Haematological Conditions Flashcards
1
Q
What is polycythaemia?
A
=increased haemoglobin, PCV and red blood cell count
2
Q
What is is PCV?
A
Packed Cell Volume = proportion of blood made up from red blood cells
3
Q
What is primary polycythaemia?
A
Increase in red blood cells is due to a problem with red blood cell production
4
Q
What are the causes of primary polycythaemia?
A
- genetic mutation in EPO receptor (EPO triggers RBC production)
- mutation in hypoxia-sensing pathway (hypoxia triggers RBC production)
5
Q
What are the symptoms of polycythaemia?
A
- tiredness
- itching
- vertigo
- headache
- visual disturbance
6
Q
What are the complications of polycythaemia?
A
- thrombosis
- haemorrhage
- gout
- peptic ulceration
7
Q
How is polycythaemia diagnoses?
A
- measure haemoglobin
- investigate presence of genetic mutations
8
Q
How is polycythaemia managed?
A
- chemotherapy
- low dose aspirin (reduce risk of thrombosis)
- venesections (regular removal of blood)
- anagrelide (inhibits megakaryocyte differentiation)
9
Q
What is secondary polycythaemia?
A
increase in RBC due to an underlying condition
10
Q
What are the causes of secondary polycythaemia?
A
appropriate increase in EPO -high altitude -chronic lung disease -cardiovascular disease -smoking inappropriate increase in EPO -renal disease -adrenal tumour -hepatocellular carcinoma
11
Q
What is relative polycythaemia?
A
decrease in plasma volume causes a relatively high proportion of RBC/haematocrit
12
Q
What are the causes of relative polycythaemia?
A
- smoking
- high alcohol
- obesity
- high blood pressure
13
Q
What are bleeding disorders? Give examples
A
Conditions affecting coagulation of blood
- thrombocytopenia
- thromobocytosis
- platelet function disorders
- Haemophilia
- Von Willebrand’s disease
14
Q
What is thrombocytopenia?
A
=low platelet number in the blood
15
Q
What are the causes of thrombocytopenia?
A
reduced platelet production -bone marrow failure -myeloma -drugs excessive peripheral platelet production -immune -auto-immune -drugs -post-transfusions sequestration into an enlarged spleen -splenomegaly -hypersplenism
16
Q
What are the symptoms of thrombocytopenia?
A
- excessive/easy bruising
- superficial bleeding into skin = purpura
- prolonged bleeding from cuts
- bleeding from gums/nose
- blood in urine or stools
17
Q
How is thrombocytopenia managed?
A
treat underlying condition
platelet transfusion
18
Q
What is immune thrombocytopenic purpura?
A
immune system destroys platelets, causing superficial bleeding under the skin
19
Q
How is immune thrombocytopenic purpura managed?
A
- corticosteroids
- IV immunoglobulins
- splenectomy
- platelet transfusion
20
Q
What is thrombotic thrombocytopenic purpura?
A
Clotting in small blood vessels results in low platelet count in the blood
21
Q
What are the symptoms of thrombotic thrombocytopenic purpura?
A
- florid purpura
- fever
- haemolytic anaemia
22
Q
How is thrombotic thrombocytopenic purpura managed?
A
- corticosteroid
- plasma exchange
23
Q
What are platelet function disorders?
A
inherited or acquired conditions where platelets are dysfunctional
24
Q
Give some examples of acquired platelet function disorder
A
- renal or liver disease
- drug use
- myeloproliferative disorders
25
Give some examples of congenital platelet function disorders
- glanzmann's thromboasthenia =abnormality in genes encoding for glycoproteins IIb/IIIa
- bernard-soullier syndrome =abnormality in genes for glycoprotein IIb/IIIa
26
What are the symptoms of platelet function disorders?
- easy bruising
- bleeding
- thrombosis
27
How are platelet function disorders managed?
- platelet transfusion
| - dialysis
28
What is thrombocytosis?
too many platelets in the blood
29
What are the causes of thrombocytosis?
- splenectomy
- malignancy
- inflammatory disorders
- major surgery
- myeloproliferative disorders
- iron deficiency
30
What are the symptoms of thrombocytosis?
- usually asymptomatic
- headaches
- weakness/numbness
31
How is thrombocytosis managed?
treat the underlying cause
32
What are the complications of thrombocytosis?
Increased risk of thrombosis formation, leading to increased risk of MI and stroke
33
What are inherited coagulation disorders?
