haematology Flashcards

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1
Q

signs and symptoms of anaemia

A
tiredness
pallor
koilonychia
dizziness
angina/claudication if concomitant arterial disease
breathlessness
tachycardia
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2
Q

investigations into red blood cell disorders

A
FBC - HCT, MCV, Hb
serum iron
ferritin
TIBC
reticulocytes
haematinics (b12/folate)
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3
Q

causes of anaemia with low MCV, normal MCV, high MCV

A

low mcv - iron def, thalassaemia, anaemia of inflammation

normal mcv - renal anaemia, multiple myeloma, anaemia of inflammation, mixed haematinic and iron def, bleeding, haemolytic anaemia

high mcv - b12/folate, pregnancy, hypothyroid, liver disease, bleeding with increased retics, alcohol

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4
Q

how is high or low ferritin interpreted?

A

low ferritin is almost always iron deficiency

high ferritin is non-specific

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5
Q

what is pernicious anaemia?

A

inability to make or use intrinsic factor, which binds B12 to be absorbed

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6
Q

what is TIBC?

A

total iron binding capacity, surrogate measurement of transferin. combined with serum iron, can determine % blood iron saturation.

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7
Q

what is haemochromatosis?

A

abnormally high iron in the body causing multisystem disease

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8
Q

signs/symptoms of haemochromatosis?

A
heart failure, palpitations
liver failure
joint pains
diabetes
bronzed skin
hypogonadism
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9
Q

investigations into haemochromatosis?

A

iron studies
genetic test for HFE gene
liver biopsy
MRI

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10
Q

2 broad categories of causes of anaemia?

A

high destruction

decreased production

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11
Q

causes of decreased production in anaemia?

A

haematinics deficiency
bone marrow failure
anaemia of inflammation
renal anaemia

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12
Q

what kind of anaemia would you get with renal anaemia?

A

normocytic

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13
Q

examples of anaemia caused by high destruction

A

haemolysis

haemorrhage

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14
Q

what is multiple myeloma and when should it be suspected?

A

cancer of BM causing over production of antibodies which causes destruction of BM and anaemia.

suspect in normocytic anaemia

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15
Q

signs/symptoms of multiple myeloma

A

CRAB

high calcium
renal failure
anaemia
bone lytic lesions

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16
Q

diagnostic investigation of multiple myeloma?

A

serum/urine test for lightchains

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17
Q

what can cause haemolysis?

A

odd shaped RBCs e.g. spherocytosis

autoimmune haemolysis

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18
Q

what would haematinic levels be like in autoimmune haemolysis?

A

normal

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19
Q

how to investigate autoimmune haemolysis?

A

blood film
reticulocytes
direct agglutanin test (ab)
LFTs: bilirubin raised

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20
Q

what can cause changes in white cells?

A

high - infection, leukaemia

low - drugs, disease, sepsis

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21
Q

what is the risk in thrombocytosis

A

stroke, clotting events

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22
Q

what is the risk in low platelets?

A

bleeding

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23
Q

what can cause high platelet count?

A

reactive to infection

bone marrow dysfunction e.g. myeloproliferative neoplasm

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24
Q

how to investigate abnormal platelet counts?

A

redo the FBC
serial platelet monitoring
markers of inflammation

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25
Q

what can cause low platelets?

A
platelet clumping during testing
haemtinic deficiency
acute leukaemia
sepsis
DIC
ITP
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26
Q

what is polycythaemia vera?

A

primary cause of polycythaemia, genetically linked

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27
Q

how to investigate polycythaemia?

A

rule out secondary causes
genetic test for primary cause
FBC, ferritin, EPO, blood film

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28
Q

what is the gene involved in polycythaemia vera?

A

JAK2

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29
Q

what are secondary causes of polycythaemia?

A

alcohol
smoking
exogenous testosterone/EPO/steroids
EPO secreting tumour

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30
Q

signs and symptoms of polycythemia?

