neuro and abdominal symptoms

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56
Q

acute intermittent porphyria
CFs

A

CFs
abdominal: abdominal pain, vomiting
neurological: motor neuropathy
psychiatric: e.g. depression
hypertension and tachycardia common

classically urine turns deep red on standing
raised urinary porphobilinogen

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57
Q

Paroxysmal nocturnal haemoglobinuria
CFs

Ix

Mx

A

CFs
haemolytic anaemia
RBCs , WBCs, platelets or stem cells may be affected== pancytopenia
haemoglobinuria: classically dark-coloured urine in the morning
thrombosis e.g. Budd-Chiari syndrome
aplastic anaemia may develop

Ix:
GOLD Flow cytometry of blood - CD59, CD55
hams test = acid induced haemolysis

Mx_ blood product replacement
anticoagulation
monoclonal antibody eculizumab
stem cell transplant

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58
Q

low levels of CD59 and CD55 == ?

A

Paroxysmal nocturnal haemoglobinuria

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59
Q

most common cause of antithrombin III def?

A

CKD

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60
Q

ADAMTS13 ass’d with which type of vasculitis

A

TTP

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61
Q

when should skin prick testing be read
when shoud skin patch testing be read

A

skin prick- 15mins
skin patch- 48hrs

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62
Q

which type of chemo causes hypomagesia

A

cisplatin

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63
Q

meigs syndrome =

A

ovarian fibroma

64
Q

Irinotecan MOA

A

Irinotecan inhibits topoisomerase I,

65
Q

what is Multiple myeloma
what type of cells

and CFs

Investigations

A

MM = haem malignancy
clonal proliferation of plasma cells

CFs
CRABBI
Ca raised (osteoclast reabsorp)
Renal (light chain dep)
Anaemia
Bleeding (bruises)
Bone (lytic lesions)
Infection

Investigations:
XR -skull XR = raindrop lytic lesions
Urine- monoclonal band BENCE JONES protein IgA IgG

66
Q

Diagnostic criteria Multiple Myeloma

A

either : one major criteria OR 3 minor criteria

Major criteria
Plasmacytoma (as demonstrated on evaluation of biopsy specimen)
30% plasma cells in a bone marrow sample
Elevated levels of M protein in the blood or urine

Minor criteria
10% to 30% plasma cells in a bone marrow sample.
Minor elevations in the level of M protein in the blood or urine.
Osteolytic lesions (as demonstrated on imaging studies).
Low levels of antibodies (not produced by the cancer cells) in the blood.

67
Q

sickle cell crisis- ANAEMIA crises
2 types and how to differetiate them

A

anaemia crisis in sickle cell- 1. sequestration and 2. aplastic

Aplastic = sudden fall in Hb, low reticulocyte count , PARVOVIRUS infection

sequestration =Low Hb, increased reticulocyte count

68
Q

common childhood haem malignancy

A

ALL

69
Q

C1-INH deficiency= what condition?

A

hereditary angioedema

70
Q

bronchial carcinoma and mesothelioma associated with exposure to WHAT

A

asbestos

71
Q

what is mesna and when to give it

A

mesna prevents bladder irritation after cyclophosphamide chemo treatment

72
Q

reed sternberg cell associated with?

A

hodgkins lymphoma

73
Q

only which electrolyte is lowered in tumour lysis syndrome? all rest elevated

A

calcium is lowered

74
Q

DOCETAXEL AND TAXANE MOA

A

PREVENTS microtubule depolymerisation. Creates free tubulin

75
Q

sickle cell patient which medication for long term use to prevent sickle crises?

A

hydroxycarbamid

76
Q

Bombesin tumour marker for?

