Haematology Flashcards
(209 cards)
1
Q
What is the lifespan of a RBC ?
A
4 months (120 days)
2
Q
What is the lifespan of a platelet ?
A
10 days
3
Q
What is a cause of target cells on a blood film ?
A
- Iron deficiency anaemia
- Sickle cell/thalassaemia
- Post-splenectomy
4
Q
What are heinz bodies on a blood film and what is it
A
- G6PD deficiency
- Alpha-thalassaemia
5
Q
What causes Howell-Jolly bodies to be seen on a blood film ?
A
- Post splenectomy
- Non functioning spleen (sickle cell anaemia)
- Coeliac disease (hyposplenism)
6
Q
When would reticulocytes be seen on a blood film ?
A
Haemolytic anaemia
7
Q
When are smudge cells seen on a blood film ?
A
- CLL : they are ruptured white blood cells
8
Q
When are spherocytes seen on a blood film ?
A
- Autoimmune haemolytic anaemia or hereditary spherocytosis
9
Q
When are ‘Tear-drop’ poikilocytes seen on a blood film ?
A
Myelofibrosis
10
Q
What is associated with Schistocytes on a blood film ?
A
- Intravascular haemolysis
- Mechanical heart valve
- DIC
11
Q
What is associated with ‘pencil’ poikilocytes on blood film ?
A
IDA
12
Q
What is associated with Burr cells on blood film
A
Uraemia
Pyruvate kinase deficiency
13
Q
What is associated with hypersegmented neutrophils on blood films ?
A
Megaloblastic anaemia
14
Q
Give the 5 causes of microcytic anaemia (low MCV)
A
- T : thalassaemia
- A : anaemia of chronic disease
- I : IDA
- L : lead poisoning
- S : sideroblastic
15
Q
Give the 5 causes of normocytic anaemia
A
- A : anaemia of chronic disease
- A : acute blood loss
- A : aplastic anaemia
- H : haemolytic anaemia
- H : hypothyroid
16
Q
What is anaemia of chronic disease
A
Anaemia caused by CKD -> reduced production of erythropoietin
17
Q
Give the 2 causes of megaloblastic macrocytic anaemia
A
B12 deficiency
Folate deficiency
18
Q
Give 5 causes of normoblastic macrocytic anaemia
A
- Alcohol
- Reticulocytosis
- Hypothyroid
- Liver diease
- Drugs (e.g. azathioprine)
19
Q
What can cause reticulocytosis ?
A
- Haemolytic anaemia of blood loss
- It is due to rapid RBC cell turnover
20
Q
Give 5 generic Sx of anaemia
A
Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
21
Q
Give 4 generic signs of anaemia
A
Pale skin
Conjunctival pallor
Tachycardia
Raised respiratory rate
22
Q
What is the most common cause of IDA in adults
A
Blood loss
23
Q
What is the most common cause of IDA in children ?
A
Dietary insufficiency -> Pica (eating soil) is a common presentation in children
24
Q
Give 2 symptoms specific to IDA
A
Pica
Hair loss
25
Give 4 signs specific to IDA
Koilonychia
Angular cheilitis
Atrophic glossitis
Brittle hair and nails
26
Explain the blood profile of someone with IDA
- FBC : hypochromic microcytic anaemia
- Ferritin : low (correlates with iron stores)
- TIBC : increased
- Transferrin : increased
27
What is the transferrin saturation ?
- Amount of iron bound to transferrin
- Low iron = low saturation
Transferrin saturation = serum iron/TIBC
28
What should be done in new IDA without a clear underlying cause ?
- Colonoscopy and OGD
- Any pt >60 with new IDA = 2 week wait for colorectal cancer
29
Give the stepwise management of IDA
- Oral iron (ferrous sulfate for 3 mnths)
- Iron Infusion
- Blood transfusion
30
Give 4 SE of ferrous sulfate
Nausea
Abdo pain
Constipation
Diarrhoea
31
When should iron infusions be avoided ?
