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Haematology Flashcards

1
Q

What are the components of blood

A 
Plasma 
Cells - RBC, WBC, platelets 
Clotting factors - fibrinogen
Glucose
Electrolytes - sodium and potassium 
Protein - immunoglobulins and hormones
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2
Q

What are pluripotent haematopoietic stem cells

A

Undifferentiated cells that have the potential to become a variety of blood cells

  • Myeloid
  • Lymphoid
  • Dendritic cells
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3
Q

How do red blood cells develop

A

Reticulocytes from the myeloid stem cells

Survive up to 3 months

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4
Q

How do platelets form?

A

Megakaryocytes

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5
Q

How are white blood cells formed

A

Myeloid stem cells become promyelocytes that become monocytes, neutrophils, eosinophils, mast cells, basophils
Lymphocytes from lymphoid stem cells and become B or T cells
B lymphocytes become plasma cells or memory B cells
T lymphocytes become CD4/CD8/ Naturasl killer cells

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6
Q

What is anisocytosis and what does it mean

A

Variation in size of RBC

Myelodysplastic syndrome and anaemia

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7
Q

What are target cells and what do they signify

A

Central pigmented area surrounded by pale area surrounded by thick ring of cytoplasm

Iron defiency anaemia and post splenecotmy

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8
Q

What are Heinz bodies and where are they seen

A

Individual blobs inside RBC - denatured globin

G6PD and alpha thalassaemia

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9
Q

What are Howell Jolly bodies and where are they seen

A

Individual blobs of DNA material seen inside RBC, normally the DNA material is removed by the spleen during RBC circulation

Post splenectomy or in severe anaemia

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10
Q

What are reticulocytes and where are they seen

A

Immature RBC - slightly larger than standard RBC and have RNA material in them
Reticular mesh inside

Haemolytic anaemia

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11
Q

What are schistocytes and where are they found?

A

Fragments of RBC
Indicate RBC physically damaged by trauma during their journey through the blood vessels
Indicate clots in small vessels

HUS
DIC
TTP
Metallic heart valves
Haemolytic anaemia
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12
Q

What are sideroblasts and where are they found

A

Immature RBC with blobs of iron
Occur when bone marrow unable to incorporate iron into Hb molecule

Myelodysplastic syndrome

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13
Q

What are smudge cells and where are they found

A

Ruptured white blood cells that occur in the process of preparing blood film due to aged or fragile white blood cells

CLL

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14
Q

What are spherocytes and what do they indicate

A

Spherical RBC without normal bi-concave shape

Autoimmune haemolytic anaemia or hereditary spherocytosis

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15
Q

List the causes of microcytic anaemia

A 
Thalassaemia 
Anaemia of chronic disease
Iron deficiency anaemia 
Lead poisoning
Sideroblastic anaemia
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16
Q

List the causes of normocytic anaemia

A 
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
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17
Q

List the causes of macrocytic anaemia

A

Megaloblastic anaemia - B12 deficiency and folate deficiency

Normoblastic anaemia - Alcohol, reticulocytosis. hypothyroidism, liver disease, Drugs (azathioprine)

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18
Q

List some symptoms of anaemia

A 
Tired
Breathless
Headache
Dizzy 
Palpitations
Worsen of other conditions - angina, HF and PVD 
Pica - dietary cravings for abnormal things such as dirt 
Hair loss
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19
Q

List some signs of anaemia

A 
Pale skin 
Tachycardia
Raised respiratory rate
Conjunctival pallor 
Koilonychia - spoon shaped nails 
Angular cheilitis 
Atrophic glossitis - smooth tongue due to atrophy of the papillae and can indicate iron deficiency
Jaundice 
Brittle hair and nails 
Bone deformities - thalassaemia
Oedema, HTN, excoriations - CKD
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20
Q

How do you investigate anaemia

A 
Hb
MCV
B12
Folate
Ferritin 
Blood film
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21
Q

What further investigations may you do for someone with anaemia and unclear cause

A

OGD and colonoscopy 0 GI blood loss

Bone marrow biopsy

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22
Q

Where and how is iron absorbed

A

In the duodenum and jejunum

Relies on stomach acid to keep it in its soluble Fe2+ form instead of Fe3+ (ferric)

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23
Q

List some causes of iron deficiency anaemia

A

Medication - PPI (interfere with absorption)
Blood loss
Dietary insufficiency
Increased requirement - pregnancy