=genetic condition which results in impaired coagulation
34
What are is haemophilia?
x-linked condition where there is a deficiency in a clotting factor
35
What is the difference between haemophilia A and B?
Haemophilia A factor VIII (8) is deficiency. Haemophilia B factor IX (9) is deficient
36
What are the symptoms of haemophilia?
- easy bruising
- excessive bleeding
- bleeding gums
37
How is haemophilia managed?
Replacement of the deficient clotting factor
38
What is Von Willebrand's disease?
deficiency in VWG causing platelet dysfunction
39
What are the symptoms of Von Willebrand's disease?
- excessive bleeding
- epistaxis (nose bleed due to damage of blood vessels in the nasal mucosa)
- menorrhagia (abnormally heavy periods)
40
How is Von Willebrand's disease managed?
- desmopressin (increases conc of VMWF and factor 8)
- tranexamic acid (prevents enzymes from breaking down blood clots)
- VWF concentrate
- avoid aspirin and anti-inflammatory drugs
- manage menorrhagia with contraception
41
What are acquired coagulation disorders?
conditions which are not genetic which cause impaired coagulation
42
Name 3 acquired coagulation disorder?
- vitamin k deficiency
- liver disease
- disseminated intravascular coagulation (DIC)
43
How does vitamin K deficiency impair coagulation
vitamin K is essential for the production of factors 2, 7, 9 ,10
44
What are the causes of vitamin K deficiency?
- inadequate storage
- malabsorption
- anticoagulants which act as vitamin K antagonisists
45
How does liver disease impair coagulation?
- reduced coagulation factor synthesis
- thrombocytopenia
- vitamin K deficiency
- functional abnormalities in platelets and fibrinogen
46
What is disseminated intravascular coagulation?
activation of the coagulation cascade results in the lots of small intravascular thrombi and a depletion in coagulation factors
47
What are the causes of disseminated intravascular coagulation?
- malignancy
- septicaemia
- haemolytic transfusion reaction
- liver disease
48
What are the symptoms of disseminated intravascular coagulation?
- acutely ill
- range from no bleeding to widespread haemorrhage depending on the severity
- thrombotic events
49
How is disseminated intravascular coagulation managed?
- treat the underlying cause
- platelet transfusion
- anti-coagulant e.g. heparin
50
What is lymphoma?
malignancy of the lymphoid tissue
51
What is the difference between Hodgkin's and non-Hodgkin's lymphoma?
Hodkin's lymphoma contains Reed-Sternberg cells which are not found in non-Hodgkin's lymphoma
52
What are the clinical feature of Hodgkin's lymphoma?
- painless cervical lymphadenopathy
- mediastinal lymphadenopathy
- hepato/splenoegaly
53
How is Hodgkin's lymphoma managed?
chemotherapy
54
What are the 2 types of non-Hodgkins lymphoma?
- B cell lymphoma (80%)
| - T cell lymphoma (20%)
55
What are the symptoms of B cell lymphoma?
painless lymphadenopathy at 1 site
56
How is B cell lymphoma managed?
- radiotherapy
| - chemo/immunotherapy
57
What are the symptoms of T cell lymphoma?
- inflammatory disease
- fever
- electrolyte abnormalities
58
How is T cell lymphoma managed?
chemotherapy
59
What is primary extranodal lymphoma?
malignancy of lymphoid tissue which originated outside the lymph nodes
60
What is Malaria?
parasitic infection caused by the Plasmodium parasite which is transmitted via female Anopheles mosquitos
61
What parasites cause malaria?
Plasmodium
- falciparum
- vivax
- ovale
- malariae
62
What is the vector for the malaria parasite?
Female Anopheles mosquito
63
Describe the parasitology of malaria.
- infected mosquito bites human
- sporozites transferred from the saliva into human blood
- sporoxites accumulate in the heaptocytes of the liver
- hepatocytes bursts and merorzoites are released into the blood
- merozoites invade RBC
- meorzoites replicate
- RBC burst and merozoites are released into the blood
- gamaetocytes alose prodcued in the RBC
- gametocytes taken up by a non-infected mosquito when bitten
64
Which species of Plasmodium have a latent period in malaria?
P. vivax and P.ovale.
| Some sporozites remain dormant in the liver and can reactivated to cause malaria long after infection
65
What are the clinical features of malaria?
- fever
- malaise
- headaches
- vomitting
- diarrhoea
66
What symptoms occur in severe complicated malaria and what species of parasite causes this?