A

SCLC, neuroblastoma, gastric ca

77
Q

systemic mastocytosis investigation-

A

urinary histamine

78
Q

CLL : is chr 17 or 13 deletion associated with GOOD prognosis

A

chr 13 deletion is good prognosis

79
Q

CLL poor prognostic factors

A

male sex
age > 70 years
lymphocyte count > 50
prolymphocytes comprising more than 10% of blood lymphocytes
lymphocyte doubling time < 12 months
raised LDH
CD38 expression positive
TP53 mutation

80
Q
A
81
Q

CLL : is chr 17 or 13 deletion associated with GOOD prognosis

A

chr 13 deletion is good prognosis

82
Q

acute intermittent porphyria
CFs
inheritance
Ix
Tx

A

AUT DOM
CFs
Neuro=motor neuropathy
Psych- depression
abdo pain, n+v
HTN, tachycardia

Ix= urine turns dark red on standing oxidation
URINARY POROBILLOGEN RAISED

Tx=
IV haemtin/arinate
or IV glucose

83
Q

Tx CML

A

imatinib is now considered first-line treatment
inhibitor of the tyrosine kinase associated with the BCR-ABL defect
very high response rate in chronic phase CML
hydroxyurea
interferon-alpha
allogenic bone marrow transplant

84
Q

most common bac cause of neutropenic sepsis

A

Staph epidermis
coagulase-negative, Gram-positive bacteria

85
Q

conditions thymomas associated with (4)

A

myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH

86
Q

how does desmopressin affect ppl with VWB disease

A

desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells

87
Q

DIC =? caused by
CFs, Ix, blood results

A

Causes of DIC
sepsis
trauma
obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome)
malignancy

Ix bloods:
low platelets
incr PT, incr APTT
high d dimer
low fibrinogen
BLOOD FILM=SCHISTOCYTES (helmet cells)

88
Q

Lead poisoning
CFs
Ix
Tx

A

CFs
abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue. constipation
blue lines on gum margin

Ix: lead blood level
FBC= microcytic anaemia, basophilic stippling
increased urinary coproporphyrin

Mx:
chelation
dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol

89
Q

how to treat severe b12 def , with NO folate deficiency

A

if no folate deficiency-
IM 1mg hydroxycobalamin 3x a week for 2 weeks and then once every 3 months

If folate deficient- treat b12 deficiency first

90
Q

what to give pt with Von willebrand deficiency before dental procedure

A

desmopressin

91
Q

when is leukocyte ALP raised :

A

leukamoid reactions
myleofibrosis
polycythaemia vera rubra
infections
steroids, cushings
pregnancy
COcp

92
Q

when is leukocyte ALP low

A

CML
pernicious anaemia
paroxysmal nocturnal haemoglobinuria
infec mono

93
Q

causes of extravascular haemolysis

A

Wa Ha Ha Ha

W warm autoimmune hemolytic anemia
H hemolytic disease of the newborn
H hereditary spherocytosis
H hemoglobinopathies: sickle cell, thalassemi

94
Q

IgM paraproteinaemia?>?????

Tx of this condition

A

waldenstroms

Tx= rituximab

95
Q

what actually causes methaeglobinaemia?

A

OIL RIG

ferrous ions (Fe2+) of haemoglobin are oxidised to the ferric (Fe3+) ions

96
Q

what abx precipitates G6DP haemolysis

A

primaquine
ciproflocaxin
sulphonamides
suphonylureas
sulphasalazine

97
Q

hyposplenism findings on blood film

A

Howell-Jolly bodies and siderocytes are typical blood film findings of hyposplenism

98
Q

smudge cells=?

A

CLL- need to do immunophenotyping

99
Q

transfusion of WHAT type of blood product = most likely bacterial infection

A

platelets

100
Q

tear drop poikiolocytes= ?

A

myelofibrosis

101
Q

beta thalassemia trait- inheritance

and major

A

beta thalassemia trait= aut rec
HbA2 raised
Usually asymptomatic apart from : mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia

Beta thalassemia major= chr 11
Presents in 1st year of life with severe anaemia, hepatosplenomegaly and failure to thrive
Extra-medullary haematopoiesis 🡪 facial abnormality
Blood film 🡪 hypochromic, microcytic cells. Also target cells and nucleated RBCs
Mx 🡪 lifelong transfusions

102
Q

good prognostic markers ALL

and poor prgnostic

A

good prognostic markers:
FAB 1 type
caucasian
initial low WBC
common ALL
pre B phenotype
del 9p

Poor prognostic factors
FAB L3 type
T or B cell surface markers
Philadelphia translocation, t(9;22)
age < 2 years or > 10 years
male sex
CNS involvement
high initial WBC (e.g. > 100 * 109/l)
non-Caucasian

103
Q

poor prognostic features AML

A

> 60 yo
20% blasts after first chemo
deletion chr 5 or 7

104
Q

CML chr affected
first line tx

A

philadelphia- 9:22
imatinib- tyrosine kinase inhibitor
BCR-ABL

105
Q

smudge cells seen in ?

