Haem Flashcards
(269 cards)
1
Q
haemophilia A affects what factor
A
8
2
Q
haemophilia B affect what factor
A
9
3
Q
haemophilia has what inheritance
A
X linked recessive
4
Q
von Hillebrands most inheritance is
A
Autosomal dom
5
Q
what is the one thing that is prolonged in haemophilia
A
APTT
6
Q
what is the bleeding time like in haemophilia
A
normal - the one yhtign that is prolonged is aPTT
7
Q
what is used to treat haemophilia A
A
factor 8
8
Q
what is done to diagnose haemophilia
A
Increased aPTT, factor 8 assay
9
Q
what is prolonged in von willebrands disease
A
bleeding time
10
Q
what is used to treat von willebrands
A
desmopressin
11
Q
DIC affects what clotting factors
A
1,2,5,8,11
12
Q
acute intermittent porphyria is defect in what enzyme
A
porphobilinogen deaminase
13
Q
there is a toxic accumulation of what in acute intermittent porphyria
A
delta aminolaevulinic acid and porphobilinogen
14
Q
what classically does acute intermittent porphoryia present with
A
abdo and neuropsychiatric symptoms in 20-40y/o
15
Q
what id dx of classically urine turns red on standing
A
acute intermittent porphyria
16
Q
porphobilinogen, porphobilonogen deaminase, delta amniolaevulinic acid
A
acute intermittent porphoryia
17
Q
what can be used for acute attacks of acute intermittent porphoryia
A
Iv haematin/haem arginate
Iv glucose if these not available
18
Q
Auer rods are seen in a subtype of AML called
A
acute promyelocytic leukemia
19
Q
bone marrow failure ie in AML presents as
A
anaemia, neutropenia, thrombocytopenia, splenomegaly, bone pain
20
Q
if antiphospholipis is likely to occur secondary to another condition what is it most likely be
A
SLE
21
Q
antiphosplipid predisposes to
A
venous &arterial thromboses,
recurrent fetal loss
thrombocytopenia
22
Q
mx of antiphospholipid syndrome in adults
A
low dose aspirin once pregnant is confirmed on urine dip
LMWH once a fetal heart is seen on US &this is usually discontinued at 34 weeks gestation
23
Q
aplastic anemia is characterised by
A
pancytopenia and hypo plastic bone marrow
24
Q
what is the anaemia like in a aplastic anaemia
A
normochromic, normocytic
25
aplastic anaemia can be the symptom of leukemias
chloramphenicol and phenytoin can cause
26
warm autoimmune haemolytic anaemia is what antibody
igG
27
is warm aihi more commonly intravscualr
no extravascular
28
anaemia
reticulocytosis
low haptoglobin
raised lactate dehydrogenase (LDH) and indirect bilirubin
blood film: spherocytes and reticulocytes
general features of haemolytic anaemia
29
spherocytes think
autoimmune haemolytic anaemia
30
what has microcytic anaemia and raised HbF and HbA2
beta thalassaemia major
31
how does beta thalassaemia major present
within first year of life with failure to thrive and hepatosplenomegaly
32
mx of beta thalassaemia major
repeated transfusions and iron chelation therapy to reduce iron overload
33
think fo what if got microcytosis disproportionate to the anaemia
beta thalassamia trait
34
hyperhsegmented neutrophils
megaloblastic anaemia
35
Heinz bodies
G6PD deficiency
alpha thalassaemia
36
what can also present as hyposplenism
coeliac
37
hyposplenism can present as
target cells
Howell jolly
pappenheimer bodies
38
intravascular haemolytic
schistocytes
39
febrile blood transfusion reaction are more common if what transfusion of what
platelets
40
minor allergic reaction eg itch and urticaria do what
temporarily stop the transfusion and antihistamine
41
what increases the risk of anaphylactic blood transfusion reaction
pts with IgA deficiency
42
acute haemolytic reaction has
fever, abdo pain and +hyptension !!
