Haem Flashcards
1
Q
What is one key cause of recurrent VTE?
A
Antiphospholipid syndrome
2
Q
How do DVTs present?
A
- Calf or leg swelling
- Dilated superficial veins
- Tenderness to the calf
- Oedema
- Colour changes to the leg
3
Q
What is D-dimer useful for?
A
Excluding DVT if there is a low suspicion
4
Q
What investigation is required to diagnose DVT?
A
Doppler USS
5
Q
When would you repeat a negative Doppler USS for DVT?
A
- If a positive D-dimer and Well score suggesting DVT is likely
- Repeat 6-8 days later
6
Q
What is the acute management of DVT?
A
- Apixaban/rivaroxaban
- LMWH is the alternative
- Consider cather-directed thrombolysis
7
Q
Long term anticoagulation for VTE:
1. First line?
2. First line in pts with anti phospholipid syndrome?
3. First line in pregnancy?
A
- DOAC
- Warfarin
- LMWH
8
Q
What is Budd-Chiari syndrome? How does it present?
A
Where a blood clot develops in the hepatic vein, blocking the outflow of blood. Causes acute hepatitis
Triad of;
- Abdo pain
- Hepatomegaly
- Ascites
9
Q
What is the pathophysiology of myeloma?
A
- Myeloma is cancer of plasma cells
- Plasma cells are a type of B cell (they are found in bone marrow)
- Plasma cells produce immunoglobulins
- In myeloma a specific type of plasma cell will be affected meaning one type of immunoglobulin will be affected
- If you measure the immunoglobulins of a patient with myeloma, one will be significantly raised (normally IgG)
10
Q
What are the features of myeloma?
A
Old CRAB
- Old age
- Calcium elevated
- Renal failure
- Anaemia
- Bone lesions
11
Q
Why is anaemia a feature of myeloma?
A
Cancerous plasma cells invade bone marrow causing suppression of the development of other blood cells e.g. red blood cells, neutrophils and platelets
12
Q
What are risk factors for myeloma?
A
- Older age
- Male
- Black African ethnicity
- FHx
- Obesity
13
Q
What patients complaints should make you consider myeloma?
A
> 60 yrs
- Persistent bone pain
- Persistent back pain
- Unexplained fractures
14
Q
What are initial investigations for suspected myeloma?
A
- FBC
- Calcium
- ESR
- Plasma viscosity
15
Q
Why is plasma viscosity increased in myeloma? What does this cause?
A
- Plasma viscosity increases when there are more proteins in the blood
- Myeloma results in raised immunoglobulins, immunoglobulins are proteins
Increased plasma viscosity causes:
- Easy bruising
- Easy bleeding
- Vascular disease in eye -> reduced vision
- Purple discolouration to the extremities
16
Q
How do you test for myeloma? What is the diagnostic test?
A
BLIP
- Bence-Jones protein (urine electrophoresis)
- serum-free Light-chain assay
- serum Immunoglobulins
- serum Protein electrophoresis
Bone marrow biopsy is diagnostic
17
Q
What are XR features of myeloma bone disease?
A
- Lytic lesions
- Punched out lesions
- ‘Raindrop skull’
18
Q
What is first line treatment of myeloma?
A
Combination chemotherapy
19
Q
What is the management of myeloma bone disease?
A
- Bisphosphonates to suppress osteoclast activity
- Radiotherapy to bone lesions to improve bone pain
- Orthopaedic surgery to stabilise bones/treat fractures
20
Q
What is the pathophysiology of lymphoma?
A
- Cancers that affect lymphocytes in the lymphatic system
- Cancerous cells proliferate within lymph nodes -> lymphadenopathy
21
Q
What age is Hodgkin’s lymphoma more prevalent?
A
- Bimodal age distribution
- Peaks at 20 years and 75 years
22
Q
What are risk factors for Hodgkin’s lymphoma?
A
- HIV
- EBV
- Autoimmune conditions e.g. RA, sarcoidosis
- FHx
23
Q
How does lymphoma present?
A
- Non-tender lymphadenopathy
- Pain in the lymph nodes when patients drink alcohol (Hodgkin lymphoma)
- B symptoms
24
Q
What is the diagnostic test for lymphoma? What is the key finding?