Plasmodium falciparum
- impaired consciousness
- convulsions
- shock
- hypogylcaemia
- death
67
How is malaria diagnosed?
- blood films (thick and thin)
| - rapid diagnostic test (identifies antigen - useful when microscopy not available)
68
How is malaria treated?
- chloroquine (unless P. falciparum as resistance has developed)
- IV antimalarials (quinine and artesunate)
69
How is malaria prevented?
- reduce breeding sites (standing water)
- long-lasting insecticide treated nets
- insecticide spray
- chemoprophylaxis (medicine to people at risk)
70
What is viremia?
viral infection of the blood
71
What is fungemia?
fungal infection of the blood
72
What is septicaemia?
infection of the blood stream
73
What are the causes of septicaemia?
- bacteria infection enters the blood (bacteraemia)
| - UTI, lung infection, kidney infection, abdominal infection spreads
74
What are the clinical features of septicaemia?
- chills
- fever
- clammy pale skin
- hyperventilation
- tachycardia
- confusion
- nausea/vomiting
- dec urine volume
- inadequate blood flow (septic shock)
75
How is septicaemia diagnoses?
medical history of infection
| test body fluids
76
How is septicaemia managed?
- antibiotic (if bacterial)
- IV fluids
- oxygen
77
What is Sepsis?
life threatening organ dysfunction caused by dysregulated host response to infection
78
How many deaths occur due to sepsis in the UK per year?
>44,000
79
What are the clinical feature of mild and severe sepsis?
```
mild
RR: 21-24 (high)
BP: 91-100 (low)
HR: 91-130 (high)
severe
RR: >24
BP: <90
HR: >120
```
80
How is sepsis managed?
- assess risk using HR, BP, RR
- antibiotics
- IV fluids
- oxygen
- AKI
- blood tests
81
What is myeloma?
malignant disease of the bone marrow plasma cells
82
Explain the pathogenesis of myeloma.
- clonal expansion of abnormally proliferating plasma cells
- increased production of paraproteins (IgG/A)
- organ dysfunction
- bone destruction (due to infiltration with plasma cells)
83
What are the symptoms of myeloma?
- bone pain
- anemia symptoms (fatigue, faintness, headache, dyspnoea)
- recurrent infections
- renal failure symptoms (confusion, oedema, dec urine)
- hypercalcaemia symptoms (nausea, vomiting, loss of appetite, inc thirst, weakness, muscle pain, disorientation)
84
How is myeloma diagnosed?
- FBC (anaemia, neutropenia)
- increased Ca
- bone marrow biopsy (inc plasma cell)
- monoclonal protein band in serum from paraprotein
- bone lesions on x-ray
85
How is myeloma managed?
- analgesia for bone pain
- bisphosphates
- treat anaemia (EPO, blood transfusion)
- antibiotics for infection
- treat renal failure (fluid and dialysis)
- chemotherapy (may need stem cell transplant after)
86
What are the complications of myeloma?
- hypercalcaemia
- spinal cord compression
- hyperviscosity
- acute renal injury
87
What is leukaemia?
Cancer originating from blood-forming tissue normally in the bone marrow
88
What are the 4 common types of leukaemia?
Aute lymphoblastic
Acute myeloid
Chronic lymphocytic
Chronic myeloid
89
What is acute lymphoblastic leukaemia?
Malignancy of the lymphoid cells (B and T cells)
90
What is the clinical presentation of acute lymphoblastic leukaemia?
- bone marrow failure (anaemia, infection, bleeding)
| - tissue infiltration (hepatomegaly, splenomegaly, lymphadenopathy, bone pain)
91
How is acute lymphoblastic leukaemia treated?
- blood/platelet transfusion
- IV antibiotics for infections
- chemotherapy (multi-drug and multi-phase)
- bone marrow transplant
- subcutaneous port system of Hickman's line
92
Whats is acute myeloid leukaemia?
malignancy of myeloid cells (progenitors of granulocytes and monocytes -RBC, neutrophils etc)
93
What is the clinical presentation of acute myeloid leukaemia?
- marrow failure (anaemia, infection, bleeding)
| - tissue infiltration (hepatomegaly, splenomegaly, gum hypertrophy, bone pain)
94
How is acute myeloid leukaemia treated?