A

CLL

106
Q

In ER positive breast cancer- when to use tamoxifen vs letrozole/anastrazole

A

Tamoxifen= ER positive PRE menopausal women
SE’s incr risk VTE and endometrial Ca

Aromatase inhibitors= ER positive POST menopausal women

107
Q

staging system for lymphoma

A

Ann Arbor system.
Stage 1 - One node affected
Stage 2 - More than one node affected on the same side of the diaphragm
Stage 3 - Nodes affected on both sides of the diaphragm
Stage 4 - Extra-nodal involvement e.g. Spleen, bone marrow or CNS

108
Q

poor prognosis lymphoma

A

age > 45 years
stage IV disease
haemoglobin < 10.5 g/dl
lymphocyte count < 600/µl or < 8%
male
albumin < 40 g/l
white blood count > 15,000/µl

109
Q

treatment of aplastic anaemia

A

stem cell transplant- allogenic
anti thymocyte
anti lymphocyte
platelet tramsfusion

110
Q

Leucocyte alkaline phosphatase
raised and lowered in:

A

Raised in
myelofibrosis
leukaemoid reactions
polycythaemia rubra vera
infections
steroids, Cushing’s syndrome
pregnancy, oral contraceptive pill

Low in
chronic myeloid leukaemia
pernicious anaemia
paroxysmal nocturnal haemoglobinuria
infectious mononucleosis

111
Q

howel jolly bodies on blood film

A

hyposplenism

112
Q

TTS deficiency

A

ADAMTS13

113
Q

haemophilia INHERITANCE

2 types and their causes
CFs
Ix

A

x linked recessive

haemophilia A= factor 8 def (a sounds like 8)

haemophilia B = factor 9 def

CFs
BOYS/MEN
prolonged bleeding
haemoarthrosis
haematomas

Ix increased APTT but everything else normal

114
Q

why pt with CLL has repeated pneumonia

A

Hypogammaglobulinaemia

115
Q

acute intermittent porphyria investigation

A

urinary porphobilinogen

116
Q

how to tell if warm or cold haemolytic anaemia

A

Manchester is cold and in the UK. Hence, IgM(anchester) associated cold AIHA, and leads to intravascular haemolysis (Manchester in the UK)

Ghana is hot and outside the UK. Hence cause IgG(hana) associated warm AIHA, and leads to extravascular haemolysis (Ghana outside the UK)

SLE ass’d with warm

117
Q

waldenstrom’s macroglobulinaemia

A

Waldenstrom’s macroglobulinaemia: lymphoplasmacytic lymphoma
associated with an IgM monoclonal (paraprotein) protein in the serum. bone marrow based disease

Features
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
the pentameric configuration of IgM increases serum viscosity
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s

Investigations
monoclonal IgM paraproteinaemia
bone marrow biopsy is diagnostic
infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

Management
typically rituximab-based combination chemotherapy

118
Q

monoclonal IgM paraproteinaemia
bone marrow biopsy is diagnostic of ???

A

waldenstroms macroglobulinaemia

119
Q

essential thrombocytosis: gene mutation

what is it

A

JAK2 mutation

Essential thrombocytosis is one of the myeloproliferative disorders: CML, polycythaemia rubra vera and myelofibrosis. Megakaryocyte proliferation results in an overproduction of platelets.

Features
platelet count > 600 * 109/l
both thrombosis (venous or arterial) and haemorrhage can be seen
a characteristic symptom is a burning sensation in the hands
a JAK2 mutation is found in around 50% of patients

Management
hydroxyurea (hydroxycarbamide) to reduce the platelet count

interferon-α is also used in younger patients

low-dose aspirin may be used to reduce the thrombotic risk

120
Q

what gene mutation do all myeloproliferative disorders have

A

JAK2 mutation

121
Q

hydroxyurea MOA=

A

reduce platelemt count
antimetabolite- type of chemo

used in essential thrombocytosis

122
Q

topoisomerase I chemo given- what else is also given at the same time

A

Irinotecan

inhibits topoisomerase I which prevents relaxation of supercoiled DNA

123
Q

skin necrosis following the commencement of warfarin:
CAUSED BY?

