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Haematology Flashcards

1
Q

What are the key presentations of iron deficiency anaemia?

A

fatigue
pallor
weakness

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2
Q

What are the causes of microcytic anaemia?
(TAILS)

A

Thalassaemia
Anaemia of chronic diseasae
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

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3
Q

What can cause normocytic anaemia? (3A’s 2H’s)

A

acute blood loss
anaemia of chronic disease
aplastic anaemia
haemolytic anaemia
hypothyroidism

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4
Q

What is megaloblastic anaemia?

A

impaired DNA synthesis, cell grows larger instead of dividing

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5
Q

What causes megaloblastic anaemia?

A

B12 or folate deficiency

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6
Q

What can cause normoblastic macrocytic anaemia?

A

alcohol
reticulocytosis
hypothyroidism
liver disease
drugs e.g. azathioprine

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7
Q

What are the general symptoms of iron deficiency anaemia?

A

tiredness
shortness of breath
headaches
dizziness
palpitations

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8
Q

What are the specific symptoms of iron deficiency anaemia?

A

pica
hair loss

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9
Q

What are the specific signs of iron deficiency?

A

koilonychia (spoon shaped nails)
angular cheilitis
atrophic glossitis
brittle hair and nails

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10
Q

What are the 1st line investigations for iron deficiency anaemia?

A

serum ferritin
FBCs - reduced Hb, MCV
B12
folate

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11
Q

What other investigations might you do for iron deficiency anaemia?

A

FIT test
bone marrow biopsy
colonoscopy, GI investigations (cancer)

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12
Q

What is the 1st line management for iron deficiency anaemia?

A

oral iron replacement
ferrous sulphate

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13
Q

What is the 2nd line treatment for iron deficiency anaemia?

A

IV iron replacement

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14
Q

What is haemolytic anaemia?

A

a number of conditions that result in the premature destruction of RBCs

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15
Q

What are the key presentations of haemolytic anaemia?

A

pallor
jaundice
fatigue

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16
Q

What are the signs of haemolytic anaeamia?

A

splenomegaly
episodic dark urine (haemoglobinuria)

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17
Q

What are the symptoms of haemolytic anaemia?

A

SOB
dizziness
triggered by exposure to cold

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18
Q

What are the inherited haemolytic anaemias?

A

hereditary spherocytosis
hereditary elliptocytosis
thalassaemia
sickle cell anaemia
G6PD deficiency

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19
Q

What are the acquired haemolytic anaemias?

A

autoimmune and alloimmune anaemia (transfusion reactions and disease of newborn)
paroxysmal nocturnal haemoglobinuria
microangiopathic haemolytic anaemia
prosthetic valve related haemolysis

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20
Q

what is hereditary spherocytosis?

A

autosomal dominant conditions, RBCs are spheres and easily broken down when passing through spleen

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21
Q

How do you treat hereditary spherocytosis?

A

folate supplementation and splenectomy

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22
Q

How does hereditary elliptocytosis differ from spherocytosis?

A

RBCs are ellipse shape, presentation and treatment is the same as spherocytosis (folate supplementation and splenectomy)

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23
Q

What type of inheritance is G6PD deficiency?

A

X linked recessive

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24
Q

What triggers haemolysis in G6PD deficiency?

A

broad beans, infections, medications (anti-malarials)