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24
Q

In what form does iron travel round the body

A

Ferric Fe3+ form bound to carrier protein transferrin

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25
What is the total iron binding capacity
Total space on the transferrin molecules for the iron to bind Related to the amount of transferrin in the blood
26
What is ferritin
Form iron takes when it is deposited and stored in cells
27
What causes release of ferritin
Inflammation - infection and cancer
28
What happens to ferritin in iron deficiency anaemia
Can be low or normal
29
What is transferrin saturation
Good indication of the total iron in the body Normal = 30% Transferrin saturation = serum iron/total iron binding capacity
30
How is iron deficiency anaemia managed
Blood transfusion Iron infusion - cosmofer Oral iron - ferrous sulphate 200mg tds - slowly corrects iron
31
Give some side effects of ferrous sulphate
Black stool
32
When is iron infusion avoided
Sepsis - feeds bacteria
33
How much of a rise in Hb can you expect per week when correcting iron deficiency with oral iron
10g/L/week
34
Describe B12 absorption
Gastric parietal cells secrete intrinsic factor Intrinsic factor essential for absorption of B12 in ileum
35
What is pernicious anaemia
Autoimmune condition where antibodies form against the parietal cells or intrinsic factor Lack of intrinsic factor prevents absorption of vitamin B12 and the patient becomes vitamin B12 deficient
36
Describe the neurological symptoms caused by Vitamin B12 deficiency
Pins and needles - peripheral neuropathy Loss of vibration sense or proprioception Visual changes Mood or cognitive changes
37
Describe the test for pernicious anaemia
Intrinsic factor antibody Gastric parietal cell antibody - less helpful
38
Describe the management of pernicious anaemia
Oral replacement with cyanocobalamin if dietary deficiency B12 If pernicious anaemia - 1mg hydroxocobalamin IM 3 times weekly for 2 weeks then every 3 months
39
Why is it essential to treat B12 deficiency before treating folate deficiency if a patient has both
Subacute degeneration of the cord
40
What is haemolytic anaemia
Destruction of red blood cells leading to anaemia
41
List some inherited haemolytic anaemias
``` Hereditary spherocytosis Hereditary elliptocytosis Thalassaemia Sickle cell disease G6PD deficiency ```
42
List some acquired haemolytic anaemias
Autoimmune haemolytic anaemia Alloimmune - transfusion reaction and haemolytic disease of newborn Paroxysmal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve related haemolytic anaemia
43
Describe the features of haemolytic anaemia
Anaemia Splenomegaly Jaundice
44
Describe the investigations for haemolytic anaemia
FBC - normocytic anaemia Blood film - schistocytes (fragments of RBCs) Direct Coombs test - autoimmune haemolytic anaemia
45
What is the inheritance pattern of hereditary spherocytosis
Autosomal dominant
46
How does hereditary spherocytosis present
Jaundice Gallstones Splenomegaly Aplastic anaemia in prescence of parvovirus
47
How is hereditary spherocytosis diagnosed
FH Blood film - spherocytes Mean corpuscular haemoglobin concentration (MCHC) is raised on a FBC Reticulocytes will be raised due to rapid turnover of RBC
48
How is hereditary spherocytosis treated
Folate supplements Splenectomy Cholecystectomy if gallstones is a problem
49
What is the inheritance pattern for G6PD deficiency
X linked recessive
50
Who is G6PD deficiency more common in
Mediterranean and African
51
List some triggers for haemolytic anaemia crises in G6PD deficiency
Fava beans Infection Medication - antimalarials (primaquine), ciprofloxacin, sulfonylureas, sulfalazine and other sulphonamide drugs
52
What is found on a blood film in G6PD deficiency
Heinz bodies
53
What are the two types of autoimmune haemolytic anaemia
Warm | Cold
54
Describe warm autoimmune haemolytic anaemia
Antibodies against the RBC produced and work at normal or above nomral temperatures
55
What causes warm autoimmune haemolytic anaemia
Idiopathic
56
Describe cold autoimmune haemolytic anaemia
Cold agglutin disease At lower temperatures the antibodies against RBC attach themselves to the RBC and cause them to clump together - agglutination This results in the destruction of the RBC as the immune system is activated against them and gets fikltered and destroyes in the spleen
57
What is cold haemolytic anaemia secondary to