A
Lymph node biopsy showing Reed-Sternberg cells
25
What staging system is used for both Hodgkins and non-Hodgkins lymphoma?
Ann Arbor staging:
- Stage 1 - affected lymph nodes confined to one region
- Stage 2 - lymph nodes in more than one region but on the same side of the diaphragm
- Stage 3 - lymph nodes on both sides of diaphragm
- Stage 4 - widespread involvement including non-lymphatic organs (e.g. lungs/liver)
26
What is non-hodgkin lymphoma? Give 3 types?
A group of lymphomas
3 notable ones:
- Burkitt lymphoma - associated with EBV
- MALT lymphoma - associated with H pylori
- Diffuse large B cell lymphoma - older age
27
What is the most severe type of malaria?
Disease caused by plasmodium falciparum protozoa
28
How does malaria present?
- Fevers, sweats and rigors
- Vomiting
- Headache
- Pallor due to anaemia
- Hepatosplenomegaly
- Jaundice
29
How is malaria diagnosed?
- Malaria blood film
- Can be excluded after 3 -ve samples sent over 3 consecutive days
30
What is the first line treatment of uncomplicated malaria?
Artemether with lumefantrine
31
What is the treatment of severe malaria?
- IV artesunate
- IV quinine dihydrochloride
32
What is leukaemia a cancer of?
A particular line of stem cells in the bone marrow -> myeloid or lymphoid
33
What is the affect of leukaemia on other blood cells in the bone marrow?
Pancytopenia due to excessive production of a single type of cell
- Anaemia
- Leukopenia
- Thrombocytopenia
34
What age groups correlate with the different types of leukaemia?
ALL CeLLmates have CoMon AMbitions
Under 5 and over 45 - ALL
Over 55 - CLL
Over 65 - CML
Over 75 - AML
35
How does leukaemia present?
- Non specific - fever, fatigue
- Signs/symptoms related to pancytopenia
36
What is the diagnostic test for leukaemia?
Bone marrow biopsy
37
What type of leukaemia can transform to high grade lymphoma? What is this called?
CLL
38
What type of leukaemia shows smear or smudge cells on blood film?
CLL
39
Which types of leukaemia are associated with the Philadelphia chromosome?
- ALL
- CML
40
What is the most common type of leukaemia in adults?
CLL
41
What will a blood film show in AML?
Blast cells with Auer rods in their cytoplasm
42
What are the causes of microcytic anaemia?
TAILS
- Thalassaemia
- Anaemia of chronic disease
- Iron deficiency anaemia
- Lead poisoning
- Sideroblastic anaemia
43
What are the causes of normocyctic anaemia?
3 As and 2 Hs
- Acute blood loss
- Anaemia of chronic disease
- Aplastic anaemia
- Haemolytic anaemia
- Hypothyroidism
44
What are the causes of macrocytic anaemia?
Megaloblastic
- B12 deficiency
- Folate deficiency
Normoblastic
- Alcohol
- Reticulocytosis
- Hypothyroidims
- Liver disease
- Drugs - azathioprine
45
Give 2 symptoms that are specific to anaemia
- Pica - iron def anaemia
- Hair loss - iron def anaemia
46
What examination findings would suggest anaemia?
- Pale skin
- Conjunctival pallor
- Tachycardia
- Raised resp rate
- Koilonychia - iron def
- Atrophic glossitis - iron def
- Angular chelitis - iron def
47
When someone presents with iron def anaemia, what causes should you consider?
- Reduced dietary intake
- Menorrhagia
- Pregnancy
- GI tract cancer
- IBD
48
What blood results would indicate iron deficiency anaemia?
- Low serum ferritin
- Raised total iron binding capacity
- Raised transferrin
49
What is first line management of iron deficiency anaemia?
Oral iron -> ferrous sulphate 200mg TDS
50
What are side effects of taking oral iron?
- Constipation
- Black stools
51
What happens in pernicious anaemia?
- Autoimmune condition
- Antibodies form against intrinsic factors which is essential for the absorption of vitamin B12
- Results in B12 deficiency
52
How can B12 deficiency present?
- Peripheral neuropathy
- Loss of vibration and proprioception
- Visual changes
- Mood/cognitive changes
53
What is first line for diagnosis of pernicious anaemia?
