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Haematology Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

malignant clonal proliferation of lymphoblastic cells (most commonly B cells)

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2
Q

What is the most common childhood cancer?

A

ALL - most common <5 years

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3
Q

What are 3 risk factors for ALL?

A

Down’s
Kleinfelter’s syndrome
Fanconi anaemia

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4
Q

what are 5 poor prognostic factors for ALL?

A

<2 years or >10 years
WBC >20
T or B cell surface markers
Non-Caucasian
Male

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5
Q

what is the management of ALL?

A

4-8 weeks corticosteroids, vincristine, doxorubicin

Up to 1 year of high dose chemo started after remission

2 years of mercaptopurine and methotrexate after remission

Bone marrow transplant

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6
Q

What are 9 presentations of leukaemia?

A

Fatigue
Fever
Pallor
Petechiae/bruising/bleeding
Frequent infections
Bone pain
Lymphadenopathy
Hepatosplenomegaly
Failure to thrive

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7
Q

what are 7 general investigations for leukaemia?

A

FBC - within 48h
Blood film
Lactate dehydrogenase
Bone marrow biopsy
CT/PET staging
Lymph node biopsy
Genetics and immunophenotyping

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8
Q

where is a bone marrow biopsy taken from?

A

iliac crest

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9
Q

what is the gold standard investigation for leukaemia?

A

bone marrow aspiration and biopsy

> 20% lymphoblasts/myeloblasts in bone marrow is diagnostic

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10
Q

what is the treatment for ALL?

A

Chemo - vincristine + corticosteroids
imatinib - if Philadelphia +ve
mercaptopurine + methotrexate => 2 years maintenance

bone marrow transplant

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11
Q

What is acute myeloid leukaemia?

A

the clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extramedullary tissues.

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12
Q

what are 5 risk factors for AML?

A

65+
previous chemo/radiation
Down’s syndrome
benzene - painters, petroleum, rubber
Myeloproliferative disorders

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13
Q

which leukaemia is associated with DIC?

A

AML - abnormal promyelocytes release granules which can cause thrombocytopenia

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14
Q

what is the most common leukaemia in Downs?

A

AML

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15
Q

what is the pathophysiology of AML?

A

Accumulation of myeloid blasts unable to differentiate into mature neutrophils, RBCs or platelets resulting in bone marrow failure

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16
Q

what is characteristically seen on blood smear in AML?

A

Auer rods

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17
Q

what is the management of AML?

A

Cytarabine and an anthracycline (daunorubicin)

All-trans retinoid acid - promyelocytic leukaemia

stem cell transplant

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18
Q

what is the AML classification system?

A

French-American-British (FAB) classification

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19
Q

what is the prognosis for AML?

A

high incidence of relapse

5 year survival - 25%

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20
Q

what is reticulocyte count?

A

measure of immature RBCs

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21
Q

What are 5 causes of microcytic anaemia?

A

TAILS

Thalassaemia
Anaemia of chronic disease
Iron deficiency
Lead Poisoning
Sideroblastic anaemia

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22
Q

what are 5 causes of normocytic anaemia?

A

AAAHH

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia - CKD, bone marrow suppression
Haemolytic anaemia
Hypothyroidism

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23
Q

what are 7 causes of macrocytic anaemias?

A

FAT RBC

Foetus - pregnancy
Alcohol - normoblastic
Thyroid disease - hypo

Reticulocytosis - haemolytic anaemia/blood loss
B12 and Folate deficiency - megaloblastic
Cirrhosis and liver disease

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24
Q

what are 3 causes of macrocytic megaloblastic anaemia?