During infection -> 'feeds' the bacteria'
32
What is the genetic inheritance of thalassaemia
Autosomal recessive
33
Give 4 signs of anaemia more specific to thalassaemia
Jaundice
Gallstones
Splenomegaly
Poor growth and development
34
Why does thalassaemia lead to splenomegaly ?
- The RBCs are more fragile -> broken down more easily -> destroyed RBCs are collected by the spleen -> splenomegaly
35
What chromosome is involved in alpha thalassaemia
Chromosome 16 -> 2 separate alpha-globulin genes are located on each chromosome
36
Explain the 3 clinical severities on thalassaemia
- Hypochromic and microcytic blood picture but normal Hb : 1 or 2 affected alleles
- Hb H disease : 3 alleles affected = hypochromic microcytic anaemia
- Hydrops fetalis : 4 alleles -> death in utero
37
What chromosome is involved in Beta-Thalassaemia ?
- 11
- Involves abnormal copies or deleted genes
38
What is Beta-thalassaemia trait ?
- One abnormal and one normal gene
- Mild-hypochromic microcytic anaemia
- Usually asymptomatic
39
What is thalassaemia intermedia ?
- Two abnormal copes (either 2 defective or one defective and 1 deleted)
40
What is Beta-thalassaemia major ?
- Absence of beta globulin chains
41
How does beta-thalassaemia major present
- First year of life with failure to thrive and and hepatosplenomegaly
- Microcytic anaemia
- HbA2 & HbF raised
- HbA absent
42
How is Beta-thalassaemia major managed ?
repeated transfusion
this leads to iron overload → organ failure
iron chelation therapy is therefore important (e.g. desferrioxamine)
43
What is the blood profile of someone with anaemia of chronic disease
- Low serum iron
- Low TIBC
- Low transferrin saturation
- High ferritin
44
If not congenital, give 4 acquired causes of sideroblastic anaemia
Myelodysplasia
Alcohol
Lead
Anti-TB medications
45
What is seen on Ix in sideroblastic anaemia
- FBC : hypochromic microcytic anaemia
- Iron studies : high ferritin, iron and transferrin saturation
- Blood film : basophilic stipling of RBCs
46
What staining is used in the bone marrow for diagnosing sideroblastic anaemia ?
Perussian blue and it will show ringed sideroblasts
47
What are 3 signs of haemolytic anaemia
- Anaemia (fatigue, palpitations, SOB)
- Splenomegaly
- Jaundice : bilirubin is released when RBCs are destroyed
These are the signs of RBC destruction
48
What is seen on FBC and blood film in haemolytic anaemia ?
- FBC : normocytic anaemia
- Blood film : shistocytes (RBC fragments)
49
Give 5 hereditary causes of haemolytic anaemia
- Hereditary elliptocytosis
- Hereditary spherocytosis
- Thalassaemia
- Sickle cell anaemia
- G6PD deficiency
50
Give 5 acquired conditions that can cause haemolytic anaemia
- Autoimmune
- Alloimmune
- Paroxysmal nocturnal haemoglobinuria
- Microangiopathic haemolytic anaemia
- Prosthetic valve-related haemolysis
51
What is the inheritance of hereditary spherocytosis and Elliptocytosis ?
Autosomal dominant
52
Give 3 key blood findings in hereditary spherocytosis
- Raised MCHC
- Raised reticulocyte count
- Spherocytes on blood film
53
What will the parvovirus cause in someone with hereditary spherocytosis ?
- Aplastic crisis
54
How is hereditary spherocysis managed ?
- Folate and blood transfusions when needed
- Splenectomy
55
Explain the genetic inheritance of G6PD deficiency
- X linked recessive
- Defect in the gene coding for glucose-6 phosphate dehydrogenase
56
What is a characteristic feature of G6PD deficiency ?