A

itching
flushing
splenomegaly
clotting event

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31
Q

treatment for polycythaemia

A

aspirin
venesection
hydroxycarbamide

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32
Q

adverse effect of hydroxycarbamide?

A

leg ulcers

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33
Q

what is essential thrombocytosis?

A

primary cause of raised platelets

treat like polycythaemia

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34
Q

what is myelofibrosis?

A

cancer of bone marrow causing fibrosis and progressive damage

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35
Q

signs and symptoms of myelofibrosis?

A
sweats
massive splenomegaly
cachexia
fatigue
bone pain
gout
pancytopaenia
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36
Q

investigations into myelofibrosis?

A

blood film - poikilocytes
bone marrow biopsy
look for splenomegaly

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37
Q

what causes chronic myeloid leukaemia?

A

associated with philadelphia gene - 9:22 translocation causing combination of BCR/ABL genes - upregulate tyrosine kinase causing overproliferation of cells

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38
Q

features of CML?

A

high wcc and platelets

splenomegaly

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39
Q

investigations into CML

A

blood film
BM biopsy
genetic/chromosome test

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40
Q

treatment of CML

A

tyrosine kinase inhibitors

stem cell transplant

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41
Q

complication of tyrosine kinase inhibitors?

A

pleural effusion

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42
Q

what neurological symptoms do you get with b12/folate deficiency?

A

paraesthesia

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43
Q

what vaccinations must be given before splenectomy?

A

Haemophilus influenza
meningococcal
pneumococcal

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44
Q

what kind of anaemia would also present with jaundice?

A

haemolytic anaemia

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45
Q

what will reticulocyte % be like in haemolytic anaemia?

A

raised %

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46
Q

what haematological condition do you give hydroxycarbamide?

A

polycythaemia, essential thrombocytosis

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47
Q

describing the classical description of the coagulation pathway and which tests it corresponds to

A

Tissue factor activates F7, F7a activates F10, F10a converts prothrombin into thrombin. Thrombin converts fibrinogen into fibrin which key ingredient in forming stable clots

F7 is extrinsic pathway (PT), F8, F9, F11 and (F12) is intrinsic pathway (APTT).

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48
Q

some examples of inherited bleeding disorders

A

haemophilia A and B
VW disease
other factor disorders

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49
Q

examples of acquired bleeding disorders

A
DIC
liver failure
renal failure - platelets
anticoagulant and antiplatelet drugs
vitamin K deficiency
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50
Q

questions to ask in history taking of bleeding d/o

A

history of abnormal bleeding
menstrual bleeds
family history of bleeding
bruises

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51
Q

investigations used in bleeding disorders?

A

coagulation screen - PT and APTT
specific factor levels
VWF levels
genetic testing

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52
Q

what is haemophilia A?

A

deficiency in F8. X linked recessive disease

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53
Q

how does haemophilia A usually present

A

childhood, boys, around age start to be mobile

abnormal bleeding, bruising, haemarthrosis

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54
Q

what is the function of F8 in clotting?

A

key factor in forming thrombin.

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55
Q

what will coagulation screen look like in haemophilia A

A

prolonged APTT. low or none F8 levels

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56
Q

if APTT comes back prolonged, what can be done to correct it to investigate further?

A

addition of individual Factors to find deficient factor

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57
Q

treatment of haemophilia A

A

lifelong F8 replacement

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58
Q

what is haemophilia B

A

less severe version of haemophilia A. deficiency in F9. usually present in adulthood

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59
Q

what is von willebrand disease

A

deficiency in VWF

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60
Q

what is the function of VWF?

A

binds platelets to endothelium
binds platelets to platelets
binds to F8 and brings it to site of coagulation

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61
Q

what is the most common inherited bleeding disorder?

A

von willebrand disease

62
Q

what is the inheritance pattern of von willebrand disease?

A

autosomal dominant

63
Q

what will coagulation screen look like in von willebrand disease?

A

mildly prolonged APTT

reduced F8 and VWF

64
Q

what is disseminated intravascular coagulation

A

state where body depletes all procoagulant and anticoagulant factors

65
Q

what is the process and clinical progression of DIC?

