MRCP Haem Flashcards
1
Q
Triad:
Haemolytic anaemia
pancytopenia
Large vessel thrombosis
- Dark urine
- microscopic haematuria
- no red cell in microscope
A
PNH
paroxysmal nocturnal haemoglobinuria
intravascular haemolysis
associated with VTE
Mx: eculizumab monoclonal antibody inhibit complement
2
Q
Ferritin
A
Normal- not iron deficient
In inflammation may mask IDA
3
Q
Beta thalassaemia
A
trait
- microcytic anaemia, raised HbA2
major
- no beta globulin, chr 11, microcytic anaemia, raised HbA2 and HbF, absent HbA
4
Q
NHL
A
painless LN
B symptoms
cytopenia
extranodal disease
raised LDH in proliferative
4
Q
Most common extranodal lymphoma
A
upper GI tract
5
Q
Haematological cause of nephrogenic DI
A
sickle cell
6
Q
PNH gene defect in…
A
PIG A gene
7
Q
Von willebrand disease types
A
Types
type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)
8
Q
Management of von willebrand disease
A
Management
- tranexamic acid for mild bleeding
- desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
- factor VIII concentrate
9
Q
Heparin-induced thrombocytopaenia (HIT)
A
bind to the PF4-heparin complexes on the platelet surface and induce platelet activation
10
Q
Anaemia and jaundice
A
HAEMOLYTIC ANAEMIA
11
Q
Raised reticulocytes mean…
A
intravascular haemolysis
12
Q
When is osmotic fragility high?
A
hereditary spherocytosis
13
Q
Howel jolly bodies indicate…
A
hyposplenism
14
Q
Crohns patient with macrocytic anaemia
A
terminal ileal disease
B12 and folate deficiency
without b12, RBC cannot mature -> haemolyse
15
Q
When to do haemoglobin electrophoresis
A
non white patient with microcytic anaemia
16
Q
Aplastic anaemia
A
pancytopenia
hypoplastic bone marrow
Mx: haematopoietic stem cell transplant
17
Q
Lead poisoning associated with…
A
normocytic anaemia with sideroblastic change
accumulation of **iron around nuclei **of developing RBC
18
Q
Invasive aspergillosis test
A
galactomannan test via ELISA
19
Q
vitamin K dependent clotting factors
A
1972
2, 7, 10, 9
20
Q
Poor prognosis for AML
A
AGE > 60 years
more than > 20% blasts after first course of chemo
cytogenetics: deletions of chromosome 5 or 7
21
Q
Acute promyelocytic leukaemia M3
A
associated with t(15;17)
fusion of PML and RAR-alpha genes
presents younger than other types of AML (average = 25 years old)
Auer rods (seen with myeloperoxidase stain)
DIC or thrombocytopenia often at presentation
good prognosis
22
Q
CML
A
BCR/ABL fusion gene (philadephia)
9:22
tyrosine kinase production
mx: imatinib (tyrosine kinase inhibitor)
23
Q
Acute transfusion reaction
A
- non haemolytic febrile reaction
- paracetamol - minor allergic reaction
- antihistamine - anaphylaxis
- stop transfusion IM adrenalione - Acute haemolytic reaction (ABOincompatible)
- direct coombs test, stop transfusion - TACO
- stop and give diuretics - TRALI
- stop transfusion
- anti HLA antibodies donor
24
Acute haemolytic reaction (ABOincompatible) presents as...
tachycardia
back pain
25
Which immune cell does CLL affect
B cell disorder
blood film: smudge cells (also known as smear cells)
26
Thrombocytopenia with VTE risk and miscarriages
Antiphospholipid syndrome
27
Polycythaemia
primary:
- JAK 2+ve
- polycythaemia vera
- pruritis and splenomegaly
secondary:
- JAK -ve
- hypoxia ---> EPO
- COPD/fibrosis/altitude
28
Haemophilia A and B inherited pattern
X linked recessive
BOYS get it
women can be carriers
28
29
Ann arbor staging for Hodgkins
I: single lymph node
II: 2 or more lymph nodes/regions on the same side of the diaphragm
III: nodes on both sides of the diaphragm
IV: spread beyond lymph nodes
Type:
A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)
30
31
Hodgkin 2 types
nodular lymphocyte predominant
- good prognosis
classical hodgkins lymphoma
- nodular sclerosis
- mixed cellularity
- lymphocyte rich
- lymphocyte depletes
32
Coagulation factor deficiency...