Lymphoma Leukaemia SLE Infection - EBV, HIV, CMV, mycoplasma
58
How is autoimmune haemolytic anaemia treated
Blood transfusion Prednisolone Rituximab - CD20 monoclonal antibody Splenectomy
59
Describe prosthetic valve haemolysis
Haemolytic anaemia as a complication of prosthetic heart valves Can be bio prosthetic and metallic Turbulence around the valve and collision of RBC with the implanted valve
60
Describe the management of prosthetic valve haemolysis
Monitoring Oral iron Blood transfusion or revision surgery if severe
61
Describe the normal chains in normal Hb
2 alpha and 2 beta globin chains
62
What is thalassaemia
Genetic defect in the protein chains which make up haemoglobin RBC more fragile and break down easily
63
Describe the inheritance pattern of thalassaemia
Autosomal recessive
64
List some signs and symptoms of thalassaemia
``` Splenomegaly Pronounced forehead and malar eminences - bone marrow expands to produce extra RBC Microcytic anaemia Fatigue Pallor Jaundice Gallstones Splenomegaly Poor growth and development ```
65
How is thalassaemia diagnosed
FBC - microcytic anaemia Haemoglobin electrophoresis - globin abnormalities DNA testing - genetic abnormality Pregnant women screened at booking
66
Describe iron overload in thalassaemia and its management
Faulty creation of RBC, recurrent transfusion and increased absorption of iron in response to anaemia Symptoms - fatigue, joint pain, liver cirrhosis, infertility and impotence, heart failure, arthritis, osteoporosis and diabetes Serum ferritin levels checked to monitor for iron overload Management - iron chelation and limit transfusions
67
Describe the management of alpha thalassaemia
``` FBC monitoring Complication monitoring Blood transufsions Splenectomy Bone marrow transplant may be curative ```
68
Which chromosome si the faulty alpha globin gene located
16
69
Which chromosome is the faulty beta globin gene located
11
70
Name the 3 types of beta thalassaemia
Minor Intermedia Major
71
Why do multiple types of beta thalassaemia exist
Gene defects either consist of abnormal copies that retain function or deletion genes with no function
72
What is the management for thalassaemia minor
No active treatment - just monitoring
73
Describe the management of thalassaemia intermedia
Monitoring | Transfusions and maybe Iron chelation
74
Describe the management of thalassaemia major
Regular transfusions, iron chelation and splenectomy | Bone marrow transplant may be curative
75
Describe the pathophysiology of sickle cell disease
At 6 weeks of age fetal haemoglobin is replaced by HbA In sickle cells disease abnormal HbS causes cells to be sickle shaped Autosomal recessive defect of beta globin on chromosome 11
76
Why do people in Africa, India, Caribbean and the middle east have high proportion of sickle cell trait
Selective advantage to malaria - reduces severity
77
Describe how sickle cell disease is diagnosed
Pregnant women at risk of being carriers are tested during pregnancy Heel prick test on newborn at day 5 of age
78
List some complications of sickle cell disease
``` Anaemia Infection Stroke Avascular necrosis of the hip Pulmonary hypertension Priapism CKD Sickle cell crises Acute chest syndrome ```
79
Give some general management options for sickle cell disease
``` Avoid dehydration Vaccination Antibiotic prophylaxis - Pen V Hydroxycarbamide - stimulates fetal haemoglobin production and has a protective effect against crises and acute chest syndrome Blood transfusion Bone marrow transplant ```
80
Describe sickle cell crises
Umbrella term | Can occur spontaneously or be triggered by stresses such as infection, dehydration, cold or significant life events
81
How are sickle cells crises managed
``` Low threshold for admission Treat any infection Keep warm IV fluids Simple analgesia Penile aspiration in priaprism ```
82
Describe vaso-occlusive crises
Sickle shaped blood cells clog capillaries and cause distal ischaemia Associated with dehydration and raised haematocrit Symptoms are pain, fever, triggering infection, priaprism
83
Describe splenic sequestration crisis
RBC block blood flow in spleen Acutely enlarged and painful spleen Pooling of blood - severe anaemia and hypovolaemic shock Management with blood transfusion and fluid resus Splenectomy prevents sequestration crisis Recurrence leads to splenic infarction and infection
84
Describe aplastic crisis