A

stage 1 starts of covertly with increased coagulation times, increased fibrin degredation products
stage 2 shows signs of haemostatic imbalance and decreased fibrinogen
stage 3 is full on clinical symptoms with raised FDPs, haemostatic failure

66
Q

what are symptoms of DIC

A

bruising, petechiae, ecchymosis, purpura
blood clots symptoms - PE, DVT, stroke
abnormal bleeding

67
Q

what are some causes of DIC

A

sepsis - gram -ve septicaemia, malaria, viral
malignancy
trauma - burns, crush injury
obstetric emergencies - placental abruption, bleeding
liver disease
major haemorrhage

68
Q

how to diagnose DIC?

A

clinical signs

lab signs - low platelets, prolonged PT and APTT, low fibrinogen, high D dimers

69
Q

management of DIC

A

resuscitation

blood products - RBC, FFP, platelets

70
Q

what do mixed myeloid progenitor cells differentiate into?

A

colony forming unit -> RBC or platelets or neutrophils among others

71
Q

what is the normal range for reticulocytes

A

0.5-2.5

72
Q

what can cause iron def anaemia

A

bleeding - GI or menstrual

coeliacs disease

73
Q

what is serum Fe, TIBC, and transferrin saturation like in iron def anaemia

A

low serum FE, high TIBC, low transferrin saturation

74
Q

what is serum Fe, TIBC, and transferrin saturation like in anaemia of chronic disease?

A

low serum iron, low TIBC, normal transferrin saturation

75
Q

what is the most common cause of B12 def in adults in western countries?

A

achlorhydria

76
Q

symptoms of B12 deficiency anaemia

A

glossitis, angular stomatitis, mild jaundice. neurological symptoms e.e. peripheral paraesthesia

77
Q

what specialised investigations to do in B12 deficiency anaemia?

A

autoantibodies, LFTs: bilirubin, intrinsic factor

78
Q

what will bone marrow biopsy show in aplastic anaemia?

A

hypocellularity - empty BM

79
Q

what will retics look like in aplastic anaemia?

A

low

80
Q

what kind of haematological condition would someone be predisposed to if he/she had a metallic heart valve?

A

haemolytic anaemia

81
Q

what will LDH be in haemolytic anaemia?

A

raised

82
Q

uncontrolled clonal proliferation of one or more cell lines is the definition of what kind of haematological disorder?

A

myeloproliferative disorder

83
Q

which disorder would you be more likely to find splenomegaly? primary or secondary polycythaemia

A

primary

84
Q

of the three cell types which ones would you find raised in primary and secondary polycythaemia?

A

all 3 in primary, only Hb in secondary

85
Q

how to differentiate myelofibrosis VS Chronic myeloid leukaemia?

A

philadelphia gene - chromsomal study

86
Q

what will Hb, WCC and PTs be in myelofibrosis?

A

low Hb, raised WCC and PTs at first then low in late stage

87
Q

what some causes of pancytopenia?

A

increased destruction - immune mediated e.g. SLE, sepsis,splenomegaly

decreased production - bone marrow failure, aplastic anaemia

88
Q

features of pancytopaenia?

A

anaemia, neutropenia, thrombocytopenia

89
Q

risks of pancytopenia?

A

infection, bleeding

90
Q

how to investigate pancytopenia?

A

FBC, retics, haematinics, autoantibodies, bone marrow biopsy, blood film. drug history

91
Q

what are 2 functions of neutrophils and consequently the biggest risk in neutropenia?

A

stopping infections from getting in and localising infections by inducing inflammation. without neutrophils, many pathogens can get in and without the process of inflammation, the pathogens can enter the blood very easily and quickly, causing distant infections within hours

92
Q

how does a neutropenic infection defer from a normal infection?

A

less inflammatory signs, no redness or swelling. very quick spread contiguously and haematogenically. patients are also prone to other types of infections that healthy people dont usually get e.g. candidiasis

93
Q

what causes ITP?