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25
What investigations might you do for G6PD deficiency?
blood film- Heinz bodies G6PD enzyme assay
26
What is autoimmune haemolytic anaemia and what types can it be split into?
antibodies are created against the persons RBCs, it can be split into warm and cold type (symptoms occur at warmer or colder temps)
27
What can cause warm type autoimmune haemolytic anaemia?
it is mainly idiopathic
28
How does cold type autoimmune haemolytic anaemia work?
antibodies cause agglutination of RBCs at cold temperatures, they are destroyed by the spleen
29
How do you manage autoimmune haemolytic anaemia?
blood transfusions prednisolone rituximab (monoclonal antibody against B cells) splenectomy
30
What two scenarios can alloimmune haemolytic anaemia occur?
haemolytic transfusion reactions haemolytic disease of the newborn
31
What happens in haemolytic transfusion reactions?
immune system produces antibodies against antigens of transfused RBCs
32
What happens in haemolytic disease of the newborn
mothers antibodies attack foetus RBCs
33
What three features result from the destruction of RBCs in haemolytic anaemia?
anaemia splenomegaly - spleen filled with destroyed blood cells jaundice - unconjugated bilirubinaemia
34
What are the 1st line investigations for haemolytic anaemia?
FBCs -shows normocytic anaemia blood film shows schistocytes (RBC fragments) direct Coombs test
35
What is the direct Coombs test?
tests for circulating antibodies that have the potential to induce RBC haemolysis
36
What is the gold standard investigation for haemolytic anaemia?
reticulocyte count (increased >1.5%)
37
What is the first line treatment for haemolytic anaemia?
removing offending agent/underlying condition/supportive care (folic acid + transfusion)
38
What is aplastic anaemia?
pancytopenia (deficiency of all components of blood) hypocellular marrow (decreased production in haematopoietic cell lineages) and no abnormal cells
39
What are the key presentations of aplastic anaemia?
recurrent infections fatigue pallor bruising
40
What are the signs for aplastic anaemia?
tachycardia signs of congenital aetiology - persistent warts, osteoporosis, hearing loss, short stature
41
What is the main symptom of aplastic anaemia?
dyspnoea
42
What is the first line investigation for aplastic anaemia?
FBCs reticulocyte bone marrow biopsy (gold standard)
43
How do you manage aplastic anaemia?
immunosuppressive therapy blood transfusions antibiotic/fungal agents stem cell transplant
44
What inheritance pattern is sickle cell anaemia?
autosomal-recessive single gene defect in beta chain of haemoglobin
45
What are the key presentations of sickle cell anaemia?
often picked up because of heel prick test dactylitis
46
What are signs of sickle cell anaemia?
pallor, jaundice, tachycardia acute chest syndrome: chest pain, fever, tachypnoea, dyspnoea, hypoxaemia
47
What are the symptoms of sickle cell anaemia?
bone pain, lethargy, dizziness, visual floaters, SOB, cold peripheries
48
What are the first line investigations for sickle cell anaemia?
peripheral blood smear FBC/reticulocyte Hb electrophoresis (separates normal and abnormal Hb)
49
What are the gold standard investigations for sickle cell anaemia?
Hb electrophoresis high performance liquid chromatography (HPLC)
50
What is the general management for sickle cell anaemia?
abx prophylaxis - penicillin V hydroxycarbamide - stimulate HbF production blood transfusions bone marrow transplant (curative)
51
What is splenic sequestration crisis (sickle cell)?
blockage of blood flow to spleen - leads to severe anaemia and hypovolaemiac shock
52
How is a splenic sequestration crisis managed?
fluid resus, blood transfusion splenectomy if recurrent
53
What is an aplastic crisis (sickle cell)?
halt in creation of new blood cells triggered by parvovirus B19
54
What is the management in sickle cell anaemia with vaso-occlusive crisis?
analgesia treatment of priapism
55
What is the management for sickle cell anaemia with acute chest syndrome?
abx or antiviral for infection blood transfusions incentive spirometry artificial ventilation
56
What can all types of leukaemia lead to?