- Episodes of haemolytic anaemic triggered by :
- Infection
- Fava beans
- Drugs : ciprofloxacin, sulfonylureas, sulfasalazine
57
What is seen on a blood film in G6PD deficiency and how is it diagnosed
- Heinz bodies
- G6PD enzyme assay
58
What is warm AIHA
- Antibodies are created against RBCs
- Usually IgG
- Haemolysis occurs at normal or above-normal temperatures
- Most common
- Usually idiopathic
59
What is cold, reactive AIHA
- Antibodies are created against RBCs
- Usually IgM
- Haemolysis occurs at low temps
60
What is cold AIHA secondary too ?
- Lymphoma
- Leukaemia
- SLE
- Infections (e.g., mycoplasma, EBV, CMV and HIV).
61
What is usued first line to treat warm AIHA?
Steroids (+/- rituximab)
62
Other than showing normocytic anaemia with shistocytes, what is a specific finding in AIHA ?
Positive Direct Coombs test
63
A patient presents with haemolytic anaemia, haemoglobinuria (RED URINE), thrombosis (DVT) and smooth muscle dystonia (erectile dysfunction).
What is the likely cause ?
- Paroxysmal noctunal haemoglobinuria
64
How is paroxysmal noctunal haemoglobinuria managed ?
- Eculizumab
- Bone marrow transplant
65
What is microangiopathic haemolytic anaemia dna give 5 causes
- Destruction of RBCs as they pass through circulation due to abnorm al clotting obstruction the blood vesels
- HUS
- DIC
- TTP
- SLE
- Cancer
66
Explain the genetic inheritance of sickle cell anaemia
- Autosomal recessive
- Affects the beta-globin chain on chromosome 11
- Results in the prodction of HbS = sickle shaped RBCs
67
How is sickle cell anaemia diagnosed
Haemoglobin electrophoresis
68
What is the general management of sickle cell anaemia ?
- Antibiotic prophylaxis with Penicillin V (phenoxymethylpenicillin)
- Long term Hydroxycarbamide
- Crizanlizumab
- Blood transfusions for severe anaemia
- Bone marrow transplant can be curative
- Pneumococcal polysaccharide vaccine every 5 yrs.
69
What can be given to increase the amount of HbF production in sickle cell anaemia
Hydroxyurea
70
Why is Crizanlizumab given in sickle cell anaemia ?
- Monoclonal antibody targetting P-selectin, an adhesion molecule on endothelial cells inside of blood vessel and platelets.
- Reduces frequency of vaso-occlusive crises by preventing RBCs sticking to the wall.
71
What is a vaso-occlusive crisis ?
- The RBCs clog the capillaries causing distal ischaemia
- Precipitated by infection, dehydration, deoxygenation
- Presents with pain in hands or feet and associated fever
- Urological emergency = priapism
72
What is a splenic sequestration crisis ?
- RBCs block blood flow to spleen
- Causing painful splenomegaly
- Causes increased reticulocyte count
73
What triggers an aplastic crisis in sickle cell anaemia
- Parvovirus B19
- Causes a sudden fall in haemoglobin and bone marrow supression leading to reduced reticulocyte count
74
What is acute chest syndrome and how does it present?
- Vessels supplying lungs become blocked by RBCs
- Fever, SOB , Chest pain, cough and hypoxia
75
What will be seen on a chest X-ray in acute chest syndrome ?
Bilateral pulmonary infiltrates
76
Define leukaemia
Cancer of a particular line of stem cells in the bone marrow
77
what are the 4 types of leukaemia ?
- Acute myeloid leukaemia
- Acute lymphoblastic leukaemia
- Chronic myeloid leukaemia
- Chronic lymphocytic leukaemia
78
Give a characteristic feature for each of the 4 types of leukaemia
- ALL : children and is associated with Down syndrome
- CLL is associated with warm haemolytic anaemia, Richter’s transformation and smudge cells
- CML has three phases, including a long chronic phase, and is associated with the Philadelphia chromosome
- AML may result in a transformation from a myeloproliferative disorder and is associated with Auer rods
79
How can the features of ALL be divided ?