A

B12 deficiency
Folate deficiency
Meds - methotrexate

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25
what are 7 causes of macrocytic normoblastic anaemia?
Alcohol Liver disease Hypothyroidism Pregnancy Reticulocytosis Myelodysplasia Drugs - cytotoxic
26
what are 6 symptoms of anaemia?
often asymptomatic Fatigue/faintness headache, confusion, dizziness SOB angina/palpitations/claudication Pica - abnormal cravings - IDA only Hair loss - IDA only
27
what is the reference range for MCV?
80-100 femtolitres
28
What are the WHO the reference ranges for Hb?
Women - 120-165 g/litre Men - 130-180 g/Litre
29
where is B12 found?
Meat fish dairy eggs NO PLANTs
30
how long do B12 stores last?
4 years
31
what are 3 causes of B12 deficiency anaemia?
diet malabsorption pernicious anaemia (most common)
32
How is B12 absorbed?
combines with INTRINSIC FACTOR secreted from PARIETAL CELLS in stomach and absorbed in TERMINAL ILEUM
33
why is B12 necessary?
needed for DNA and thymine synthesis
34
what are 7 neurological complications of B12 deficiency?
Loss of cutaneous sensation loss of mental/physical drive Muscle weakness Optic neuropathy Psychiatric disturbance Symmetrical neuropathy affecting legs more than arms Urinary/faecal incontinence
35
what is the name of the test for measuring total B12?
serum cobalamin
36
what can be seen on blood film in B12/folate deficiency?
Oval macrocytes, hypersegmented neutrophils and circulating megaloblasts in the blood film, as well as megaloblastic change in the bone marrow, are typical features of clinical cobalamin deficiency.
37
what are 4 conditions associated with pernicious anaemia?
Thyroid disease Vitiligo Stomach Cancer Addisons
38
what is the test for pernicious anaemia?
anti-intrinsic factor anybody test
39
what is pernicious anaemia?
autoimmune destruction of parietal cells leading to B12 deficiency anaemia
40
what are 3 risk factors for B12 anaemia?
vegan history of GI surgery H.pylori infection
41
what are 3 differentials for B12 anaemia?
B9 deficiency myelodysplatic syndrome alcoholic liver disease
42
what is the treatment for B12 anaemia?
oral cyanocobalamin 50-150mg OD IM hydroxocobalamin 1g 2x yearly
43
what are 3 complications of B12 anaemia?
neurological deficits neural tube defects optic atrophy and psychiatric symptoms
44
What is chronic lymphocytic leukaemia?
a malignant monoclonal proliferation of B lymphocytes
45
what is the most common leukaemia in adulthood?
CLL
46
what are 3 risk factors for CLL?
70+ male FHx
47
what is seen on blood smear in CLL?
Smudge cells also spherocytes and polychromasia
48
what are 3 treatment options for CLL?
monitoring - early stage Chemo - FCR, chlorambucil and rituximab stem cell transplant
49
what are 3 CLL specific complications?
Richter's transformation to high grade B-cell lymphoma Warm autoimmune haemolytic anaemia Hypogammaglobulinaemia
50
what is the rule of 3s in CLL?
1/3 don't progress 1/3 progress slowly 1/3 progress actively
51
what is chronic myeloid leukaemia?
the malignant proliferation of partially mature myeloid cells (especially granulocytes) in bone marrow and blood
52
what are the 3 phases of CML?
Chronic - usually asymptomatic, can last several years before progressing Accelerated - blasts increase leading to pancytopenia Blast phase - >20% blasts, severe symptoms often fatal pancytopenia
53
what gene is related to CML??
the Philadelphia chromosome on chromosome 22 Associated with BCR-ALB1
54
what leukaemia is the Philadelphia gene related to?
CML
55
when is the peak incidence of CML?
65-75
56
what is a known risk factor for CML?
ionising radiation
57
what is the gold standard for CML?
presence of Philadelphia chromosome - cytogenetics/FISH
58
what is the treatment for CML?
1 - Imatinib - tyrosine kinase inhibitor Hydroxyurea Interferon alpha Bone marrow transplant
59
what is the prognosis for CML?
75% 5 year survival
60
what is folate (B9) present in?