pancytopenia (low RBCs, platelets, WBCs)
57
When are the different kinds of anaemia most common? (ALL CeLLmates have CoMmon AMbitions)
acute lymphocytic leukaemia (ALL) - under 5 and over 45 Chronic lymphoid leukaemia (CLL) - over 55 Chronic myeloid leukaemia (CML) - over 65 Acute myeloid leukaemia (AML) - over 75
58
What are the key presentations of leukaemia?
fatigue fever pallor petechiae abnormal bleeding hepatosplenomegaly lymphadenopathy (children or young adults with petechiae or hepatosplenomegaly should be referred to the hospital)
59
What blood tests are used if leukaemia is suspected?
FBCs (pancytopenia) blood film - look for abnormal cells and inclusions lactate dehydrogenase (raised)
60
What is the gold standard investigation for leukaemia?
bone marrow biopsy (taken from the iliac crest) bone marrow trephine provides a better assessment but takes a few days
61
What is acute lymphoblastic leukaemia?
malignant change in lymphocyte precursor cell, causes acute proliferation of one type of lymphocyte (normally B lymphocyte)
62
When is it most common to see acute lymphoblastic leukaemia?
2-4 year olds, associated with downs syndrome
63
What gene translocation is ALL associated with?
philadelphia chromosome translocation
64
What is seen on a blood film in ALL?
blast cells
65
What is the typical treatment for ALL?
chemo - methotrexate steroids - prednisolone
66
What is chronic lymphocytic leukaemia?
chronic proliferation of one type of lymphocyte - typically B lymphocyte
67
What do patients typically present with with CLL?
often asymptomatic anaemia bleeding weight loss infections (most common leukaemia in adults)
68
What can CLL cause in patients? (hint: type of anaemia)
warm autoimmune haemolytic anaemia
69
CLL can transform into high-grade lymphoma. What is this transformation called?
Richters transformation
70
What is seen on a blood film in CLL?
smear or smudge cells - fragile RBCs are ruptured and leave a smudge on the film
71
What is the typical treatment for CLL?
chemo - fludarabine can be used alongside rituximab and cyclophophamide
72
What is chronic myeloid leukaemia?
When an abnormal blast cell takes up a high proportion of the blood (10-20%)
73
What are the three phases of CML?
chronic phase accelerated phase blast phase
74
What are the features of the chronic phase of CML? (time/symptoms)
last around 5 years normally asymptomatic may be diagnosed accidentally with raised WCC
75
What are the features of the accelerated phase of CML?
abnormal blast cell takes up high proportion of blood - symptomatic: anaemia thrombocytopenia immunocompromised
76
What are the features of the blast phase in CML?
higher proportion of blast cells (>30%) severe symptoms pancytopenia fatal
77
What gene translocation is CML associated with?
Philadelphia chromosome translocation
78
What is the typical treatment for CML?
tyrosine kinase inhibitor (imatinib)
79
When does acute myeloid leukaemia typically present?
middle age (most common acute leukaemia in adults
80
What myeloproliferative disorders can transform into AML?
polycythaemia ruby vera myelofibrosis
81
What can be seen on a blood film in AML?
blast cells with rods in their cytoplasm called Auer rods
82
What are the chemotherapies used to treat AML?
cytarabine and an anthracycline drug (daunorubicin)
83
What are the typical treatments for leukaemia?
chemotherapy and steroids
84
What other therapies can be used to treat leukaemias?
radiotherapy bone marrow transplant surgery
85
What are some complications of leukaemia?
failure stunted growth in kids neurotoxicity infertility secondary malignancy cardiotoxicity tumour lysis syndrome
86
What is tumour lysis syndrome?
release of uric acid from destroyed cells during chemo, can cause acute kidney injury
87
What medications can reduce uric acid levels in tumour lysis syndrome?
allopurinol or rasburicase
88
What other chemicals can be released in tumour lysis syndrome that need to be monitored?
potassium and phosphate
89
What is Hodgkin's lymphoma (HL)?
malignancy arising from mature B cells
90
What are the key presentations of HL?
lymphadenopathy
91
What are the signs of HL?
Fever weight loss night sweats
92
What would blood tests show in HL?