prolonged PT and APTT
33
Causes of cold agglutinin
| autoantibodies react at <37 degrees
Lymphoproliferative
- non hodgkins
infections
- mycoplasma
- EBV
34
What vitamin aids iron absorption
vitamin C
35
HRT risk proportion
DVT: 2-3 times risk
Breast ca: 1 in 1000/yr
35
CLL investigation
**blood film: smudge cells **(also known as smear cells)
**immunophenotyping** is the key investigation
most cases can be identified using a panel of antibodies specific for CD5, CD19, CD20 and CD23
36
Another name for immunophenotyping
Flow cytometry
37
Antiphospholipid syndrome management of clots
lifelong with target INR 2-3
38
Diagnostic criteria for antiphospholipid syndrom
antibody should be rechecked after 3 months to ensure persistence
39
Anaemia causes...
retinopathy including haemorrhage
40
Haemolytic anaemia due to primaquine
G6PD deficiency
heinz bodies (denatures haemaglobin)
41
Side effects of vincristine
peripheral neuropathy
42
DIC
prolonged clotting time
low fibrinogen
43
Sickle cell anaemia
howell jolly body (hyposplenism)
frontal bossing of skull
44
Spleen size difference between sickle cell and thalassaemia
sickle cell- hyposplenism from infarct
thalassaemia- megaly due to haematopoiesis
45
Secondary polycythaemia due to hypoxia...
increased affinity haemoglobin
46
What is reactive thrombocytosis
infection/inflammation/surgery etc causing increased plt
47
Complication of multiple blood transfusion
iron overload --> pericarditis
Mx: iron chelator desferrioxamine
48
Italian women with Hypochromic microcytic anaemia
nucleated RBC
Thalassaemia
49
Pancytopenia
Bone marrow disorder
50
Elderly
lymphocytosis
splenomegaly
pancytopenia
CLL
51
Diarrhoea
abdo pain
AKI
rash in lower limbs
HUS
52
ITP
isolated thrombocytopenia
brusing and bleeding
53
Myeloproliferative - myelofibrosis
fibroblast overstimulation
splenomegaly
high WCC and plt
tear drop poikilcytes blood film
dry tap on marrow biopsy
54
Isolated prolonged APTT
antiphospholipid syndrome
55
Idiopathic hypereosinophilic syndrome
steroids to treat
56
AML chromosome associated with///
5 or 7
57
Myeloproliferative disorder may transform into...
AML
58
Irradiated blood is for...
| inactivated donor lymphocyte
CLL on purine analogue are T cell depleted hence risk of graft vs host
59
GVHD ....
Allogenic bone marrow transplant
**T cell in donor** mount immune on host
NOT a transplant rejection
Acute - within 100 days (skin, liver, GI)
Chronic- after 100 days (+lung, eyes)
60
Managmenent of GVHD
Steroids
61
reversal of alteplase (tPA)
FFP
62
Beta thalassaemia common in which ethnic groups
Mediterranean
Middle east
Central and south east asia
63
Auer rods seen in...
AML
64
(Richter's transformation)
CLL transformation to high-grade lymphoma
65
(Richter's transformation)
lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain
66
Most common inherited prothrombotic disorder
Factor V Laden
67
Cytotoxic Purine analogue abx prophylaxis
| affects t cell function
co-trimoxazole for PCP
68
Capecitabine vs 5-FU
5-FU -fluiiidd (intravenous)
capecitabine (prodrug of 5FU)
69
Common side effect of imatinib
nausea
70
Tumour lysis syndrome prevention
- IV fluids
- higher risk s- allopurinol or rasburicase
- rasburicase- high risk group
a recombinant version of urate oxidase
- allopurinol- lower-risk groups
- rasburicase and allopurinol should not be given together
71
G6PD hallmarks
red blood cell enzyme defect Mediterranean and Africa
inherited in an X-linked recessive drugs can precipitate a crisis as well as infections and broad (fava) beans
reduced NADPH
72
Haemolytic anaemia
reduced haptoglobulin
raised reticulocytes
73
Waldenstrom's macroglobulinaemia
Low grade lymphoma
monoclonal IgM (Walden and M) paraprotein
hyperviscosity syndrome
cryoglobulinaemia e.g. Raynaud's`
| W upside down M
74
Koilonychia
IDA
75
CD20 targetted therapy for..
lymphoma (B cell)
Rheumatoid arthritis
76
Anicytosis
| variation in RBC size
macrocytic anaemia due to B12 deficiency
77
Gallstones and anaemia
Hereditary spherocytosis
77
Which medication should be checked with Haem for VWD?