green vegetables legumes some fruits yeast liver nuts
61
how long do folate stores last?
4 months
62
where in folate absorbed?
proximal jejunum/duodenum
63
what are 4 risk factors for folate deficiency?
elderly/young poverty chron's/coeliac pregnant
64
what is folate needed for?
DNA synthesis and repair
65
what are 7 clinical features of folate (B9) and B12 deficiency?
anaemia symptoms - SOB, Headache, cognitive changes, weakness Glossitis - red smooth shiny tongue Oropharyngeal ulceration Diarrhoea Heart murmur Mild jaundice Pallor of mucous membranes or nail beds
66
What is the management of folate deficiency anaemia?
oral folic acid 5mg OD for 4 months ALWAYS CHECK B12 and replace if low - replacing folate can mark low b12 and allow neurology to develop
67
what are 3 differentials of folate deficiency?
B12 deficiency hypothyroidism alcoholic liver disease
68
what are 2 complications of folate deficiency?
pregnancy complications (prematurity) Cardiovascular disease
69
what is G6PD deficiency?
Glucose-6-phosphate dehydrogenase deficiency X-linked condition causing haemolytic anaemia due to susceptibility of RBCs to free radicals common in middle eastern, Mediterranean and African populations
70
what are 3 triggers for haemolysis in G6PD deficiency?
Infection Medications Broad (fava) beans
71
what are 6 medications that can trigger haemolysis in G6PD?
Primaquine - antimalarial Ciprofloxacin Nitrofurantoin Trimethoprim Sulfonylureas (gliclazide) Sulfasalazine
72
what are 5 presentations of G6PD?
jaundice - can be neonatal Anaemia Intermittent jaundice - in response to triggers Gallstones Splenomegaly
73
what ca be used to diagnose G6PD?
G6PD enzyme assay - checked 3 months after acute episode of haemolysis
74
what can be seen on blood film in G6PD deficiency?
Heinz bodies - blobs of denatured haemoglobin within RBCs Bite and blister cells
75
what are 3 inherited causes of haemolytic anaemia?
RBC membrane defects (spherocytosis, elliptocytosis) Enzyme defects (G6PD) haemoglobinopathies (thalassaemia, sickle cell)
76
what are 5 acquired causes of haemolytic anaemia?
Autoimmune haemolytic anaemia Alloimmune haemolytic anaemia (transfusion reaction, haemolytic disease of newborn) Paroxysmal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve related Haemolysis
77
what are 4 risk factors for haemolytic anaemia?
autoimmune disorders FHx drugs prosthetic heart valves
78
what are 4 manifestations of haemolytic anaemia?
anaemia - pallor, fatigue, dizzy, SOB Jaundice Dark urine Splenomegaly
79
what is the gold standard investigation for autoimmune haemolytic anaemia?
Coombs test (direct antiglobulin test)
80
what is seen on blood film in haemolytic anaemia?
Schistocytes - fragments of RBCs
81
what are the 2 different types of autoimmune haemolytic anaemia?
Warm - more common, Haemolysis occurs at normal or higher temperatures Cold - haemolysis occurs at <10 degrees after RBCs agglutinate due to antibodies attaching
82
what are 5 conditions that cold autoimmune haemolytic anaemia can be associated with?
Lymphoma Leukaemia SLE Infection - mycoplasma, EBV, CMV, HIV
83
what is the treatment for autoimmune haemolytic anaemia?
Rituximab Prednisolone Blood transfusion splenectomy
84
what is paroxysmal nocturnal haemoglobinuria?
mutation in haematopoietic stem cells in bone morrow causing loss of RBC surface proteins which inhibit complement cascade leading to RBC destruction
85
what is the classical presentation of paroxysmal nocturnal haemoglobinuria?
red urine in the morning - contains haemoglobin and haemosiderin Anaemia thrombosis smooth muscle dystonia - oesophageal spasm, erectile dysfunction
86
what is the management of paroxysmal nocturnal haemoglobinuria?
Eculizumab - MAB targeting complement component 5 Bone marrow transplant
87
what is microangiopathic haemolytic anaemia?
destruction of RBCs due to abnormal activation of clotting cascade causing micro thrombi associated with haemolytic uraemic syndrome, DIC, TTP, SLE, Cancer