raised lactate dehydrogenase - non specific
93
What is the gold standard investigation for HL?
excisional lymph node biopsy
94
When is HL most common?
peaks at 20 and 75 yrs
95
What is the a key finding on lymph node biopsy in HL?
Reed-Sternberg cells - abnormally large B cells with multiple nuclei that have nucleoli inside them
96
What staging system is used for HL and non-HL?
Ann Arbor staging
97
What is stage 1 lymphoma?
confined to one region of lymph nodes
98
What is stage 2 lymphoma?
more than one region but only either above or below the diaphragm
99
What is stage 3 lymphoma?
affects lymph nodes above and below the diaphragm
100
What is stage 4 lymphoma?
involvement of non-lymphatic organs such as liver or lungs
101
What is the chemo management for HL? (ABVD)
doxorubicin (adriamycin) bleomycin vinblastine dacarbazine (sometimes followed with radiotherapy)
102
Name a common non-Hodgkins lymphoma
Burkitt lymphoma MALT lymphoma (mucosal-associated lymphoid tissue) diffuse large B cell lymphoma
103
What is Burkitt lymphoma associated with?
EBV, HIV and malaria
104
What is MALT lymphoma associated with/
H. Pylori
105
What pesticide is associated with non-HL?
trichloroethylene
106
What chemo combination is most commonly used in non-HL? (CHOP)
cyclophosphamide doxorubicin vincristine prednisolone
107
What other treatments may be used in non-HL?
monoclonal antibodies - rituximab radiotherapy stem cell transplant
108
What is myeloma?
a cancer of plasma cells that causes rapid multiplication and the production of one type of antibody (usually IgG) called a monoclonal paraprotein.
109
What is MGUS?
monoclonal gammopathy of undetermined significance. There is an abundance of one type of antibody without features of myeloma or cancer
110
What is smouldering myeloma?
progression of MGUS, premalignant
111
What is myeloma bone disease?
increased osteoclast and decreased osteoblast activity due to interaction of cytokines from plasma cells - increased risk of fractures
112
How can myeloma effect the kidneys?
can cause myeloma renal disease - tubules can become blocked by the immunoglobulins
113
How does myeloma effect plasma viscosity?
it increases it
114
What can increased plasma viscosity lead to?
easy bruising/bleeding purple discolouration of extremities heart failure vision loss
115
What are the key presentations of myeloma? (CRAB)
calcium (elevated renal failure anaemia bone lesions/pain
116
When should you consider investigations for myeloma?
anyone over 60 with persistent bone pain
117
What first line investigations should you do for myeloma?
FBC - low WCC Calcium - raised ESR - raised plasma viscosity - raised
118
What are the 2nd line investigations for myeloma? (BLIP)
Bence jones protein (urine electrophoresis) serum free Light-chain assay serum Immunoglobulin serum Protein electrophoresis
119
What is the gold standard investigation for myeloma?
bone marrow biopsy
120
How can you assess for bone lesions in myeloma?
whole body MRI or CT skeletal survey (X-ray)
121
What is the initial chemotherapy for myeloma?
bortezomid thalidomide dexamethasone
122
What prophylaxis is needed for chemotherapy with thalidomide?
VTE prophylaxis with aspirin or LMWH
123
How can you manage myeloma bone disease?
bisphosphonates (suppress osteoclasts) radiotherapy orthopaedic surgery (stabilise bones) cement augmentation of fractures/lesions
124
Name three myeloproliferative disorders
primary myelofibrosis polycythaemia vera essential thrombocythaemia
125
What cell line undergoes proliferation in primary myelofibrosis?
haematopoietic stem cell
126
What cell line undergoes proliferation in polycythaemia vera?
erythroid cells
127
What cell line undergoes proliferation in essential thrombocythaemia?
megakaryocytes
128
myoproliferative disorders are associated with which genes?
JAK2 MPL CALR
129
What is polycythaemia?
a high concentration of RBCs in the blood
130
What are the types of polycythaemia?
absolute (increased number of erythrocytes) - primary and secondary relative (normal RBCs, reduced plasma)
131
what is myelofibrosis?
the proliferation of a cell line leads to fibrosis of the bone marrow
132
Why does myelofibrosis happen?