NSAID (affects plt aggregation)
78
79
Most common sx of myelofibrosis
fatigue
80
Burkitts lymphoma
c-Myc
high grade NHL
81
T(11;14)
Mantle cell lymphoma
82
t(14;18)
follicular lymphoma
83
Aplastic crisis
parvovirus
joint pain
fever
facial rash
84
ITP antibody
Antibodies against the glycoprotein IIb/IIIa or Ib-V-IX complex.
85
Evan's syndrome
ITP + autoimmune haemolytic anaemia (AIHA)
| Mx: steroid
86
Management of ITP
Prednisolone
IVIG
Splenectomy
87
ALL poor prognosis
age < 2 years or > 10 years
WBC > 20 at diagnosis
T or B cell surface markers
non-Caucasian
male sex
presence of philadelphia Chr
88
89
Fat embolism syndrome
recent traumatic fracture
petechiae in upper body and axillae
90
Hodgkins
supraclavicular
mediastinal
young patient
91
Prolonged PT
Lack of vit K dependent clotting factors
1972
92
False positive VDRL
Antiphospholipid syndrome
93
VWD
autosomal dominant
post op bleed
nose bleed
menorrhagia
94
Most common inherited clotting disorder
F V Laden
95
Most common inherited bleeding disorder
VWD
96
Most common cause of death in CLL
Infection
97
LMWH action
potentiates antithrombin 3
98
Major complication of polycythaemia vera
bleeding
thrombosis
99
Platelet target
more than 20 if fever
more than 10 if no fever
100
Bite cells on blood film
G6PD
101
rbc life span
120 days
| 17 days in sickle
102
TTP pentad
FATRN
fever
anaemia
thrombocytopenia
renal
neurological symptom
102
mycosis fungoides
T-cell lymphoma that affects the skin.
103
Fragment cells on blood film
TTP
104
Hairy cell leukaemia
BRAF mutation
105
Sideroblastic anaemia
genetic
increased iron stores in RBC (mitochondria)
- ringed sideroblasts around the nuclei
106
107
Myelodysplastic syndrome
older population
can lead to AML
unexplained cytopenia and macrocytosis
Ix: bone marrow biopsy + cytogenetic
108
Haemophilia A and B
A- 8
B- 9
109
haemophilia A complication
factor 8 inhibitor can happen in multiple factor 9 infusions
110
Methaemaglobinaemia
drugs: sulphonamides, nitrates (including recreational nitrates e.g. amyl nitrite 'poppers'), dapsone, sodium nitroprusside, primaquine
chemicals: aniline dyes
| methylene blue
111
112
Cabot rings
lead poisoning
113
Warfarin reduced effect by...
azathioprine
COCP
carbamazepine
114
Poor prognosis of hodgkins
LYMPHOCYTE DEPLETED
115
Post transplant complications
most common:
- skin cancer
second most common:
- lymphoma
116
Management of essential thrombocytosis
aspirin and hydroxycarbamide (reduce plt)
117
Long term risk of radiotherapy
secondary cancer
118
Which leukaemia can lead to DIC
AML
119
Complication of heparin induced thrombocytopenia
- THROMBOCYTOPENIA
- THROMBOSIS
120
Painless lymph node in neck
PUO
HOdgkins
121
Which heparin has higher risk of HIT
UFH > LMWH
122
Thrombocytopenia on chemotherapy
myelosuppression
123
What is the most important prognostic factor for AML
cytogenetics:
- poor - Chr 5 or 7
- good - t(8:21) or 16
124
Action of desmopressin in haemophilia A
leads to release of pre-formed VWF --> bind to factor 8 and prolong factor 8 lifespan
125
Complication of myeloproliferative disorder
gout
126
Myeloproliferation
raised wcc
raised plt
127
Long term complication of chemotherapy and radiotherapy
leukaemia
128
Why do you see raised LDH and BR
haemolysis
129
basophilic stippling
lead poisoning
impaired Hb synthesis
alcoholism
megaloblastic anemias
130
Pappenheimer body
- iron containing inclusion body in reticulocyte
myelodysplastic syndrome (MDS), sideroblastic anemia
hemolytic anemia
lead poisoning
sickle cell disease.