-Anaemia : lethargy and pallor
- Neutropaenia: frequent or severe infections
- Thrombocytopenia: easy bruising, petechiae
80
Give 5 poor prognostic factors for ALL
- Age < 2 years or > 10 years
- WBC > 20 * 109/l at diagnosis
- T or B cell surface markers
- Non-Caucasian
- Male sex
81
What is the most common form of leukaemia in adults
- CLL
- Usually seen in adults >60
- Proliferation of well-differentiated lymphocytes
82
What is seen on a blood film in CLL?
Smudge cells (smear cells)
83
Give 4 complications of CLL
- Anaemia
- Hypogammaglobulinaemia leading to recurrent infections
- Warm autoimmune haemolytic anaemia in 10-15% of patients
- Transformation to high-grade lymphoma (Richter's transformation)
84
How would Richter's transformation present in an exam question ?
- Patient with leukaemia becomes unwell very suddenly.
- Lymph node swelling, fever without infection, weight loss etc
85
What are the 3 phases of CML ?
- Chronic phase
- Accelerated phase
- Blast phase
86
Explain the chronic phase of CML
- Often asymptomatic, diagnosed after an incidental finding of a raised white cell count.
- This phase can last several years before progressing.
87
Explain the accelerated phase of CML
- Occurs when the abnormal blast cells take up a high proportion (10-20%) of the bone marrow and blood cells.
- Patients are more symptomatic and develop anaemia, thrombocytopenia and immunodeficiency.
88
Explain the blast phase of CML
- Involves an even higher proportion (over 20%) of blast cells in the blood.
- Severe symptoms and pancytopenia and is often fatal.
89
What is CML particularly associated with ?
- Philadelphia chromosome
- Abnormal chromosome 22
- Caused by reciprocal translocation of a section of chromosome 9 and 22.
- BCR-ABL1 = abnormal tyrosine kinase enzyme
90
what is now considered first line treatment of CML
Imatinib -> inhibits tyrosine kinase
91
What are the characteristic findings on blood film and bone marrow biopsy in AML ?
- High proportion of blast cells
- Aure rods
92
What are the 4 U&E findings characteristic of tumour lysis syndrome
- High uric acid
- Hyperkalaemia
- High phosphate
- Low calcium
93
What can the high uric acid and hyperkalaemia seen in tumiur lysis syndrome cause ?
- Uric acid -> AKI
- Hyperkalaemia -> cardiac arrhythmias
94
What is the usual presentation of acute promyelocytic leukaemia
- Younger patient (around 25) presenting with DIC
95
what is lymphoma ?
- A type of cancer affecting the lymphocytes inside the lymphatic system.
- They proliferate inside the lymph nodes causing lymphadenopathy
96
What age group is affected by Hodgkin's lymphoma ?
- Bimodal age distribution (20-25 and 80 yrs)
97
Give 4 RF for Hodgkin's lymphoma
- HIV
- Epstein-Barr virus
- Autoimmune conditions, such as rheumatoid arthritis and sarcoidosis
- Family history
98
what is the main feature of Hodgkin's lymphoma ?
- Lymphadenopathy, most commonly cervical
- Painless, non tender and asymmetrical
- Alcohol-induced lymph node pain
99
What B symptoms are seen in Hodgkin's lymphoma
weight loss
pruritus
night sweats
fever
100
what is the diagnostic investigation for lymphoma and what is seen in Hodgkin's lymphoma ?
Lymph node biopsy
Reed-Sternberg cells
Large multinucleated cells with prominent eosinophilic nucleoli
101
What is the treatment of Hodgkin's lymphoma ?
- Chemo and radiotherapy
102
What is the new staging system used for lymphoma ?