in response to cytokines (typically fibroblast growth factor) from proliferating cells
133
What can myelofibrosis lead to?
anaemia and leukopenia
134
what is it called when blood cell production starts to happen in other areas like the liver and spleen?
extramedullary haematopoiesis
135
What can extramedullary haematopoiesis lead to?
hepatosplenomegaly
136
What are the typical systemic symptoms of myelofibrosis?
fatigue weight loss night sweats fever
137
What signs may show in myelofibrosis due to underlying problems?
anaemia (not in polycythaemia) abdominal pain (splenomegaly) ascites, varices, abdo pain (portal hypertension) bleeding and petechiae (low platelets) thrombosis, red face (raised RBCs) infections (low WCC)
138
What are the key presentations of polycythaemia vera?
conjunctival plethora (redness in conjunctiva) a ruddy complexion (red) splenomegaly
139
What would and FBC show in polycythaemia vera?
raised Hb
140
What would an FBC show in primary thrombocythaemia?
raised platelet count
141
What would an FBC show in myelofibrosis (due to primary MF, PV or ET)
anaemia High or low WCC high or low platelets
142
What are the gold standard tests for myelofibrosis?
bone marrow biopsy bone marrow aspiration - dry genetic testing or JAK2, MPL, CALR
143
How can you manage polycythaemia?
venesection - to keep Hb down aspirin VTE prophylaxis chemo - hydroxycarbamide
144
How do you manage primary myelofibrosis if symptomatic/severe?
allogenic stem cell transplant chemo - ruxolitinab (JAK2 inhibitor) supportive management for anaemia/hypertension/splenomegaly
145
How do you manage essential thrombocythaemia?
aspirin chemo - hydroxycarbamide
146
What is pernicious anaemia?
lack of intrinsic factor which leads to B12 deficiency
147
What does vitamin B12 do?
production of blood cells and myelination of nerves
148
What are the key presentations of pernicious anaemia?
fatigue pallor peripheral neuropathy
149
What are the neurological symptoms of pernicious anaemia?
paraesthesia (pins and needles)
150
What is the 1st line investigation for pernicious anaemia?
intrinsic factor antibody
151
What are the 1st line treatments for pernicious anaemia with no neurological features?
oral replacement with cyanocobalamin
152
What are the 2nd line treatments for pernicious anaemia?
IM injection of 1mg hydroxycobalamin 3x weekly for 2 weeks
153
What is the treatment for pernicious anaemia with neurological symptoms?
1mg hydroxycobalamin injection every other day until symptoms improve
154
What supplementation can be used to treat pernicious anaemia?
folic acid
155
What can treating B12 deficient patients with folate lead to?
subacute combined degeneration of the cord
156
What is glucose-6-phosphate deficiency (G6PD)?
inherited X linked enzyme where patients are susceptible to developing haemolytic anaemia?
157
What does glucose-6-phosphate do for RBCs?
prevent oxidation damage by reducing NADP to NADPH
158
What are the key presentations of G6P deficiency?
jaundice pallor dark urine
159
What is the 1st line test for G6P deficiency?
FBCs
160
What is the gold standard test for G6PD?
G6PD spectrophotometric assay
161
What is the 1st treatment for G6PD with acute haemolysis?
supportive care + folic acid + blood transfusion (+/- splenectomy in chronic non-spherocytic haemolytic anaemia w/ splenomegaly)
162
What is the treatment for G6PD in neonates with prolonged indirect hyperbilirubinemia?
phototherapy and exchange transfusion
163
What are the key presentations of malaria?
fever/ history of fever headache weakness
164
What are the first line investigations for malaria?
giemsa-stained thick and thin blood smears (gold standard)
165
what is the first line treatment for malaria?
chloroquine or hydroxychloroquine
166
What are the key presentations of HIV?
fevers/night sweats fatigue lymphadenopathy weight loss skin rashes oral ulcers/thrush angular cheilitis
167
What are the 1st line investigations for HIV?
serum HIV enzyme-linked immunosorbent assay (ELISA) HIV rapid test
168
What is the gold standard test for HIV?
serum western blot
169
What is the 1st line treatment for HIV?
antiretroviral therapy (ART)- INSTI, PI, NNRTI
170
What can cause problems with production of platelets?