Temporary loss of creation of new blood cells Triggered by parvovirus B19 infection Significant anaemia Supportive blood transfusion and spontaneously resolves in 1 week
85
Describe acute chest syndrome
Fever or resp symptoms and new infiltrates on CXR Due to infection or non infective cause Management with ABx or antivirals, blood transfusions, incentive spirometry that encourages dep breathing, artificial ventilation
86
What is leukaemia
Cancer of the cells of the bone marrow
87
How does leukaemia cause pancytopenia
Excess production of one form of cells leads to suppression of other cell lines and underproduction of other cell types
88
Describe the presentation of leukaemia
``` Fatigue Fever Failure to thrive Pallor due to anaemia Petechiae and abnormal bruising due to thrombocytopenia Abnormal bleeding Lymphadenopathy Hepatosplenomegaly ```
89
How is leukaemia diagnosed
``` FBC Blood film - abnormal cells and inclusions Lactate dehydrogenase Bone marrow biopsy - iliac crest, LA, sample may be aspirated (fluid of cells) or trephine (solid core sample) CXR Lymph node biopsy Lumbar puncture if CNS involvement CT/MRI/PET ```
90
What are the differentials for petechiae
``` Leukaemia Meningococcal septicaemia Vasculitis Henoch-schonlein purpura Idiopathic thrombocytopenia purpura Non accidental injury ```
91
Describe acute lymphoblastic leukaemia
Malignant change to one of the lymphocyte precursor cells Acute proliferation of B lymphocytes
92
Which chromosomal abnormalities is ALL associated with
``` Downs syndrome Philadelphia chromosome (t9:22) translocation - 30% ```
93
What age is affected by ALL
2-4yrs and >45
94
Describe chronic lymphocytic leukaemia
Chronic proliferation of single type of B lymphocyte
95
What age is most often affected by CLL
>55
96
What does blood film of CLL show
Smudge or smear cells Occur in the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film
97
What can CLL transform into?
Richters transformation - high grade lymphoma
98
What additional symptoms may CLL present with
Warm haemolytic anaemia Infection Bleeding Weight loss
99
Describe the 3 phases of CLL
Chronic phase - lasts 5 yrs, asymptomatic and patients diagnosed accidentally with raised WCC Accelerated phase - abnormal blast cells take up a high proportion of the cells in the bone marrow and blood., more symptomatic, anaemia and thrombocytopenia Blast phase - high proportion of blast cells and blood, often fatal
100
Which genetic abnormality is associated with CML
Philadelphia chromosome - Chromosome 9 and 22 translocation
101
Describe AML
Acute myeloid leukaemia Most common leukaemia in adults Middle age and onwards Transformation from myeloproliferative disorders such as polycythaemia rubra vera and myelofibrosis Blood film shows high proportion of blast cells with Auer rods in their cytoplasm
102
Describe leukaemia treatment
Chemotherapy and steroids Radiotherapy Bone marrow transplant Surgery
103
List the complications of chemotherapy
``` Failure Stunted growth and development in children Infection - immunosuppression Neurotoxicity Infertility Secondary malignancy Cardiotoxicity Tumour lysis syndrome ```
104
What causes tumour lysis syndrome
Release of uric acid from the cells being destroyed by chemotherapy Forms crystals in the interstitial fluid and renal tubules and causes AKI Potassium and phosphate are also released High phosphate causes a low calcium
105
What drugs can be given to reduce the uric acid from tumour lysis syndrome
Allopurinol and Rasburicase
106
What is the preferred treatment for CML
Imatinib (tyrosine kinase inhibitor)
107
What is the preferred treatment for non-hodgkins lymphoma
R-CHOP - rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone
108
What is the preferred treatment for CLL
FCR - fludarabine, cyclophosphamide and rituximab
109
What is a Rouleaux formation on blood film associated with
Myeloma
110
What are Reed-Sternberg cells on blood film associated with
Hodgkins lymphoma
111
What are lymphomas
Cancers that affect the lymphocytes inside the lymphatic system - proliferate inside lymph nodes causing lymphadenopathy
112
Name the two types of lymphoma
Hodgkin's | Non-Hodgkin's
113
What is Hodgkin's lymphoma
Proliferation of lymphocytes
114
List some risk factors for Hodgkin's lymphoma
HIV EBV Autoimmune conditions - RA and sarcoidosis FH
115
Describe the presentation of Hodgkin's lymphoma