A

Protein c deficiency
develops necrotic skin lesions on his lower limbs and forearms.

124
Q

how are haptoglobin levels affected by INTRAVASCULAR haemolytic anaemias

A

LOW HAPTOGLOBINS

125
Q

intravascular haemolytic anaemias

A

G6PD def
Red cell fragments - TTP, DIC, HUS, heart valves
Paroxysmal nocturnal haemoglobinuria
Cold haemolytic anaemia (IgM , manchester is cold and IN THE UK)

CAUSES LOW HAPTOGLOBIN

126
Q

extravascular haemolytic anaemias

A

Wa
Ha
He
Ha

Warm haemolytic anaemia (igG, Ghana is OUTSIDE of the UK and is WARM)
Haemoglobinopathies - Sickle cell, thalassemia
Hereditary spherocytosis
Haemolytic disease of newborn

127
Q

ring sideroblast on blood film =?

A

pyridoxine b6 deficiency
can cause sideroblastic anaemia

128
Q

resistance to heparin cause of thrombophilia

A

antithrombin 3 def

129
Q

major criteria determining the use of cryoprecipitate in bleeding

A

low fibrinogen level

130
Q

t(15;17) translocation and DIC

A

Acute promyelocytic leukaemia

131
Q

essential thrombocytosis tx

A

hydroxycarbamide

132
Q

arepipratant moa

A

neurokinin blocker

133
Q

b12 and folate def= which to treat first

A

treat b12 injection first
and then replace folate
to avoid subacute degn sc

134
Q

Features may include symptoms of Raynaud’s and acrocynaosis.

=?

A

Remember this for cold AIHA

135
Q

Investigation cold acute haemolytic anaemia

A

Direct antiglobulin test

136
Q

what virus causes Adult T-cell leukaemia/lymphoma

A

HTLV-1:

137
Q

what virus causes High-grade B-cell lymphoma

A

HIV

138
Q

what virus causes Hodgkin’s and Burkitt’s lymphoma, nasopharyngeal carcinoma

A

EBV
malaria can also cause burkitts lymphoma

139
Q

Benign ethnic neutropaenia is common in ??

A

blakc ppl

140
Q

heparin MOA

A

Activates antithrombin III.

141
Q

pernicious anaemia Ix

A

intrinsic factor testing
folate b12

142
Q

bicalutamide MOA

A

androgen receptor blocker

A==> Abiraterone==> Synthesis blocker
B==>Bicalutamide==>Receptor blocker
C==>Cyproterone==> Receptor blocker

143
Q

what is amyloidosis and the 2 types

A

Positive congo red staining
AA amyloidosis = renal involvement, inflammatory- RA, SLE

AL amyloidosis= more common
MM, WM, MGUS

144
Q

what would give a false negative TTG when investigating coeliac diagnosis

A

selective IgA deficiency- so it is important to also check endomysial IgA with TTG when testing

145
Q

DIC transfuse what product

A

Fresh frozen plasma
FFP (

low fibrinogen is an indicator for this

146
Q

Delayed haemolytic blood transfusion reaction

A

positive direct antiglobulin test

147
Q

CFs tumour lysis syndrome

A

anuric/ oligouric
rosette crystals in urine

148
Q

nivolumab MOA

A

PD1 inhibitor

149
Q

causes of warm haemolytic anaemia

A

IgG , extravascular

idiopathic
SLE
CLL, lymphoma
methyldopa

150
Q

causes of cold haemolytic anaemia

A

IgM, intravascular

infections = more likely to give you a cold

EBV, mycoplasma infections

151
Q

low transferrin sats but high ferritin causes= ?

A

alcohol XS,
inflammation, liver disease
CKD,
malignancy

152
Q

what type of drugs can be given in pts who are not tolerant of chemo and stem cell transplant?

A

thalidomide
bortezomib = proteasome inhibitor

153
Q

factor 5 leiden resistance = describe how factor 5 and others are affected

A

factor 5 disease= slow inactivation of factor v by activated protein C

154
Q

GOSRELIN MOA

A

gnrh agonist,, LHRH analogue

155
Q

what to give to von willebrand pt going for tooth extraction

A

give desmopressin
avoid factor 8 conc bc risk of transfusion infection

M R C P (18 decks)

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