88
What is Hodgkin's lymphoma?
Uncommon haematological malignancy arising from mature B cells (lymphocytes) Characterised by the presence of Hodgkin's cells and Reed-Sternberg cells.
89
what are 4 risk factors for Hodgkin's lymphoma?
EBV infection FHx HIV + immunosuppression autoimmune conditions - RhA, Sarcoidosis
90
what is the age distribution for Hodgkin's lymphoma?
Bimodal - 20-25 years >80 years
91
what are 9 presentations of Hodgkin's lymphoma?
Painless Lymphadenopathy Hepatosplenomegally (less common than in NHL) B symptoms - Fever, Weight loss, Night Sweats Pruritus Fatigue Recurrent infections SOB + cough Abdo pain Alcohol induced lymph node pain
92
what are 3 investigations for Hodgkin's lymphoma?
FBC - anaemia, lymphopenia, thrombocytopenia, neutrophilia PET/CT for staging Bone marrow biopsy
93
what is the diagnostic investigation for Hodgkin's lymphoma?
exicisional lymph node biopsy
94
what cells are seen on lymph node biopsy in Hodgkin's lymphoma?
Reed-sternberg cells - Owls eye appearance - huge multinucleated lymphocytes Also: Lacunar cells popcorn cells - in nodular lymphocyte predominant hodgkin's lymphoma
95
what are 3 differentials for Hodgkin's lymphoma?
non-hodgkin's lymphoma infectious mononucleosis Other malignancy - head and neck cancer, breast cancer
96
what is the treatment for Hodgkin's lymphoma?
Chemo - usually ABVD - Adriamyacin - Bleomycin - Vinblastine - Dacarbazine Radiotherapy bone marrow transplant
97
what are 4 complications for Hodgkin's lymphoma?
Secondary malignancies chemo related cardio toxicity pulmonary toxicity Superior vena cava syndrome
98
what staging is used in Hodgkin's lymphoma?
Lugano classification
99
what are the Lugano classification stages?
For Hodgkin's lymphoma I - single lymph node/group II - 2+ lymph nodes one side of diaphragm III - nodes on both sides of diaphragm IV - spread beyond lymph nodes A/B => presence of B symptoms or not E = extranodal disease S = Spleen involvement X = Bulky disease (large tumour)
100
what is the most common type of anaemia?
iron deficiency
101
what percentage of menstruating women have iron deficiency anaemia?
14%
102
what are 4 causes of iron deficiency?
low dietary iron impaired absorption (coeliac) increased iron loss (bleeding) increased iron required (children, pregnant, lactating)
103
what are 6 risk factors for Iron deficiency anaemia?
pregnancy veggie/vegan diet Menorrhagia hook worms CKD gasterectomy/NSAIDs
104
where is iron absorbed?
duodenum and jejunum
105
what is the treatment of iron deficiency anaemia?
oral iron replacement - ferrous sulphate 200mg OD PO - Ferrou fumarate 210 mg OD PO for 3 months after iron deficiency is corrected absorbic acid IV iron - iron dextran/sucrose, ferruc carboxymaltose, ferric Deriso-maltose Packed RBC - Hb <70
106
what are 4 reasons for poor absorption of iron?
Absorption - coeliac and crohns Low stomach acid - PPIs, H. Pylori
107
what is the criteria for 2 week wait in IDA?
Over 60 Post menopausal women Hb <100 Men Hb <110
108
what are 5 side effects of iron supplementation?
Nausea Abdo pain constipation diarrhoea black stools
109
what are 7 complications of iron deficiency anaemia?
Cognitive impairment Impaired muscular performance High output heart failure Lowered immunity Increased maternal and foetal morbidity Preterm delivery Maternal postpartum fatigue
110
what is the top cause of iron deficiency worldwide?
hook worms
111
what is malaria?
a parasitic infection caused by protozoa of the Plasmodium family which is transmitted to humans by the bite of an anopheles mosquito
112
what plasmid is the most common cause of malaria in the UK?
Plasmodium falciparum also the most severe
113
how is malaria transmitted?
by female anopheles mosquitos
114
what are 5 plasmids that cause malaria?
Palsmodium flaciparum - most common plasmodium vivax - second most common plasmodium ovale plasmodium malariae plasmodium knowlesi