A

antibody mediated platelet destruction

94
Q

signs and symptoms of ITP?

A

bruising, bleeding.

95
Q

how to diagnose ITP?

A

by exclusion of every other possible causes e.g. haematinic def, bone marrow failure, leukaemia, SLE

96
Q

what is the common presentation of ITP

A

young, easy bruising, previous infection.

97
Q

how to treat ITP?

A

steroids, IV IG, monoclonal AB in chronic cases

98
Q

2 broad causes of bone marrow failure

A

haematological cancer

non-haematological cancer (mets)

99
Q

what are 6 common cancers that metastasise to the bone marrow

A
breast
prostate
lung
renal
thyroid
melanoma
100
Q

5 haematological malignancy that causes bone marrow failure

A
acute leukaemia
myeloma
myelofibrosis
infiltration with lymphoma
myelodysplastic syndromes
101
Q

signs of bone marrow failure?

A

pancytopenia
neutropenic sepsis
renal failure
deranged coagulation

102
Q

presence of blast cells is the differentiator between what two types of leukaemia?

A

acute vs chronic leukaemia

103
Q

what are the possible complications of leukaemia

A

bone marrow failure
neutropenic sepsis
DIC
tmour lysis syndrome

104
Q

how does acute leukaemia present?

A

bone marrow failure
general symptoms - sweats, malaise, anorexia, weight loss

tissue infiltration

105
Q

causes of acute leukaemia

A

genetic predisposition, trisomy 21,
haematological disease
irradiation
chemicals - chemotherapy

106
Q

how to investigate acute leukaemia

A

FBC - pancytopenia, raised WCC due to blasts
blood film - blast cells
BM biopsy - hypercellularity

107
Q

a patient on chemotherapy treatment starts to become confused and dehydrated, what should be checked and what are the possible causes? how to manage?

A

hypercalcaemia, tymour lysis syndrome. rehydration, allopurinol

108
Q

complications of leukaemia treatment?

A

hair loss, nausea, vomiting, tiredness, short stature, loss of intellect, depression, infertility, cardiac risks

109
Q

blasts cells can be found on BM biopsy in chronic lekaemia - T or F

A

F, blast cells are found in acute leukaemia

110
Q

what is a dermatological symptom of lymphoma?

A

itch without rash

111
Q

why are women less likely to present with haemochromatosis?

A

due to more frequent loss of blood through out life e.g. menstrual cycle, pregnancy

112
Q

what endocrine symptoms can occur in haemochromatsosi?

A

pituitary dysfunction, bronzed skin, hypogonadism

113
Q

what is ferritin like in haemochromatosis?

A

very very very high, hundred even thousands

114
Q

what can cause macrocytic anaemia with normal blood counts?

A

chronic alcohol use

115
Q

why should you not transfuse someone with macrocytic anaemia due to b12 def?

A

normal circulating volume, fluid overload will cause heart failure

116
Q

what are possible complications of blood transfusion during haemorrhage?

A

hypothermia due to cold blood

depletion of other blood components e.g. platelets,

117
Q

what is the cause of RUQ pain in haemolytic anaemia?

A

gall stones -> cholecystisis/biliary colic

118
Q

low Hb, high ESR, normal MCV is indicative of?

A

anaemia of chronic inflammation

119
Q

when should someone with polycythaemia be investigated for renal tumour?

A

if no signs of chronic hypoxic disease

120
Q

WCC of over 50 is until proven otherwise?

A

leukaemia

121
Q

how to differentiate acute and chronic lekaemia?

A

chronic will have mature cells

acute will have blast cells

122
Q

someone presenting with normocytic anaemia with raised calcium and renal failure, what is one possible haematological disease that can cause this

A

multiple myeloma

123
Q

what can cause raised PT? with normal APTT?

A

vitamin k deficiency, liver disease causing fat malabsorption, factor 7 def,

124
Q

old man with normocytic anaemia and difficulty voiding urine and numbness, what is a very important emergency not to miss out?