Lugano classification
- Stage 1: Confined to one node or group of nodes
- Stage 2: In more than one group of nodes but on the same side of the diaphragm (either above or below)
- Stage 3: Affects lymph nodes both above and below the diaphragm
- Stage 4: Widespread involvement, including non-lymphatic organs, such as the lungs or liver
103
Give 7 RF for Non-Hodgkin Lymphoma
- HIV
- EBV
- H. pylori -> MALT lymphoma
- Hep B or C
- Exposure to pesticides
- Exposure to trichloroethylene
- FHx
104
What is associated with Burkitt's lymphoma and what is seen on microscopy
- EBV
- 'Starry sky' appearance on lymph node biopsy
105
what is associated with gastric MALT lymphoma and what is a common feature ?
- H. pylori
- Paraproteinaemia
106
What is often seen on routine bloods in lymphoma ?
- Normocytic anaemia
- Eosinophilia
- Raised LDH
107
What is essential for the absoprtion of B12 ?
- Intrinsic factor produced by the parietal cells of the stomach
108
What is pernicious anaemia ?
- Autoimmune condition where antibodies are produced against parietal cells or intrisic factor (1st line Ix).
- lack of B12 = macrocytic anaemia
109
What 5 specific signs can be seen in pernicious anaemia ?
- Peripheral neuropathy
- Loss of vibration sense
- Loss of proprioception
- Visual changes
- Mood and cognitive changes
110
What is given initially for the management of B12 deficiency
- IM hydroxycobalamin
- No neurological Sx : 3x weekly for 2 wks
- Neurological Sx : alternate days until no further improvement in Sx
111
How is control of B12 deficiency maintained ?
- Pernicious anaemia : 2-3 mnthly injections for life
- Diet related : oral cyanocobalamin or twice-yearly injections
112
What can happen if folate is given before treating B12 deficiency if a deficiency in both is present ?
Subacute combined degeneration of the cord
113
What is myeloma ?
Malignancy of the plasma cells in the bone marrow
114
Define multiple myeloma, MGUS and smouldering myeloma
- Multiple myeloma : multiple bone marrow areas affected.
- Monoclonal gammopathy of undetermined significance : production of paraprotein without other features of myeloma or cancer.
- Smouldering : abnormal plasma cells & paraproteins but no Sx.
115
What are the 5 feaures of myeloma ?
CRABBI
- C : Calcium (elevated)
- R : Renal damage
- A : Anaemia
- B : Bleeding (thrombocytopenia )
- B : Bones : lytic bone lesions
- I : Infection
116
What is seen on bloods in myeloma ?
- FBC : anaemia
- Blood film : rouleaux formation
- U&Es : renal failure
- Bone profile : hypercalcaemia
117
What would be seen on protein electrophoresis in myeloma ?
- Serum : paraproteinaemia
- Urine : Bence Jones in the urine
118
what is used to confirm the diagnosis of myeloma ?
Bone marrow biopsy -> shows raised plasma cells
119
What imaging is preferred for assessing bone lesions in myeloma ?
- Whole-body MRI
- Will show 'punched out' lesions, diffuse osteopenia and abnormal fracures
- In the skull : 'raindrop/pepper pot skull'
120
What is the diagnostic criteria of myeloma
- One major and one minor or 3 minor
- Major : Plasmacytoma,
30% plasma cells in a bone marrow sample, Elevated levels of M protein in the blood or urine.
- Minor : 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,
Low levels of antibodies (not produced by the cancer cells) in the blood.
121
what is a myeloproliferative disorder and give 3 examples
Uncontrolled proliferation. of a single line of stem cells.
- Primary myelofibrosis
- Polycythaemia vera
- Essential thrombocythaemia
122
what mutation is thought to be associated with myeloproliferative disorders
JAK2
123
what is myelofibrosis ?
- When proliferation of a single line of stem cells leads to bone marrow fibrosis.
- Can be : primary myelofibrosis, polycythaemia vera or essential thrombocytopenia
124
What would be seen on bloods in myelofibrosis ?