115
what is the life cycle of a malaria plasmodium?
Plasmodium reproduces in mosquito's gut producing thousands of sporozoites which are injected into whoever they bite The sporozoites travel to liver - P. vivax and P. ovale can lay dormant for months to years Sporozoites mature in liver to merozoites which enter blood and infect RBCs where they reproduce leading the RBCs to rupture causing haemolytic anaemia
116
what are the fevers like in P. vivax and p. ovale malaria infections?
Spikes every 48 hours - tertian malaria
117
what are the fevers like in P. falciparum malaria?
spikes more frequently and less regularly - Subtertian
118
what are the fevers like in p. malariae malaria?
spikes every 72 hours - quartan
119
what are 6 manifestations of malaria infection?
High cyclical fevers, sweats and rigors Fatigue Myalgia Headache nausea and vomiting Pallor - anaemia Hepatosplenomegaly Jaundice - haemolysis
120
what are 2 investigations for malaria?
Giemsa stain blood film - gold rapid diagnostic test thin and thick blood smear
121
what is required to rule out malaria?
3 negative blood smears from 3 consecutive days due to life cycle of release of merozoites
122
what is the management of uncomplicated malaria?
arthemether + lumefantrine OR Quinine + Doxycycline/clindamycin OR Proguanil + atovaquone OR Chloroquine - high resistance OR Primaquine
123
what is a contraindication to administration of primaquine?
G6PD deficiency - causes severe haemolysis
124
what is the management of severe/complicated malaria?
1 - IV artesunate IV artemether IV quinine dihydrochloride
125
what are 8 complications of malaria?
Cerebral malaria seizures reduced consciousness AKI pulmonary oedema DIC Severe haemolytic anaemia Multi-organ failure Death
126
what is the advice for travelling to malaria endemic areas?
No method 100% effective alone Mosquito spray - 50% DEET Mosquito nets and barriers Seek medical advice if symptomatic take antimalarials
127
what are 4 prophylactic antimalarials?
proguanil with atovaquone (malarone) - 2 days before to 7 days after Doxycycline - 2 days before to 4 weeks after Mefloquine - risk of psychiatric side effects - 2 weeks before to 4 weeks after Chloroquine with proguanil - high resistance
128
what is multiple myeloma?
cancer of plasma cells (differentiated b lymphocytes) which causes production of large quantities of specific paraprotein (M protein), an abnormal antibody or part antibody Multiple when affecting multiple bone marrow areas of the body
129
what is MGUS?
Monoclonal gammopathy of undetermined significance production of a specific paraprotein without the features of myeloma 1% risk per year of development into myeloma
130
what is smouldering myeloma?
abnormal plasma cells and paraproteins without organ damage or symptoms - 10% risk of progression to myeloma per year Monoclonal protein in serum >30 g/L or urine >500mg/24h or clonal bone marrow plasma cells (10-60%)
131
what are 5 risk factors for multiple myeloma?
MGUS - monoclonal gamopathy of undetermined significance Smouldering myeloma Increasing age FHx African ancestry
132
what is the pathophysiology of multiple myeloma?
There is genetic mutation in a b cell leading to production of clonal plasma cells which overproduce antibodies (immunoglobulins) These abnormal immunoglobulins produced are called paraproteins or M proteins leading to abnormally high levels of paraprotein in the blood (paraproteinaemia)
133
what is the most common Ig produced in multiple myeloma?
IgG - 55%
134
what are the 3 stages of multiple myeloma?
1 - low levels of beta-2 migroglobulin <3.5mg/L and normal albumin 2 - neither stage 1 or 3 3 - High levels of beta-2 microglobulin >5.5 mg/L
135
what are 6 manifestation of multiple myeloma?
Old CRAB - Age 65+ - Calcium - high - Renal failure - due to light chain deposition in renal tubules - Anaemia - bone marrow infiltration - normocytic, normochromic - Bone pain/Bleeding infections fatigue pathological fractures weight loss fever