43
acute haemolytic reaction due to
ABO incompatible
44
if after transfusion has hypotension what needs to be done
stop the transfusion
45
in acute haemolytic reaction what do after stop transfusion
check identity of pt to name on blood group, send blood for direct Coombs, repeat typing and cross matching
give pt fluids
46
what drug may you consider in circulate overload transfusion reaction
loop
47
itravasculae haemolyisis in acute haemolytic reaction results in what antibodies
IgM
48
febrile reactions are due to
white blood cell HLA antibodies
49
RBC are primarily at risk of transmitting viral agents whereas platelets are stored at room temp so more at risk of
bacterial proliferation
50
blood transfusion with prion
Creutzfeldt- Jakob disease
51
when are packed red cells used
transfusion in chronic anaemia and cases where infusion of large volumes of fluid may result in cardiovascular compromise
52
what blood product has clotting factors
fresh frozen plasma
53
cryoprecipitate has a rich source of
factor 8 and fibrinogen
54
what is a blood product whereby the pts don't need to be ABO compatible
platelets
55
cell saver devices that collects pts blood during surgery and then rein fuse to pt are contraindicated in
malignant disease for risk fo facilitating disease dissemination
56
what can reverse warfarin within 1hr
Human prothrombin complex
57
Vit K takes how long to reverse warfarin
4-24hrs (4-6 for IV, 34 for oral)
58
when is fresh frozen plasma used if what is not available for reversal of warfarin
fresh frozen plasma
59
human prothrombin complex should be given with
vit K as HPC has rapid action but short half life
60
why are irradiated blood products depleted of T lymphocytes
avoid transfusion associated graft versus host disease
61
CMV is transmitted in
leukocytes
62
CMV neg blood if
granulocyte transfusion or relates to baby
63
when do you not need to give irradiated blood
preg or HIV
64
when is FFP given
If Pt or APTT >1.5
65
universal donor of FFP
AB blood
66
cryoprecipitate is most commonly used to replace
fibrinogen
67
what is used in emergency reversal of anticoagulation in pts with severe bleeding or head injury with suspected intracerebral haemorrhage
prothrombin complex concentrate
68
universal donor group for RBC is
group 0 red cells
69
best recipients for group O red blood cells
AB as they don't have anti A or anti B antibodies
70
in a non urgen scenario how long is a unit of RBC transfused over
90-120 mins
71
when should get transfusion with no ACS and with
no acs - 70
with acs - 80
72
antibodies are in the plasma antigen are on the
red cells
73
burkitts lymphoma is associated with c-myc translocation usually
t(8,14)
74
starry sky appearance also seen in
burkitts lypmhoma
75
what is given to prevent tumour lysis syndrome
rasburicase
76
what is high in tumour lysis syndrome
phosphate, potassium and uric acid
77
chemo can cause
tumour lysis syndrome
78
endemic form of burkitts lymphoma is usually where
maxilla or mandible
79
sporadic form of burkitts lymphoma tend to be where
abdominal
80
4 points to know about CLL
ANAEMIA
HYPOGAMMAGLOBINUAMEI LEADING TO RECURRENT INFCTIONS
WARM AIHA
TRANFORMATION TO NHL (RICHTERS)
81
what indicates righters transformation
lymph node swelling
fever without infection
night sweats
weight lsos
82
how does Richter transformation present
pt becomes unwell very suddenly
83
most common form of leukemias seen in adults
CLL
84
CLL is almost always due to what cell
B
85
How might CLL present
incidental finding of lymphocytosis
86
anaemia, thrombocytopenia but what is seen in CLL
lymphocytosis
87
what cells are seen in CLL
smudge or smear
88
key investigation for CLL
immunophenotyping
89
what is present in 95% of cases of CML
Philadelphia chromosome
90
philiadelhia chromosome
T9,22
91
what has an increase in granulocytes at different stages of maturation
AML
92
first line for CML
imatinib (inhibitor of tyrosine kinase)
93
what can be marked in CML causing abdo discomfort
CML
94
cryoglobulinaemia
purport and raynauds
95
what might give in uncontrolled bleeding due to haemophilia
cryoprecipitate - has fibrinogen , factor 8...