Bimodal age distribution 20 and 75yo Lymphadenopathy - non-tender and rubbery, axilla, groin, neck. pain when drink alcohol B symptoms - fever, weight loss, night sweats ``` Fatigue Itching Cough SOB Abdominal pain Recurrent infection ```
116
Describe the investigations for Hodgkin's lymphoma
Lactate dehydrogenase - non specific Lymph node biopsy - diagnostic - reed Sternberg cells (abnormally large B cells with multiple nuclei and nucleoli) - owl eyes CT/MRI/PET - staging and diagnosis
117
Describe the Ann-Arbor staging system
Used for both Hodgkins and non-hodgkins lymphoma Puts importance on wether the affected nodes are above or below the diaphragm Stage 1 - confined to one region of lymph nodes Stage 2 - in more than one region but on the same side of the diaphragm Stage 3 - affects lymph nodes above and below the diaphragm Stage 4 - widespread involvement including non-lymphatic organs such as the lungs and liver
118
Describe the management of lymphoma
Chemo - risk of leukaemia and infertility | Radiotherapy - risk of cancer, damage to tissues and hypothyroidism
119
Describe Burkitt lymphoma
Associated with EBV, HIV, malaria
120
Describe MALT lymphoma
Mucosa associated lymphoid tissue Usually around the stomach Associated with Hpylori
121
Describe diffuse large B cell lymphoma
Rapidly growing painless mass in patients over 65
122
List some risk factors for non-hodgkins lymphoma
``` HIV EBV H.pylori Hep B or C Exposure to pesticides - trichloroethylene FH ```
123
Describe the management of non-hodgkins lymphoma
``` Watchful waiting Chemo Monoclonal antibodies - rituximab Radiotherapy Stem cell transplantation ```
124
What is myeloma
Cancer of plasma cells - B lymphocyte | Excess production of antibodies
125
What is multiple myeloma
Myeloma affecting multiple areas of the body
126
What is monoclonal gammopathy of undetermined significance
Excess of a single type of antibody or antibody compenets without myeloma or cancer Incidental finding May progress to myeloma - monitor patient
127
What is smouldering myeloma
Progression of MGUS with higher levels of antibodies Premalignant
128
Describe waldenstroms macroglobulinaemia
Type of smouldering myeloma where there is excessive IgM
129
What are antibodies made up of
2 heavy and 2 light chains
130
Which type of antibody are most myelomas
IgG
131
What can be found in the urine of patients with myeloma
Bence Jones protein - subunit of antibody (light chain)
132
Describe myeloma bone disease
Plasma and stromal cells release cytokines which stimulate osteoclast activity to absorb bone and to decrease osteoblast depositing bone activity. Skull, spine, ribs, long bones Osteolytic - predispose to pathological fractures Hypercalcaemia
133
Describe myeloma renal disease
Immunoglobulins block tubule flow Dehydration Hypercalcaemia Nephrotoxic medications - bisphosphonates eg
134
Describe how myeloma affects plasma viscosity and what this results in
Increased plasma viscosity - more proteins Essy bruising, bleeding Loss of sight due to vascular disease in eye Purple discolouration of extremeties Heart failure
135
Give the acronym used to remember features of myeloma
Calcium - raised Renal failure Anaemia - normochromic, normocytic Bone lesions and pain
136
List some myeloma risk factors
``` Old age Male Black African FH Obesity ```
137
List the investigations for myeloma
``` FBC Calcium ESR Plasma viscosity Serum protein electrophoresis - serum immunoglobulins/Serum light chain assay Urine bence Jones protein test Bone marrow biopsy Imaging - whole body MRI, whole body CT, skeletal survey ```
138
List some X-ray changes of myeloma
Punched out lesions Lytic lesions Raindrop skull - many punched out lesions that give appearance of raindrops splashing on skull
139
Describe the management of myeloma
MDT Chemotherapy - bortezomid, thalidomide, dexamethasone Stem cell transplant VTE prophylaxis Bone disease - bisphosphonates, radiotherapy, ortho surgery, cement augmentation
140
List some complication of myeloma
``` Infection Pain Renal failure Anaemia Hypercalcaemia Peripheral neuropathy Spinal cord compression Hyperviscositiy Amyloidosis ```
141
What are myeloproliferative disorders and give examples
Uncontrolled proliferation of single type of stem cell Primary myelofibrosis - haematopoetic stem cells Polycythaemia vera - erythroid cells Essential thrombocythemia - megakaryocytes
142