136
what causes hypercalcaemia in multiple myeloma?
1 - Myeloma bone disease - Cytokines released from abnormal plasma cells leads to increased osteoclastic activity leading to bone resorption, osteolytic lesions and pathological fractures Also Renal failure - reduced excretion
137
what causes renal disease in multiple myeloma?
Paraproteins deposited in renal tubules Hypercalcaemia affects kidney function Dehydration Glomerulonephritis Medications
138
what is hyper viscosity syndrome?
Increased plasma viscosity due to increased proteins in the blood In multiple myeloma due to paraproteins Causes bleeding, visual symptoms and eye changes, neurological complications (stroke), heart failure
139
what are 8 investigations for multiple myeloma?
Urine bence-jones protein electrophoresis test Serum protein electrophoresis Serum-free light-chain assay FBC serum calcium ESR + plasma viscosity - raised U+E
140
What is needed to confirm a diagnosis of multiple myeloma?
Bone marrow biopsy Assess bone lesions with whole body MRI/low dose CT/skeletal survey
141
what is the diagnostic criteria for multiple myeloma?
Clonal bone marrow plasma cells >10 or biopsy proven plasmacytoma PLUS one of: SLIM CRAB S - >60% plasma cells in marrow LI - Involved:uninvolved Light chain ration >100 MRI focal lesions 2+ >5mm C - Hypercalcaemia Renal insufficiency Anaemia <100 Bone lesion x1 on x-ray/CT >5mm
142
what are 3 differentials for multiple myeloma?
MGUS smouldering myeloma amyloidosis
143
what 3 things can be seen on X-ray in multiple myeloma?
Well defined lytic lesions - punched out Diffuse osteopenia Abnormal fractures Lytic lesions in skull may be referred to a raindrop or pepper pot skull
144
Is multiple myeloma curable?
NO - has chronic relapsing remitting course
145
what is the treatment for multiple myeloma?
Good function, <70 - Bortezomib + Dexamethasone +/- Thalidomide + Stem cell transplant Poor function, >70 - Bortezomib + Prednisolone + Melphalan Bortezomib - 1st for relapse
146
what is the management of bone disease in multiple myeloma?
Bisphosphonates - Zoledronic acid 1st line radiotherapy ortho surgery
147
what are the 2 different types of stem cell transplant?
Autologous - using own stem cells Allogenic - using donor cells
148
what are 6 complications of multiple myeloma?
recurrent infections Fatigue pathological fractures renal failure anaemia hypercalcaemia
149
what is non-Hodgkin's lymphoma?
Malignant proliferations of B or T lymphocytes without reed-Sternberg cells
150
what are 3 different types of Non-Hodgkin's lymphoma?
Diffuse large B cell lymphoma - rapidly growing painless mass in older men Burkitt lymphoma - particularly associated with EBV MALT lymphoma - affects mucosa-associated lymphoid tissue usually around stomach
151
What are 6 risk factors for non-Hodgkin's lymphoma?
FHx >60 years EBV infection Hep B/C H. Pylori - associated with MALT lymphoma Autoimmune disease
152
are non-hodgkin's lymphomas more commonly of T or B cell origin?
B cells - 80-90%
153
what are 6 presentations of non-hodgkin's?
Painless lymphadenopathy B symptoms - weight loss, fever, night sweats Mass effect symptoms - SOB, cough, Jaundice, hydronephrosis, vomiting and constipation
154
what are 3 investigations of nom-hodgkin's lymphoma?
FBC with differential - thrombocytopenia, pancytopenia, lymphocytosis blood smear excision lymph node biopsy
155
How is nodal spread in Hodgkin's versus non-Hodgkin's lymphoma?
Contiguous in Hodgkin's lymphoma non contiguous in Non-Hodgkin's lymphoma
156
what is the 2ww criteria for lymphadenopathy?
>6 weeks lymphadenopathy 1+ nodes >2cm diameter Rapidly increasing lymphadenopathy Generalised lymphadenopathy Persistent and unexplained splenomegaly
157
what are 3 differentials for non-hodgkin's lymphoma?
Hodgkin's lymphoma ALL infectious mononucleosis
158