Intrinsic factor antibody
54
How do you manage pernicious anaemia?
1mg of IM hydroxycobalamin 3 times weekly for 2 weeks then every 3 months
55
How do you manage a patient that has folate and B12 deficiency?
- Treat B12 deficiency first
- Treating patients with folic acid when they have B12 deficiency can lead to combined degeneration of the cord
56
What are features of thalassaemia?
- Microcytic anaemia - RBC are more fragile
- Splenomegaly - spleen collects destroyed RBCs -> splenomegaly
- Fatigue
- Pallor
- Jaundice
- Poor growth and development
57
What are the two types of thalassaemia?
- Alpha - defects in alpha globin chains
- Beta - defects in beta globin chains, thalassaemia minor/intermedia/major
58
What is the management of thalassaemia?
- Monitoring
- Blood transfusions
- Iron chelation
- Splenectomy
- Bone marrow transplant
59
Why can iron overload occur in thalassaemia? How is this monitored? What is the management?
- Increased iron absorption in the GI tract
- Blood transfusions
Serum ferritin levels are monitored
Rx: limiting transfusions, iron chelation
60
What are myeloproliferative disorders?
Slow growing blood cancers that involve the uncontrolled proliferation of a single type of stem cell
61
What are examples of myeloproliferative disorders?
- Primary myelofibrosis
- Polycythaemia vera
- Essential thrombocythaemia
62
What do myeloproliferative disorders have a potential to transform into?
Acute myeloid leukaemia
63
What genes are commonly affected in myeloproliferative disorders?
- JAK2
- MPL
- CALR
64
How are myeloproliferative disorders diagnosed?
- Bone marrow biopsy
- Testing for genes
65
What is PCV?
A myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets
66
What are features of PCV?
- Pruritis (particularly after hot bath)
- Ruddy complexion (red face)
- Conjunctival plethora (opp of conjunctival pallor)
- Splenomegaly
- HTN
- Hyper-viscosity - thrombosis
- Haemorrhage (due to abnormal platelet function)
67
What is the management of PCV?
- Aspirin (due to increased thrombosis risk)
- Venesection (to keep Hb in normal range)
- Chemo
68
Myelofibrosis:
1) What is it?
2) Causes?
3) Complications?
4) What would blood films show?
1. Where the proliferation of a single cell line -> bone marrow fibrosis (bm replaced w scar tissue)
2. Primary myelofibrosis/PCV/essential thrombocythaemia
3. Extramedullary haematopoiesis
4. Teardrop shaped RBCs, anisocytosis (varying sizes of RBCs), blasts
69
What is extramedullary haematopoiesis?
- When the bone marrow is replaced with scar tissue, the production of blood cells happens in other areas e.g. liver/spleen -> hepato/splenomegaly
- When it occurs in the spine -> spinal cord compression
70
What is myelodysplastic syndrome?
A type of cancer caused by a mutation in the myeloid cells in the bone marrow -> inadequate production of blood cells (ineffective haematopoiesis). Results in:
- Anaemia
- Neutropenia
- Thrombocytopenia
71
What does myelodysplastic syndrome have the potential to transform into?
Acute myeloid leukaemia
72
What are the top differentials for abnormal or prolonged bleeding?
- Haemophilia
- Thrombocytopenia
- Von Wilebrand disease
- Disseminated intravascular coagulation (usually secondary to sepsis)
73
What is haemophilia?
X-linked recessive bleeding disorder
74
What are the two types of haemophilia? What are their causes?
- Haemophilia A - factor VIII deficiency
- Haemophilia B - factor IX deficiency
Haemophilia B is also called Christmas disease
75
How does haemophilia present?
- Most cases present in neonates/early childhood - intracranial haemorrhage/haematomas
- Spontaneous bleeding - oral mucosa, nosebleeds, GI/urinary tract
- Excessive bleeding in response to mild trauma
- Bleeding into joints -> haemarthrosis
- Bleeding into muscles -> compartment syndrome
76
What is the management of haemophilia?
- IV infusion of the affected clotting factors - regularly/in response to bleeding
- Complication = formation of antibodies against tx -> becomes ineffective
77
What is Immune Thrombocytopenia Purpura?