131
adult T cell leukaemia/lymphoma
HTLV1 virus
skin lesion
lymphadenopathy
132
Lymphoma diagnosis
EXCISION biopsy
133
Felty syndrom
neutropenia
splenomegaly
rheumatoid arthritis
133
134
G6PD drug induced
heinz body --> oxidised --> removed by spleen --> extravascular haemolysis --> bite cells
135
Black water fever
falciparum + malariae spp
acute jaundice, haemolysis, AKI
due to acute anaemia
follows acute treatment for malaria
136
Significant splenomegaly
CML
- raised WCC
Myelofibrosis
- pancytopenia
137
138
Most useful test for haemolysis vs aplastic anaemia
reticulocyte count
- high in haemolysis
- low in aplastic anaemia
139
Methaemoglobinaemia
oxidation of Fe2+ to Fe3+
140
Antiphospholipid syndrome with x2 VTE
Lifelong warfarin
141
TTP treatment
urgent plasma exchange
142
TTP
deficiency of ADAMTS13
overlaps with haemolytic uraemic syndrome (HUS)
143
Prolonged bleeding time does not occur in
Haemophilia
144
beta thalassaemia trait
low HbF
high HbA2
145
Parvomyxovirus
measles
mumps
146
Aplastic crisis
pancytopenia
bone marrow failure
parvovirus 19
147
Polycythaemia vera complications
- thrombotic events
- myelofibrosis
- acute leukaemia (risk increased with chemotherapy treatment)
148
Complication of myelofibrosis
AML
149
Primary Polycythaemia EPO level
LOW
(negative feedback)
150
150
Paraprotein in multiple myeloma
IgG
151
Gestational thrombocytopenia
no intervention
observe
152
fatty bone marrow
aplastic anaemia
153
Blood products in UK negative for
Hep B and C
syphillis
HIV
154
Which virus must be negative for pregnant women for blood transfusion
CMV
155
DAT (coombs test)
direct antiglobulin test
- for haemolytic anaemia
- warm vs cold (acitivity)
- warm- IgG
- cold- IgM
156
Warm coombs
SLE
CLL
Lymphoma
157
Cold coombs
mycoplasma
EBV
Influenza
non hodgkins
158
MAHA
| microangiopathic haemolytic anaemia
small vessel
red cell breakdown
- RBC shredded and sheared from platelet plug (mechanism not a disease)
159
HUS
| Haemolytic uraemic syndrome
## Footnote
MAHA + renal failure + thrombocytopenia
Ecoli--> shiga like toxin --> endothelial damage --> plt plug forms --> RBC shears --> plt being consumed in plug formation --> renal failure
160
TTP
| Thrombotic throbocytopaenic purpura
## Footnote
clot form + low plt + rash
VWF sticky --> stick to healthy endothelium --> reduced ADAMTS13 (usually present) --> persistence of sticky VWF --> plug form --> consume plt --> MAHA
| NOTE: Brain (confusion and neuro)
161
DIC
| disseminated intravascular coagulation
## Footnote
all over body + within blood vessel + clotting
sepsis/tumour/pancreatitis --> XS tissue factor --> XS extrinsic clotting cascade --> lots of clots form --> deplete plt and clotting factors --> wide spread bleed
162
ADAMTS13 role
protein that cleans up dodgy sticky VWF
163
Extrinsic clotting cascade
exposed tissue factor on damaged tissue --> activate F7 to F7a --> eventual fibrinogen to fibrin --> fibrin mesh
164
VWF role
form initial plt plug
bind to factor 8 and stabilise
(therefore F8 is low in VWD)
165
Management of acute promyelocytic leukaemia
ATRA
all transretinoic acid
166
Hodgkins vs non hodgkin
Hodgkin:
- b cell only
- single group of LN usually
- reed steinberg cell
Non hodgkin
- 90% of all lymphoma
- b or t cell
- multiple groups of nodes
- painless LN, B symptoms, pruritis
167
MGUS
Smouldering multiple myeloma
multiple myeloma
| spectrum of plasma cell disease
MGUS and smouldering - premalignant
MGUS:
- M protein <3
- Marrow plasma cell <10%
- no CRAB sx
Smouldering
- M protein >3
- marrow plasma cell 10-60%
- no CRAB sx
myeloma
- CRAB
- Marrow plasma >60%
- more than 1 lesion on MRI
168
Multiple myeloma
explanded MONOCLONAL clone of plasma cells and paraprotein (immunoglobulin)
169
Non hodgkins lymphoma types
Follicular
MALT lymphoma (H.pyelori)
Mantle lymphoma (11)
Diffuse large B cell lymphoma
Burkitt (8)
Peripheral T cell lymphoma
170
Active bleed INR 8
PCC and Vit K
171
Myelodysplasia
abnormal differentiation of cells in bone marrow
therefore pancytopenia
can progress to AML (1/3)
172
Myelofibrosis
cloncal expansion -> fibrotic scar tissue
pancytopenia
tear drop cell
dry tap
massive splenomegaly (outside cell production)
173
ATRA mechanism
promote APML cell to mature to granulocyte
apoptosis of these cells
174
Most common infection in plt transfusion
s.aureus
stored in room temp for up to 5 days
175
RBC storing
fridge
176
FFP storing
frozen