- blood/platelet transfusion
- chemotherapy (intensive and long)
- bone marrow transplant
- subcutaneous port or Hickmans line
95
What is chronic myeloid leykaemia?
slow developing malignancy of the myeloid cells (cells which form granulocytes and monocytes)
96
What is the clinical presentation of chronic myeloid leukaeia?
- weight loss
- tiredness
- fever
- marrow failure (anaemia, infection, bleeding)
- hepatomegaly
- splenoegaly
97
How is chronic myeloid leukaemia treated?
- imatinib (tyrosine kinase inhibitor)
- chemotherapy
- bone marrow transplant
98
What is chronic lymphocytic leukaemia?
slow developing malignancy of lymphoid cells (precursors of lymphocytes)
99
What is the clinical presentation of chronic lymphocytic leukaemia?
- lymphadenopathy
- weight loss
- fever
- infections
- anaemia
- splenomegaly
- hepatomegaly
100
How is chronic lymphocytic anaemia treated?
- platelet/blood transfusion
- radiation therapy
- chemotherapy
- stem cell transplant
- targeted therapy (monoclonal antibodies, tyrosine kinase inhibitors
101
What is thrombosis?
formation of a solid mass of blood constituents which forms in the circulation of a living organism
102
What is thromboembolism?
Obstruction of a blood vessel due to a thrombus breaking off from its original site and being transported to another part of the circulation where it gets stuck
103
How is a thrombus formed?
-damaged to the endothelium
-platelets adhere to the exposed collagen
-platelets are activated and release granules
platelets aggregate
-coagulation cascade activated
-fibrinogen converted to fibrin
-fibrin mesh forms
104
What the symptoms of a thrombus?
Aysmptomatic unless significantly reduces blood flow
- swelling
- redness
- pain
- throbbing
- ischaemia
105
What are the complications of thrombosis?
- myocardial infarction (chest pain, short of breath)
- stroke (weakness on one side, slurred speech)
- limb ischaemia (pain, discoloured, cold)
106
How is thrombosis managed?
- decrease risk factors (smoking, hyperlipidemia)
- anti-platelet drugs (aspirin)
- anti-coagulants (warfarin)
- thrombolytic therapy (medication to breakdown of thrombus e.g. streptokinase)
- emeboletomy (surgical removal of thrombus)
107
What is deep vein thrombosis (DVT)?
thrombus which form in a deep vein, commonly in the legs
108
What are the risk factors for DVT?
- surgery
| - hospitilisation
109
What are the clinical features of DVT?
- pain in calf
- swelling and redness
- engorged superficial veins
- ankle oedema
- cyanotic discolouration
110
How is DVT managed?
- anticoagulants (warfarin, heparin)
- mobilise (after anticoagulant given)
- wear elastic stocking
- thrombolytic therapy (streptokinase)
111
What are the risk factors for thrombosis?
- smoking
- hyperlipidemia
- hypertension
- diabetes mellitus
- lack of exercise
112
What is anaemia?
insufficient haemoglobin in the blood
113
Name 4 types of anaemia
- hypochromic microcytic
- normochromic normocytic
- macrocytic
- haemolytic
114
What is hypochromic microcytic anaemia?
RBC have a love Hb content and low MCV (small RBCS)
115
What are the causes of hypochromic microcytic anaemia?
- iron deficiency
| - anaemia of chronic disease (occurs alongside a chronic disease)
116
What is normochromic normocytic anaemia?
red blood cells have a normal MCV and Hb content
117
What is macrocytic anaemia?
RBC have a high MCV
118
What is megaloblastic anaemia?
large immature RBC synthesised in a small quantity
119
What is the cause of megaloblastic anaemia?
Impaired DNA synthesis commonly due to B12/folate deficiency
120
What is haemolytic anaemia?
increased destruction of RBC - may be due to an inherited, immune or auto-immune condition
121
What is sickle cell anaemia?
genetic mutation causes the production of abnormal red blood cells - results in impaired passage and reduced lifespan of RBC
122
How is sickle cell anaemia treated?
- blood transfusion
- stem cell transplant
- analgesia
- hydroxycarbamide?
123
What is sickle cell anaemia?
genetic mutation causes the production of abnormal red blood cells - results in impaired passage and reduced lifespan of RBC
124
How is sickle cell anaemia treated?
- blood transfusion
- stem cell transplant
- analgesia
- hydroxycarbamide (chemotherapy)
125
What is haemolytic disease of the newborn?
mother produces antibodies to the rhesus antigen on the fetal RBC which cross the placenta and causes lysis of the RBC