What do myeloproliferative disorders have the potential to turn into
AML
143
Which mutations are associated with myeloproliferative disorders
JAK2 MPL CALR
144
Describe myelofibrosis
Where proliferation of cell lines leads to fibrosis of the bone marrow - replaced by scar tissue Response to cytokines such as fibroblast growth factor Fibrosis leads to anaemia and leukopenia When the bone marrow is replaced by scar tissue, the production of blood cells starts to happen in other areas such as the liver and spleen - extramedullary haematopoiesis - hepatomegaly and splenomegaly - portal hypertension and if around the spinal cord can cause spinal cord compression
145
Describe the presentation of myeloproliferative disorders
Asymptomatic Systemic symptoms - fatigue, weight loss, night sweats, fever ``` Anaemia Splenomegaly Portal hypertension Low platelets - bleeding and petichiae Thrombosis Raised RBC - red face and thrombosis Low WBC - infection ```
146
What are the 3 clinical signs on examination in someone with polycythaemia vera
Conjunctival pallor Ruddy complexion Splenomegaly
147
What are the FBC findings in polycythaemia vera
Raised Hb >185 in men and 165 in women
148
What are the FBC findings in thrombocythemia
Raised platelets >600
149
What are the FBC findings in myelofibrosis
Anaemia Leucocytosis or leukopenia Thrombocytosis or thrombocytopenia
150
What can a blood film in myelofibrosis show
Teardrop shaped RBCs, varying in size (poikilocytosis) and immature red and white cells (blasts)
151
Describe how myeloproliferative disorders are diagnosed
Bone marrow biopsy | Genetics - JAK2, MPL, CALR
152
Describe the management of primary myelofibrosis
Mild symptoms - monitor and no active treatment Allogenic stem cell transplantation Chemotherapy Supportive management
153
Describe the management of polycythaemia vera
Venesection Aspirin - reduce thrombus formation Chemotherapy - hydroxycarbamide and interferon (cyto-reductive therapy)
154
Describe the management of essential thrombocythemia
Aspirin - reduce thrombus formation Chemo to control disease
155
Describe myelodysplastic syndrome
Myeloid bone marrow cells not maturing properly and not producing healthy blood cells Anaemia Neutropenia Thrombocytopenia
156
What are some risk factors for myelodysplastic syndrome
>60yo | Previous chemotherapy or radiotherapy
157
What is myelodysplastic syndrome associated with
AML
158
Describe how myelodysplastic syndromes are diagnosed
FBC Blood film - blasts Bone marrow aspiration and biopsy
159
Describe the management of myelodysplastic syndrome
Watchful waiting Supportive treatment Chemo Stem cell transplantation
160
List the causes of thrombocytopenia
Decreased production - Sepsis - B12/folate defieicny - Liver failure - Leuakemia - Myelodysplastic syndrome Increased destruction - Medication - sodium valproate, methotrexate, isotretinoin, antihistamines PPI - Alcohol - ITP - TTP - Heparin induced thrombocytopenia - Haemolytic ureamic syndrome
161
Describe the presentation of thrombocytopenia
Incidental finding Platelets <50 - easy, spontaneous bruising and prolonged bleeding time, nosebleed, bleeding gums, heavy periods, easy bruising, blood in the urine or stool Platelets
162
Describe immune thrombocytopenic purpura (ITP)
Antibodies against platelets - creates an immune response against platelets resulting in destruction and low count
163
Describe the management of ITP
Oral prednisolone IV immunoglobulins Rituximab - monoclonal antibody against B cells Splenectomy Monitor platelets Educate patient about persistent headaches and melaena - when to seek help Control BP and suppress menstrual periods
164
Describe thrombotic thrombocytopenic purpura
Blood clots develop throughout the small vessels of the body using up platelets and causing thrombocytopenia, bleeding under the skin Microangiopathy Genetic/autoimmune Defect in ADAMTS13 protein which normally inactivates von Willebrand factor and reduces platelet adhesion to vessel walls and clot formation. Overactivity of von Willebrand factor causes platelets to be used up and blood clots form, these break down causing haemolutic anaemia
165
How is TTP treated
Plasma exchange Steroids Rituximab
166
Describe heparin induced thrombocytopenia
Development of antibodies against platelts in response to exposure to heparin Antibodies target platelet factor 4 on platelets Bind to platelets and activate clotting mechanisms causing hypercoagulable state and leads to thrombosis and break down platelets causing thrombocytopenia