sepsis B12 or folic acid deficiency liver failure - thrombopoietin deficiency leukaemia myelodysplastic syndrome
171
What can cause abnormal destruction of platelets?
medications alcohol ITP TTP heparin-induced thrombocytopenia haemolytic-uraemic syndrome
172
What symptoms might mild (<50x10^9/l) thrombocytopenia have?
easy bruising/bleeding nosebleeds bleeding gums heavy periods blood in urine or stools
173
What are the risks of severe thrombocytopenia (<10x10^9/L)?
high risk of spontaneous cranial bleeding or GI bleeds
174
what is immune thrombocytopenic purpura (ITP)?
Where antibodies (IgG) are created against platelets and cause low platelet count
175
What is secondary ITP?
all forms of ITP where associated medical conditions can be identified
176
What are the key presentations of ITP?
bleeding absence of systemic symptoms (weight loss, fever etc) absence of TP causing meds, absent spleno/hepatomegaly absent lymphadenopathy purpura
177
What is the 1st line test for ITP?
FBC - high WCC, low Hb, low platelets
178
What medicinal treatments are there for ITP?
corticosteroid - prednisolone IV immunoglobulins rituximab (monoclonal antibody against B cells)
179
What surgical procedure may be done for ITP if the spleen is trapping platelets?
splenectomy
180
What is thrombotic thrombocytopenic purpura (TTP)?
a syndrome that causes clots to develop in the small vessels
181
In which protein is there an abnormality in TTP?
ADAMTS13
182
What does the ADAMTS13 protein do?
inactivated von Willebrand factor to reduce platelet adhesion
183
How do the blood clots in TTP effect RBCs?
They break them up causing haemolytic anaemia
184
what are the key presentations of TTP?
non-specific prodrome neurological symptoms: coma, focal abnormalities, seizures, headache, confusion
185
What are the first line investigations for TTP?
FBCs - raised WCC, low Hb, low platelets raised bilirubin raised creatine schistocytes on blood smear
186
What is the 1st line treatments for TTP?
plasma exchange + corticosteroids (prednisolone) rituximab (monoclonal antibody against B cells)
187
What is heparin induced thrombocytopenia (HIT)?
exposure to heparin causes the creation of antibodies against platelets
188
How do the heparin induced antibodies effect platelets?
BOTH bind to platelets to activate clotting mechanisms AND break down platelets leads to low platelets and hypercoagulable state
189
How id HIT diagnosed?
HIT antibodies in blood
190
What is alpha thalassemia?
disorders of haemoglobin synthesis in the alpha chains
191
What are the key presentations of thalassemia?
microcytic anaemia fatigue pallor jaundice splenomegaly
192
What is the gold standard investigation for thalassemia?
Gap-PCR (shows DNA deletions or gene rearrangements) FBC shows microcytic anaemia Hb electrophoresis
193
What is the treatment for mild/severe alpha thalassemia?
frequent blood transfusions + folate supplementation chelation therapy (removes excess iron in blood) splenectomy bone marrow transplant can be curative
194
What are the signs of beta thalassemia?
low height/weight large head misaligned teeth spinal changes
195
What is the 1st line management for people with beta thalassemia trait?
genetic counselling + iron advice
196
What is the treatment for beta thalassemia intermedia?
blood transfusions (1st line) chelation splenectomy genetic counselling stem cell transplant
197
What is the treatment for beta thalassemia major?
1st line: regular transfusion + iron monitoring + chelation + genetic counselling splenectomy stem cell transplant
198
What is spherocytosis?
an autosomal dominant RBC abnormality, defects in structural membrane proteins
199
What is elliptocytosis?
autosomal dominant condition, causes ellipse shaped RBCs
200
What are the key presentations of membranopathy (spherocytosis/elliptocytosis)
pallor jaundice splenomegaly fatigue
201
What is the gold standard test for membranopathies?
NaCl osmotic fragility tests
202
What is the first line treatment for membranopathies?
RBC transfusions
203
What are alternative managements for membranopathies?
folic acid supplementation splenectomy
204
What are the 4 types of platelet disorders?