what is the management of non-hodgkin's lymphoma?
chemo - R-CHOP - Rituximab - cyclophosphamide - Doxorubicin - Vincristine - prednisolone radiotherapy Rituximab stem cell transplant
159
what are 3 complications of non-hodgkin's lymphoma?
impaired immunity myelosuppression and neutropenic sepsis tumour lysis syndrome risk of secondary malignancy
160
what are 2 causes of relative polycythaemia?
Dehydration Stress - Gaisbock syndrome
161
what are 4 secondary causes of polycythaemia?
COPD Altitude OSA Excessive EPO - cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids
162
what is Polycythaemia vera?
a myeloproliferative disorder of clonal proliferation of erythrocytes often accompanied by overproduction of neutrophils and platelets
163
what genetic mutation if associated with Polycythaemia vera?
JAK2 mutation - 95% of patients
164
what are 7 presentations of Polycythaemia vera?
Headache Pruritus - especially after hot bath splenomegaly Facial flushing Palmar erythema Plethroic appearance Hypertension
165
what are 4 first line investigations of Polycythaemia vera?
FBC - elevated Hb, neutrophils, basophils, platelets U+E +LFTS Serum ferritin JAK2 mutation
166
what are 4 treatments of Polycythaemia vera?
1 - Venesection Aspirin 75mg OD - reduce risk of thrombotic events Hydroxycarbamide - 1st line cytoreductive to reduce thrombosis risk in high risk Ruxolitinub - 2nd line cytoreductive
167
what are 5 complications of Polycythaemia vera?
Thrombosis Haemorrhage Leukaemia - 1-3% risk of progression Treatment induced leukaemia Myelofibrosis
168
what is myelofibrosis?
myelodysplastic disorder thought to be due to hyperplasia of abnormal meakaryocytes. Characterised by bone marrow fibrosis and extramedullar haematopoiesis often with splenomagaly
169
what is seen on FBC in myelofibrosis?
Anaemia high WCC High platelets
170
what can be seen on blood film in myelofibrosis?
Tear drop poikilocytes
171
what are 6 presentations of myelofibrosis?
Severe fatigue Hepatosplenomegaly B symptoms Thromboembolic events Unexplained bleeding
172
what may be seen on bone marrow biopsy in myelofibrosis?
'dry tap' without aspirate
173
what is the management of myelofibrosis?
Ruxolitinib - JAK 2 inhibitor Hydroxyurea
174
what are 7 complications of myelofibrosis?
Haemorrhage infection portal hypertension splenic infarct gout and kidney stones due to raised urea neurological manifestations thrombotic events osteosclerosis
175
what is essential thrombocytosis?
myeloproliferative disorder of excessive mature platelet production
176
what is the presentation of essential thrombocytosis?
Headache Tingling in hands/feet Fatigue Visiual disturbance bleeding gums, petechiae, easy bleeding Thrombosis Splenomegaly
177
what is seen on peripheral blood smear in essential thrombocytosis?
platelet anisocytosis (different sizes)
178
what is the management of essential thrombocytosis?
Aspirin - thrombosis prophylaxis Hydroxycarbamide Interferon alpha - in younger patients and women of childbearing age
179
what is the inheritance of sickle cell anaemia?
Autosomal recessive point mutation substituting hydrophilic glutamic acid for hydrophobic valine
180
what is the pathophysiology of sickle cell anaemia?
there is a genetic abnormality which affects the gene for beta-globin on chromosome 11 leading to rather than biconcave discoid RBC for them to be sickle shaped and more prone to haemolysis
181
what chromosome if affected in sickle cell anaemia?
chromosome 11
182
what is the screening for sickle cell anaemia?
All babies in newborn blood spot test At risk pregnant women may be offered genetic testing
183
what triggers sickling in sickle cell disease?
hypoxia acidosis dehydration Cold temperatures extreme exercise stress Infections
184
when does sickle cell disease manifest and why?
6 months There is a gradual transition between foetal haemoglobin and adult but by 6 months there is very little HbF produces and most RBCs contain HbA which is mutated to HbS in sickle cell