96
when do you give interim anticoagulation for DVT
if US cannot be done within 4hrs
97
if US scan is neg but d-dimer high then what
repeat scan in 6-8 days
98
first line doac for DVT
apex or rivarox
99
anticoagulant if renal impairment less than 15
LMWH
100
doac for anti phospholipid syndrome
LMWH followed by vit K antagonist
101
what is the preferred DOAC in renal impairment
apixaban as minimal renal drug clearance as most drug clearance is faecal
102
what converts fibrinogen to fibrin to stabilise clot
thrombin
103
what breaks down clots
plasmin
104
widespread clotting with resultant bleeding
DIC
105
what is once critical mediator of DIC
tissue factor
106
4 causes of DIC
sepsis
trauma
obstetric complications
malignancies
107
blood picture of DIC
LOW PLATELETS AND FINBROGEN
raised PT&APTT and fibrinogen degradation products
108
what is seen in DIC due to microangiopathic haemolytic anaemia
schistocytes
109
what drug on its own causes an increased bleeding time
aspirin
110
most common inherited thrombophilia
factor V leiden
111
factor V Leiden has activated
protein C resistance
112
what has anaemia, short stature and thumb/radial abnormalities
falconi anaemia
113
G6PD def INHERITED
X linked recessive
114
what can precipitate a crisis in G6PD def
infections and broad (fava) beans
115
G6PD def means there's reduced -- so increased red cell susceptibility to oxidative stress
glutathione
116
what is often seen in G6PD def
neonatal jaundice, intravascular haemolysis, gallstones are common
117
what may be seen on blood film in G6PD def
Heinz bodies - bite and blister cells may also be seen
118
diagnosis of G6PD def do
G6PD enzyme assay - 3 months after and acute episode of haemolytic
119
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
some drugs causing haemolysis in G6PD def
120
graft versus host is from what cells attacking
T
121
donor T cells attack recipient tissues
graft versus host
122
what is a common feature of graft versus host disease
painful maculopapular rash
123
other symptoms can get in graft versus host
diarrhoea, jaundice, N+V
124
abdo imaging in graft versus host may show
bowel wall thickening or air. fluid levels (ribbons sign)
125
first line in graft versus host
Iv methylprednisolone
126
what is a second line therapy for graft versus host that is a JAK inhibitor
RUxolitinib
127
Philadelphia chromosome in
CML
128
Mac oncogene t(8,14)
burkitt
129
might also have Philadelphia choromosne in ALL is this good or bad
poor prognosis
130
malaria associated with what lymphoma
Burkitts
131
drugs that can cause acquired haemolytic aanemais
methyldopa, penicillin
132
what bit more unusual thing can cause a haemolytic anaemia
prosthetic heart valve
133
Zieve syndrome
haemolytic anaemia following heavy alcohol use
resolves with abstinence from alcohol
134
what binds to free Hb
haptoglobin
135
haemophilia is
X linked recessive
136
only thing that is prolonged in haemophilia
APTT
137
annoying thing about haemophilia A
some pts can develop antibodies to factor 8 treatment
138
symptoms of hereditary angioedema
swelling of sublet tissues
139
prophylaxis of heredity angioedema
danazol (anabolic steiod)
140
widely used screening tool for hereditary angioedema
serum C4
condition due to low levels of C1 inhibitor
141
what virus can trigger an aplastic crisis in hereditary spherocytosis
parvovirus
142
diagnostic test in hereditary spherocytosis
EMA binding test
143
long term mx of heredity spherocytosis
folate replacement
splenectomy
144
most common type of HL
nodular sclerosing
145
nodular sclerosing HL associated with what cells
lacunar
146
HL that has best vs worst prognosis
best - lymphocyte predominant
worst - lymphocyte depleted
147
what type of HL is associated with a large number of reed Sternberg cells
mixed cellularity
148
do B symptoms in lymph,phoma suggest a good or bad prognosis
bad
149
most common place for lympahdenoapth in lymphoma
neck - usually painless and non tender
150
lymphoma think
lymphadenopathy and B symptoms
151
large cells that are multinucleate or bilobed nucleus with prominent eosinophilic nucleoli
reed Sternberg
152
eosinophilia and raised LDH may be seen in
HL
153
what is the mainstay of imaging for staging HL
PET/CT
154
what stages HL
Ann Arbor
155
two chemo regimens for HL
ABVD
BEACOPP
156
typical finding in hyposplenism
Howell jolly and siderocytes
157
treatment for ITP
steroids
158
Children with ITP usually have an acute thrombocytopenia that may follow infection or vaccination. In contrast, adults tend to have a more chronic condition.
159
ITP can present as
petechia, purpura
bleeding eg epistaxis
as low platelets!!!