reduced production increased clearance sequestering in spleen dilution
205
What are the key presentations for platelet disorders?
petechiae/ecchymoses retinal haemorrhage bleeding at the back of the buccal mucosa
206
What are the 1st line investigations for platelet disorders?
FBCs peripheral smear
207
What is Von Willebrand's disease (VW)?
the most common inherited bleeding disorder due to abnormalities in the VWF
208
There are many underlying genetic causes of VWD, what is the most common inheritance pattern?
autosomal dominant
209
What are the key presentations of VW?
bleeding from minor wounds post op bleeding easy bruising menorrhagia (FH of bleeding is very relevant)
210
What is the gold standard test for VW?
vWF multimer analysis
211
What is the first line management for VW?
Desmopressin - stimulates vWF vWF infusion factor VIII infusion
212
What can be given to pts experiencing menorrhagia with VW?
tranexamic acid mefanamic acid norethisterone combined oral contraceptive pill mirena coil
213
What procedure may be required in extreme cases of VWD in women?
hysterectomy
214
What deficiency causes haemophilia A?
Factor VIII
215
What deficiency causes haemophilia B?
Factor IX
216
What inheritance pattern is haemophilia?
X linked recessive
217
How does haemophilia present in neonates?
intracranial haemorrhage haematomas cord bleeding
218
What are the classic presentations of haemophilia A/B?
bleeding into muscles and joints (haemoathrosis)
219
What is the gold standard investigation for haemophilia?
plasma factor VIII, IX assay genetic testing
220
What is the 1st line treatment for haemophilia?
IV clotting factors (VII, IX)
221
What is a complication of IV clotting factor infusion?
antibodies being produced against the clotting factors
222
What is the management for acute bleeding episodes or surgery in haemophilia?
infusions of clotting factor desmopressin antifibrinolytics
223
Why is desmopressin given in haemophilia?
it activated Von Willebrand factor
224
Give an example of an antifibrinolytic
tranexamic acid
225
What is disseminated intravascular coagulopathy?
an acquired syndrome causing activation of coagulation pathways resulting in thrombi and platelet depletion
226
What are the key presentations of disseminated intravascular coagulopathy?
oliguria hypotension tachycardia
227
What are the 1st line investigations for disseminated intravascular coagulopathy?
platelet count (low) fibrinogen (low) D-dimer (high) high PT/APTT
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What is the first line treatment for disseminated intravascular coagulopathy?
treatment of underlying cause (sepsis)
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What are other treatments for disseminated intravascular coagulopathy?
platelets/ coag factor inhibitors heparin cyroprecipitate (if low fibrinogen)
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What is glandular fever?
a clinical syndrome most commonly caused by epstein-barr virus infection
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What are the key presentations of glandular fever?
fever pharyngitis cervical or generalised lymphadenopathy malaise
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What are the 1st line investigations for glandular fever?
FBCs, LFTs EBV specific antibodies
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What is the gold standard investigation for glandular fever?
in-situ hybridisation
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What is the 1st line management for glandular fever?
supportive care corticosteroid if w/ haemolytic anaemia or w/ thrombocytopenia
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What happens in haemolytic disease of the newborn
mothers antibodies attack foetus RBCs
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What are the neurological symptoms of pernicious anaemia?
paraesthesia (pins and needles)
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What is the gold standard investigation for leukaemia?
bone marrow biopsy (taken from the iliac crest) bone marrow trephine provides a better assessment but takes a few days

medicine phase 2 a (10 decks)

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