Anaemia
Leukopenia
Thrombocytopenia
125
What happens to the liver and spleen in myelofibrosis
- They start producing RBC's = hepatomegaly and splenomegaly
- If it occurs in the spine = spinal cord compression
126
What is seen on a blood film in myelofibrosis ?
- Tear drop RBCs
- Anisocytosis
- Blasts
127
What is seen on bloods in essential thrombocythaemia
Isolated raised plts
128
What is seen in polycythaemia vera ?
High haemoglobin
129
what is used to diagnose a myeloproliferative disorder and what would be seen in myelofibrosis ?
- Bone marrow biopsy
- Bone marrow aspiration might be 'dry' with myelofibrosis
130
what clinical signs are seen in polycythaemia ?
- Ruddy complexion (red face)
- Conjunctival plethora
- Splenomegaly
- Hypertension
- Pruitus
131
What is a common complication of polycythaemia vera and essential thromboythaemia
- Thrombosis
= MI, stroke or DVT/PE
132
How is polycythaemia vera managed ?
- Venesection t
- Aspirin to reduce the risk of thrombus formation
- Chemotherapy (typically hydroxycarbamide) to help control the disease
133
How is essential thrombocythaemia managed ?
- Aspirin to reduce the risk of thrombus formation
Chemotherapy (typically hydroxycarbamide) t
- Anagrelide is a specialist platelet-lowering agent
134
Myelodysplastic syndrome involves a mutation in which cells in the bone marrow ?
Myeloid cells
135
What does myelodysplastic syndrome result in and what can it transform in to?
- Ineffective haematopoiesis = lack of RBCs
- Can transform to AML
136
As it effects the myeloid cell line what is seen on bloods in myelodysplastic syndrome ?
Pancytopenia (low RBCs, neutrophils and platelets).
137
How does moderate thrombocytopenia present ?
Nosebleeds
Bleeding gums
Heavy periods
Easy bruising
Haematuria (blood in the urine)
Rectal bleeding
138
How does severe thrombocytopenia present ?
- Intracranial haemorrhage
- Gastrointestinal bleeding
139
Give 4 top differentials for prolonged and abnormal bleeding
- Thrombocytopenia (TTP, ITP, heparin induced)
- Von Willebrand disease
- Haemophilia A and B
- DIC
140
What is thrombotic thrombocytopenia purpura and what does it cause ?
- Thrombi develop in the small vessels using up the plts
- Thrombocytopenia
- Purpura
- Tissue ischaemia and end-organ damage
141
What monoclonal antibody can be used in the management of ITP and TTP ?
- Rituximab : it targets B cells which produce antbodies
142
How long after starting treatment with heparin can heparin-induced thrombocytopenia develop ?
- 5 to 10 days
- They develop both. alow plt count and abnormal blood clots
143
what is the most common inherited bleeding disorder and in what fashion is it inherited ?
- Von Willebrand disease
- Autosomal dominant
144
what are the 3 types of VWD ?
- Type 1 : partial deficiency of VWF and is the most common and mildest type
Type 2 : reduced function of VWF
Type 3 : complete deficiency of VWF and is the most rare and severe type
145
How does VWD present ?
- Bleeding gums with brushing
- Epistaxis
- Easy bruising
- Menorrhagia
- Heavy bleeding during and after surgical operations
Often a family history !
146
when necessary what can be used to manage VWD
- Desmopressin (stimulates the release of vWF from endothelial cells)
- Tranexamic acid
- Von Willebrand factor infusion
- Factor VIII plus von Willebrand factor infusion
147
What are the causes of haemophilia A and B and their pattern of inheritance ?
- A = factor VIII deficiency
- B = factor IX deficienct
- X linked recessive
148
what is seen on coagulation studies in haemophilia ?
Isolated prolonged APTT
149
How does a DVT present ?
- Calf or leg swelling (>3cm is significant)
- Dilated superficial veins
- Tenderness to the calf (particularly over the site of the deep veins)
- Oedema
- Colour changes to the leg
150
what determines the investigations done into a DVT?