160
evans syndrome is ITP associated with
AIHA
161
in ITP the anti platelet antibodies are against
glycoprotein 2b/3a/1b
162
In ITP what should be done before starting steroids to rule out leukemias
boen marrow aspiration - in ITP would show metakaryocytes in marrow
163
iron def anaemia has the highest incidence amongst who
preschool age kids
164
good source of iron
meat
165
koilonychia in iron def anaemia is
spoon shaped nails
166
does a raised ferritin rule out iron def anaemia
nope
167
what will be high in iron def anaemia
TOTAL IRON BINDING CAPACITY
168
apart from target cell and pencil poikilocytes what may be seen in iron def anaemia
anisopoikilocytosis - red blood cells of different sizes and shapes
169
in iron def anaemia how long after should be taking after stores replenished
3months
170
in contrast to normal iron def anaemia, in chronic anaemia
TIBC is low
171
alongside acute intermittent porphoryia what should be considered in pts presenting with abdo pain and neurological signs
lead posinong
172
blue lines on gum margin
lead poisoning
173
what blood film of lead pointing might show
basophilic stiplling and clover leaf morphology
174
dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol
chelate lead
175
ovaries drain to where
para aortic lymphatics
176
body of uterus drains within lymphatics in broad ligament to
iliac lymph nodes
177
other cause of megaloblastic anaemia
secondary to methotrexate
178
normoblastic causes of macrocytic anaemia
alcohol, liver disease, hypothyroidism
179
Any of the following features in a person aged 0-24 years should prompt a very urgent full blood count (within 48 hours) to investigate for leukaemia:
Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding
180
chocolate cyanosis
methaemoglobinaemia
181
Fe3+ think
methaemoglobinaemia
182
microcytic anamei a
iron-deficiency anaemia
thalassaemia*
congenital sideroblastic anaemia
anaemia of chronic disease (more commonly a normocytic, normochromic picture)
lead poisoning
183
A question sometimes seen in exams gives a history of a normal haemoglobin level associated with a microcytosis. In patients not at risk of thalassaemia, this should raise the possibility of polycythaemia rubra vera which may cause an iron-deficiency secondary to bleeding.
184
*in ---- the microcytosis is often disproportionate to the anaemia
beta thalassaemia minor
185
Monoclonal gammopathy of undetermined significance often mistaken for
myeloma
186
risk of mGUS forming myeloma
at 10 years is 10% and at 15 is 50%
187
in MGUS the paraprotein for IgG is under
30
188
myelodysplastic syndromes have the potential to transform into
AML
189
key pathophysiological feature of myelodysplastic syndrome
ineffective hematopoiesis leading to peripheral cytopenias despite a hyper cellular bone marrow
190
only curative option for myeldysplstic syndrome
haematopoetic stem cell transplant
191
bone marrow in myelodysplastic syndrome shows
dysplastic changes in hematopoeitic cells and varying degree of blasts
192
90% of myeldysplastic are primary but rest are secondary often to chemo or radiotherapy and these typically develop how long after treatment
5 years
193
in myelofibrosis unable to obtain bone marrow biopsy due to
dry tap therefore trephine biopsy needed
194
most common presenting symptom of myelofibrosis
elderly with symptoms of anaemia eg fatigue
195
what causes a massive splenomegaly
myelofibrosis
196
CRABBI
myeloma
197
peripheral blood film shows what in myeloma
roleaux formation
198
in myeloma protein electrophoresis will show raised IgA/G in serum and in the urine they are known as
Bence Jones proteins
199
what confirms the dx of myeloma
bone marrow aspiration
- if no. of plasma cells are significantly raised
200
raindrop skull
myeloma
201
major criteria for myeloma
plasmacytoma
30% plasma cells in bone marrow sample
elevated levels of M protein in blood or urine
202
in myeloma for renal part is it light chain or heavy chain deposition within renal tubules
light chain
203
2 drugs that can cause neutropenia
carbimazole and clozapine
204
can get benign ethnic neutropenia in people of black African or afro Caribbean ethnicity and this requires
no treatment
205
mechanisms include circulating antineutrophil antibodies
SLE
206
RA with hypersplenism
Feltys
207
neutropenic sepsis occurs how long after cancer therapy
7-14 dyas
208
most common cause of neutropenic sepsis
Staph epidermis (probably due to the use of indwelling lines in pts with cancer)
209
suspect neutropenic sepsis if having anti cancer treatment and temp