A

cord compression - urgent MRI

125
Q

causes of prolonged APTT?

A
lupus anticoagulant (more common than you think)
heparin
factor deficiency
126
Q

common cause of lymphadenopathy in teens?

A

EBV

127
Q

what is lymphoma

A

neoplastic changes in B/T cells in lymphoid tissue

128
Q

describe hodgkins lymphoma

A

aggressive, high grade, peaks 20s and 70s. but treatable

129
Q

describe 3 non-hodgkin’s lymphomas

A

burkitts - HIV associated, aggressive
diffused large B cell lymphoma - aggressive
follicular lymphoma - low grade, slow growing

130
Q

what are some red flags in persistent lymphadenopathy

A

painless, persistent, multiple, rapid change

on immunosuppresion, or HIV, B symptoms, supraclavicular node

131
Q

when to refer to specialist in someone with persistent lymphadenopathy

A

longer than 6 weeks, OR with B symptoms. 2 or more non-contiguous enlarged nodes,

132
Q

presentation of lymphoma

A

persistent lymphadenopathy, systemic symptoms (fever, weight loss, anorexia, fatigue, night sweats), signs of metastasis e.g. BM failure, cord compression, TLS

133
Q

how to investigate suspected lymphoma?

A
FBC - raised lymphocytes
LFTs - deranged
LDH -raised (but not diagnostic)
do monospot check 
viral screen
blood film
immunoglobulins
CT/PET
CXR
134
Q

how to diagnose lymphoma?

A

lymph node tissue sample biopsy

135
Q

what is the characteristic cell seen in hodgkin’s lymphoma?

A

reed sternberg - owl eyes

136
Q

describe the staging used in lympoma

A

ann arbour

stage 1 - 1 node
stage 2 - 2 nodes
stage 3 - cross diaphragm nodal involvement
stage 4 - extranodal mets

B - b symptoms
S - spleen involvement
E - extranodal
X - large mass >10cm

137
Q

complications of lymphoma?

A

cord compression
tumour lysis syndrome
hypercalcaemia
compression of other organs

138
Q

what can cause thromboebolisms

A
AF
metallic heart valves
ventricular assist devices
antiphospholipid syndrome
haemolytic disorders
139
Q

what can thromboembolisms cause

A
DVT
PE
budd chiari syndrome
cerebral infarct
mesenteric vein thrombosis
140
Q

what are some unmodifiable risk factors for thromboembolisms

A
age
pregnancy
cancer
inherited thrombophilic disorders
antiphospholipid antbodies
141
Q

what are some modifiable risk factors for thromboembolisms

A

air travel
immbobilisation
oral contraception
HRT

142
Q

what are 3 natural anticoagulants in your body

A

protein C
protein S
antithrombin

143
Q

what is factor V leiden

A

a point mutation in the FV gene corresponding to the cleavage site of protein C, causing inefficiency of protein C and accumulation of FV, leading to prothrombotic environment

144
Q

what is the utility of D dimer in suspected blood clot

A

it has a high NPV, i.e. if it’s negative, very unlikely to be a clot. but if it’s high, it doesn’t mean it’s a clot

145
Q

how to investigate suspected DVT?

A

wells score and ddimer. if suggestive, do leg ultrasound, and treat with lmwh. otherwise, look for alternate diagnosis

146
Q

what are some anticoagulation drugs

A
heparin
lmwh
warfarin
dabigatran
factor Xa inhibitors
147
Q

pros and cons of warfarin

A

cons: takes 3-4 days to achieve steady dose, narrow therapeutic index, requires monitoring
pros: can be reversed in bleeds

148
Q

pros and cons of DOACs

A

quick action, no need monitoring

cannot be reversed, can’t use in renal impairment

149
Q

what are some options in warfarin reversal, depending on situation

A

omit warfarin dose
oral vitamin K
IV vitamin K
prothrombin complex concentrate

150
Q

complications of anticoagulation therapy?

A

too high INR can cause spontaenous bleeding, esp worse if intracerebral