- 2 level DVT Well's score
- Likely (2+) : proximal leg vein USS within 4 hrs.
- Unlikely (1) : d-dimer, followed by USS if +ve.
151
Is D-dimer sensitive or specific ?
- Sensitive.
- Almost always raised if DVT/PE present
- But can ve raised due to other conditions
152
what should be done if a patient likely to have a DVT but an USS cannot be done within 4 hrs ?
- Interim therapeutic anticoagulation
- Dalterparin : check weight and eGFR
- 200 units/kg daily by sub cut injection
153
how is a DVT managed ?
- Doac (apixaban / rivaroxaban)
154
when is LMWH given instead of a DOAC for a DVT?
- Renal impairment (creatinine clearance of <15ml/min)
- Antiphospholipid syndrome
- Pregnancy
155
How long is anticoagulation continued following a DVT?
- 3 months with a reversible cause (then review)
- 3-6 months in active cancer (then review)
- Long-term for unprovoked VTE, recurrent VTE or an irreversible underlying cause (e.g., thrombophilia)
156
what is the first line management of a DVT in someone with ant-phospholipid syndrome and a pregnant woman
- Anti-phospholipid syndrome : warfarin
- Pregnancy : LMWH
157
What is Budd-Chiari syndrome and the triad of features ?
- Hepatic vein thrombosis
- Abdo pain : sudden and severe
- Ascites
- Tender hepatomegaly
158
Give 4 causes of Budd-Chiari syndrome
- Polycythaemia rubra vera
- Thrombophilia: activated protein C resistance, antithrombin III deficiency, protein C & S deficiencies
- Pregnancy
- Combined oral contraceptive pill
159
What clotting factors does heparin prevent the action of ?
2,9,10,11
160
What factors does warfarin affect synthesis of due to action on vit K
10,9,7,2
161
what would be seen on blood clotting tests results in VWD?
Increased APTT and bleeding time but normal PT
162
What would be seen on blood clotting test results in vitamin K deficiency ?
Increased APTT and PT but normal bleeding time
163
How is anti-phospholipid syndrome managed in pregnancy ?
- Low-dose aspirin should once the pregnancy is confirmed on urine testing
- Low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks gestation
164
What does cryoprecipitate contain ?
- Factor VIII
- Fibrinogen
- Von Willebrand factor
- Factor XIII
165
What is a typical blood picture seen in DIC ?
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia
166
What is the inheritance of fanconi anaemia ?
Autosomal recessive
167
What are the features of fanconi anaemia ?
- Haematological: aplastic anaemia,
increased risk of AML
neurological
- Skeletal abnormalities: short stature, thumb/radius abnormalities
- Cafe au lait spot
168
Pt has a likely DVT based on well's but leg scan is negative - what do you do >
- If D dimer is +ve, re scan in 6-8v days
- If D dimer is -ve, consder alt diagnosis
169
Most common inherited thrombophilia creating PE/DVT risk
Factor V leiden = activated protein C resistance
170
Manufactured version of B12, used to treat B!2 deficiency
Hydroxyocobalamin
171
Transfusion threshold in pts without ACS
70g/l
172
Transfusion threshold in pts with ACS
80g/l
173
Management of neutropenic sepsis
Piperacillin with tazobactam (Tazocin)
174
What are sickle cell patients started on long term to reduce incidence of complications and acute crises
Hydroxycarbamide
175
What medications should ppl receiving transfusions for beta thalassaemia also be taking
Desforrioxamine = prevent iron overload due to repeat transfusions
176
what are the laboratory requirements for blood in a pt with previous transplant
Irradiated blood products are used to avoid transfusion-associated graft versus host disease
177
Clotting in antiphospholipid syndrome
- Raised APTT
- Normal PT
- Possible thrombocytopenia
178
Presentation of transfusion associated circulatory overload
Hypertension
Raised jugular venous pulse
Afebrile
S3 present.