over 38
210
if pt has low neutrophil count before chemo what drug can be given as prophylaxis for neutropenic sepsis
fluoroquinolone
211
should you wait for WBC before starting antibiotics in neutropenic sepsis
NO
212
immediate antibiotics for neutropenic sepsis
piperacillin with tazobactam
213
if after first dose of antibiotics in neutropenic sepsis and still unwelll after 48hrs what may be given
meropenem
214
NHL can affect what cells
B or T
215
what differentiates HL from NHL
biopsy
216
extraa nodal disease is much more common in
NHL
217
what is diagnostic ix of choice in NHL
excision biopsy
218
what test if often performed in NHL as is a rf
HIV
219
Mx of NHL
rituximab (CHOP)
220
all pts before starting treatment with rituximab need to be
screened for Hep B
221
include
anaemia of chronic disease
chronic kidney disease
aplastic anaemia
haemolytic anaemia
acute blood loss
cause of normocytic anaemia
222
classically dark coloured urin ein morning
paroxysmal nocturnal haemoglobinuria
223
pts with paroxysmal nocturnal haemoglobinuria are more prone to
venous thrombosis
224
gold standard ix for paroxysmal nocturnal haemoglobinuria
flow cytoemtru to detect low levels of CD59 and CD55
225
mx for paroxysmal nocturnal haemoglbinuria
blood product repalcemtn
anticoagulation
226
what makes the threshold for transfusion in low platelet count higher
bleeding at critical sites such as CNS
227
what transfusion has the highest risk of bacterial contamination compared to there blood products
platelets
228
other causes of polycythaemia
dehydration
cops, altitude, OSA
229
poly rubra vera mutation
JAK2
230
prutirtis after hot bath, hypotension m hypeviscoity
poly rubra vera
231
what MUST be present to diagnose poly rubra vera
high haematocrit, raised red cell mass
232
why is aspirin given to poly ver pts
reduce the risk of thrombotic events
233
wha keeps hb in range in poly rubra vera
venesection
234
poly vera can progress to
myelofibrosis or AML
235
heavy painful calves post DVT
post thrombotic syndrome
236
Compression stockings have in the past been offered to patients with deep vein thrombosis to help reduce the risk of post-thrombotic syndrome. This is now not recommended.
However, once post---- has developed compression stockings are a recommended treatment. Other recommendations including keeping the leg elevated.
thrombotic syndrome
237
major of DVT/PE in preg occur when
last trimester
238
mx of DVT/PE in preg
Subcut LMWH
239
IPA def may get severe
reactions to blood transfusions
240
sickle cell inheritance
auto recess
241
symptoms of sickle don't tend to present till
4-6months
242
glutamte is substituted for valine
sickle
243
definitive dx of sickle is by
Hb electrophoresis
244
hydroxyurea moa in sickle
increase the HbF levels (prevention)
245
how often should sickle pts get pneumococcal vaccine
every 5 years
246
most common death in sickle after childhood
acute chest syndrome
247
aplastic crises and sequestration which one has low reticulicyte vs increased
reduced - aplastic
increased - sequestration
248
sideroblastic anaemia deposition of iron where
mitochondria
249
what may help in sideroblatic anaemia
pyridoxine
250
massive splenomegaly seen in
myelofibrosis and CML
251
burning sensation in hands is a characteristic symptom of
essential thrombocytosis
252
what drug reduce platelet count
hydroxyurea
253
thrombotic thrombocytopenia purport deficiency of
ADAM
254
abnormal large and sticky von willebrnads factor clump within vessels
TTP
255
thymoma associated with
myasthenia gravis
256
transexamic acid prevents
plasmin
257
tumour lysis leads to
high K and high phosphate and low calcium
258
what prevents tumour lysis syndrome
allopurinol or rasburicase
259
Clinical tumor lysis syndrome: laboratory tumour lysis syndrome plus one or more of the following:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure
260
meds that can cause VTE
combined pill
HRT especially if got progestogen
tamoxifen
antipsychotics
261
B12 absorbed where
terminal ileum
262
most common cause of B12 def
pernicious anaemia
263
other features of b12 def
sore tongue and mouth
dorsal column symptoms - join position, vibration
264
Management
if no neurological involvement 1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months
b12 def
265
Bleeding time is prolonged in von willebrands but normal in
haemophilia
266
mx of von willebrnads
transexamic acid and desmopression (raise VWF)
267
diagnostic of waldestroms
bone marrow biopsy
268
recurrent bacterial infection and eczema
wiskott aldrich
269