Autoimmune condition where antibodies are created against platelets
78
How does ITP present?
Characteristically presents with purpura
79
What are management options for ITP?
- Prednisolone
- IV immunoglobulins
- Thrombopoietin receptor agonists
- Rituximab
- Splenectomy
80
What is the role of Rituximab in the management of ITP?
- Rituximab is a monoclonal antibodies that targets CD20 proteins on the surface of B cells
- This reduces the number of B cells (which normally produce antibodies)
- Rituximab is used for many autoimmune conditions e.g. RA, ITP
81
What is Thrombotic Thrombocytopenia Purpura (TTP)?
A condition where tiny thrombi develop throughout the small vessels, using up platelets. Thrombi develop due to a problem with a protein called ADAMTS13
82
What does ADAMTS13 protein normally do?
- Inactivates von Willebrand factor
- Reduces platelet adhesion to vessel walls
- Reduces clot formation
83
What can cause ADAMTS13 protein deficiency?
- An inherited genetic mutation
- Autoimmune disease (antibodies are created against the protein)
84
What are features of TTP?
- Thrombocytopenia
- Purpura
- Tissue ischaemia and end-organ damage
85
What are management options for TTP?
- Plasma exchange
- Steroids
- Rituximab
86
What is HIT?
- Heparin-induced thrombocytopenia
- The development of antibodies against platelets in response to heparin
- Heparin-induced antibodies target a protein on platelets called platelet factor 4
87
What are the features of HIT?
- Presents 5-10 days after starting treatment with heparin
- HIT antibodies bind to platelets and activate the clotting system -> hypercoagulable state and thrombosis
- They also break down platelets -> thrombocytopenia
Overall presents with a counterintuitive situation where pts have a low platelet count and develop abnormal clots
88
How is HIT diagnosed?
Testing for HIT antibodies on blood sample
89
What is the management of HIT?
Stop heparin and use alternative anticoagulant
90
What is haemochromatosis?
An autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation
91
What gene is affected in haemochromatosis?
The HFE gene on chromosome 6
92
How does haemochromatosis present?
- Often asymptomatic in early disease
- Early symptoms - fatigue, erectile dysfunction, arthralgia (often of hands)
- Bronze skin pigmentation
- Testicular atrophy
- Amenorrhoea
- Cognitive symptoms - memory/mood disturbances
93
Why can haemochromatosis present later in females?
Mensuration regularly eliminates iron from the body
94
What are complications of haemochromatosis?
- DM (iron affects functioning of the pancreas)
- Stigmata of chronic liver disease, hepatomegaly, cirrhosis
- Cardiac failure (iron deposits in heart -> cardiomyopathy)
- Hypogonadism (iron deposits in the liver)
- Arthritis (especially of the hands)
95
How do you screen for haemochromatosis?
- Transferrin saturation - considered the most useful marker
- Ferritin - should also be measured but is not usually abnormal in the early stages of iron accumulation
96
What is the management of haemochromatosis?
- Venesection (first-line) - transferrin saturation should be kept < 50% and the serum ferritin concentration < 50 ug/l
- Desferrioxamine (second-line)
- Monitoring and treating complications
97
How can blood product transfusion complications be classified?
- Immunological - acute haemolytic, non-haemolytic febrile, allergic
- Infective
- Transfusion-related acute lung injury (TRALI)
- Transfusion-associated circulatory overload (TACO)
98
Acute haemolytic transfusion reaction:
1) What is the cause?
2) How does it present?
3) What is the management?
1. A mismatch of blood group which causes massive intravascular haemolysis
2. Fever, abdo and chest pain, agitation, hypotension, symptom onset mins after transfusion
3. Immediate transfusion termination, fluid resus, send blood for direct Coombs test, repeat cross match
99
What is transfusion-related acute lung injury?
- Rare complication of blood transfusion
- Characterised by development of hypoxaemia / acute respiratory distress syndrome within 6 hours of transfusion
- Features = hypoxia, fever, hypotension, chest infiltrates on CXR
100
What is transfusion-associated circulatory overload?
- Common reaction to fluid overload resulting in pulmonary oedema
- Features = pulmonary oedema + hypertension