167
How is HIT diagnosed
HIT antibodies
168
How is HIT managed
Stop the use of heparin and switch to alternative anticoagulant
169
What is Von Willebrand disease
Most common inherited cause of abnormal bleeding Autosomal dominant Deficiency/malfunctioning/absence of glycoprotein Von Willebrand factor
170
How do patients with von Willebrand present
``` Bleeding gums with brushing Nose bleeds Heavy menstrual bleeding Heavy bleeding during surgery FH ```
171
How is Von Willebrand diagnosed
``` Abnormal bleeding FH Bleeding assesssment tools Lab investigations No specific test ```
172
How is von Willebrand managed
Desmopressin - stimulate release of VWF VWF infusion Factor 8 alongside VWF infusion Women with heavy periods - mefenamic acid, tranexamic acid, norethisterone, COCP, Mirena coil and hysterectomy in severe cases
173
What is Haemophilia A a deficiency of
Factor 8
174
What is haemophilia B (Christmas disease) a deficiency of
Factor 9
175
Describe the inheritance of haemophillia
X linked recessive
176
List some signs and symptoms of haemophilia
``` Spontaenous haemorrhage Intracranial haemorrhage Haematoma Cord bleeding Hemarthrosis Bleeding into muscles Gum bleeding GI bleed Haematuria Retroperitoneal space bleeding ```
177
How is haemophilia diagnosed
Bleeding scores Cogulation factor assays Genetic testing
178
How is haemophilia managed
IV infusions of affected clotting factors (8 or 9) - complication is antibodies forming against the clotting factors Desmopressin - stimulate von Willebrand factor - stimulates clotting Tranexamic acid - antifibrinolytic
179
What defect would a patient have if a DVT travelled to the brain reuslting in a stroke
Atrial septal defect
180
List the risk factors for VTE
``` Immobiltiy Recent surgery Long haul flight Hormone replacement therapy with oestropgen or COCP Malignancy Pregnancy Polycythaemia SLE Thrombophilia (antiphispholipid syndrome, protein C/S defieicny), factor V leiden ```
181
What VTE prophylaxis is given to patients
LMWH with enoxaparin | Anti-embolic compression stockings
182
What is the main contraindication to compression stockings
Peripheral arterial disease
183
What is the contraindications to LMWH as VTE prophylaxis
Anticoagulation or active bleeding
184
Describe the presentation of DVT
``` Unilateral Calf or leg swelling Dilated superficial veins Tenderness to the calf Oedema Colour change to leg ```
185
What are the alternatives to a bilateral DVT
Chronic venous insufficiency | Heart failure
186
How do you examine for a DVT
Measure leg circumference of the calf 10cm below the tibial tuberosity - more than 3cm difference between the calves is significant
187
What does the Wells score predict
Risk of a patient presenting with DVT/PE symptoms actually having one
188
How is VTE diagnosed
D-dimer if wells score suggests Doppler USS leg - repeat 1 week after negative USS and positive D-dimer but unlikely wells PE- CTPA or VQ scan if patient is pregnant or has significant renal impairment
189
List some causes of raised D-dimer
``` Pregnancy Malignancy Heart failure Surgery Pneumonia ```
190
Describe the initial management of VTE
Apixaban or rivaroxaban
191
Describe long term anticoagulation of those with VTE
DOAC, warfarin or LMWH
192
What is first line long term anticoagulation for those with antiphopsolipid syndrome following VTE
Warfarin
193
What is the first line anticoagulant in pregnancy
LMWH
194
How long do you continue long term anticoagulation following VTE
3 months if reversible cause | 6 months if recurrent, unclear cause or cancer
195
Describe the use of inferior vena cava filters
Used to filter blood and catch clots travelling to the lungs - prevent PES Used in cases of recurrent PEs or those unsuitable for anticoagulation
196
Describe budd-chiari syndrome
Blood clot in hepatic vein blocking outflow of blood Acute hepatitis Abdominal pain Hepatomegaly Ascites Management involves anticoagulation and investigating the underlying hypercoagulation and treating the hepatitis
197
Describe the coagulation screen results for DIC
Prolonged PT, Prolonged APTT and reduced fibrinogen
198
What so you give to treat DIC when patient is bleeding
Platelets Cryoprecipitate Fresh frozen plasma

Finals (18 decks)

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