179
Management of TACO
Furosemide
180
Presentation of transfusion related acute lung injury
Hypotension
Pyrexia
Normal/unchanged JVP
181
Management of what chronic condition can lead to B12 deficiency and why
- LLeocaecal resection in crohns disease
- B12 is absorbed in the terminal ileum
182
Most concerning sign when assessing possible blood transfusion reaction
Hypotension
183
Management of co existing B12 and folate deficiency
1mg cyanocobalamin IM injections 3x weekly
Treat B12 deficiency first to avoid subacute combined degeneration of spinal cord
184
Cause of macrocytic anaemia with hyper-segmented neutrophil polymorphs on blood film
- B12 deficiency (vegan = RF)
- Folate deficiency (methotrexate = possible cause).
185
When should neutropenic sepsis be suspected whilst awaiting blood results and what should be done
- In a person with a known cause for neutropenia
- Presumed or confirmed infection
- Temperature >38ºC
- Respiratory rate >20 breaths per minute
(Begin immediate empirical antibiotic therapy before receiving blood results).
186
what can be seen on a blood film in coeliac disease and why
Howell-jolly bodies : coeliac disease is associated with hyposplenism.
187
Common presentation of CML in a question
Splenomegaly
Anaemia
Thrombocytosis
Raised neutrophils
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how can alpha and beta thalassaemia be distinguished ?
- Beat thalassamiea : raised HbaA2 on electrophoresis
AND
Disproportionate microcytic anaemia (I.e - low Hb but MUCH lower MCV).
189
Most common causative agents opf neutropenic sepsis
- Coagulase-negative, Gram-positive bacteria
- Particularly staphylococcus epidermidis = probably due to the use of indwelling lines in patients with cancer
190
Bloods and blood film seen in CML
- Blood film = myeloblasts and granulocytosis
- Bloods : anaemia, thrombocytosis, raised neutrophils
191
What can lead to gastric lymphoma (MALT)
H.pylori
192
How can you distinguish an aplastic crisis from an haemolytic crisis / acute splenic sequestration in a sickle cell crisis
- Aplastic crisis = there will be. drop in Hb but ALSO a drop in reticulocytes as the issue is with the bone marrow
- Haemolytic / splenic sequestration = rise in reticulocyte count as the issue is RBC survival NOT production
193
Management of ACS
1. Analgesia
2. Oxygen
3. Antibiotics
4. Transfusion (if indicated by Hb)
194
common presentation of cold AIHA
- In hospital with mycoplasma pneumonia (common cause)
- Develops palpitations, pale skin, dark urine (haemolysis)
- Bloods : drop in Hb, raised ESR and reticulocytes)
195
Mechanism of action of rivaroxaban
Direct factor Xa inhibitor
196
Mechanism of action of dabigatran
Direct thrombin inhibitor
197
Action of heparin
Activates anti-thrombin III
198
Action of warfarin
Inhibitors clotting factors 10, 9, 7, 2
199
How should a vaso-occlusive crisis be diagnosed ?
CLINICALLY
No additional testing is needed
200
what can cause secondary polycythaemia ?
- OSA
- COPD
201
How can you distinguish secondary from primary polycythaemia ?
- In secondary polycythaemia the SpO2 / EPO won't be normal as it is reactive
202
How to distinguish cold and warm AIHA in an exam question
Warm autoimmune haemolytic anaemia is associated with CLL
203
Blood film suggestive of hyposplenism
Howell-Jolly bodies and siderocytes
204
Reversal of dabigatran
Idarucizumab
205
5 features of post thrombotic syndrome
- painful, heavy calves
- pruritus
- swelling
- varicose veins
- venous ulceration
206
what are irradiated blood products ?
Depleted in T lymphocytes to reduce risk of graft versus host disease
207
what is given prior to surgery in a pt with VWD to reduce bleeding whilst awaiting theatre ?
Desmopressin
208
treatment of acute attacks of intermittent porphyria
Haem arginate
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