185
what haemoglobin chain is affected in sickle cell anaemia?
Beta haemoglobin
186
what is foetal haemoglobin made up of?
2 alpha chains and 2 gamma chains
187
what is adult haemoglobin made up of?
2 alpha chains 2 beta chains
188
what is the gold standard investigation for sickle cell anaemia?
haemoglobin electrophoresis
189
what are 3 investigations for sickle cell?
blood film Hb isoelectric focusing peripheral blood smear Newborn Guthrie heel prick test (5 days)
190
what is the general management of sickle cell?
Avoid triggers Vaccination Antibiotic prophylaxis - phenoxymethylpenicillin analgesia oral hydroxycarbamide (increase foetal haem) Crizanlizumab - prevents RBCs from sticking blood transfusions - for anaemia Bone marrow transplant Folic acid suplementation
191
what medication in sickle cell anaemia is used to increase foetal haemoglobin?
Hydroxycarbamide (usually 15mg/kg)
192
what vaccination should those with sickle cell anaemia get every 5 years?
pneumococcal
193
what is the management for sickle cell crisis?
Supportive Low threshold for hospital admission Tx trigger keep warm Good hydration Analgesia
194
what are 4 sickle cell crises?
Vaso-occlusive crisis Splenic sequestration crisis Aplastic crisis Acute chest syndrome
195
what is the management of splenic sequestration crisis in sickle cell anaemia?
blood transfusion IV fluids Splenectomy if recurrent
196
what can be seen on FBC in splenic sequestration crisis?
increased reticulocyte count
197
what virus can trigger aplastic crisis in sickle cell anaemia?
Parvovirus B19
198
what is the presentation of acute chest syndrome?
Hx of sickle cell Chest pain cough SOB fever hypoxia Pulmonary infiltrates on CXR
199
what is the management of Acute chest syndrome?
Analgesia Resp support Abx Transfusion
200
what is the presentation of an aplastic crisis?
Sudden fall in Hb Bone marrow suppression causes reduced reticulocyte count
201
what are 11 complications of sickle cell?
Anaemia Increased risk of infection CKD Sickle cell crisis Acute chest syndrome Stroke Avascular necrosis Pulmonary HTN Gallstones Priapism
202
what are the vitamin K dependant clotting factors?
X, IX, VII, II - 1972
203
can warfarin be used in pregnancy?
NO it's teratogenic
204
what is the name of the histological feature that appears like stacked coins?
Rouleaux formation
205
what disease do you see rouleaux formation in?
multiple myeloma stacked coin appearance of RBCs due to high levels of immunoglobulins causing cells to stick together
206
what are 5 generic signs of anaemia?
pale skin and conjunctiva tachycardia bounding pulse raised resp rate postural hypotension
207
what are 5 signs of iron deficiency anaemia?
koilonychia - spoon shaped nails angular chelitis atrophic glossitis - smooth large tongue Brittle hair and nails Pica
208
what is a sign of haemolytic anaemias?
jaundice
209
what is a sign of thalassaemia?
bone deformities
210
what are 8 investigations for anaemia?
FBC - Hb and MCV Blood film Reticulocyte count Ferritin B12 and folate Bilirubin - up in haemolysis Direct Coombs test - autoimmune haemolytic Haemoglobin electrophoresis
211
what are the 4 iron studies?
serum iron serum ferritin total iron binding capacity (increased in anaemia) transferrin saturation
212
what is total iron binding capacity?
the total space available for ferric ions (Fe3+) to bind to transferrin molecules
213
what is the formula for transferrin saturation?
serum iron / total iron binding capacity
214
what is normal transferrin saturation?
30%
215
what affects ferritin levels?
inflammation can lead to raised serum ferritin => A patient with normal ferritin can still have IDA
216
what is hereditary spherocytosis?
condition where RBCs are sphere shaped making hem fragile and easily destroyed in spleen common in northern europeans
217
what is the inheritance pattern of hereditary spherocytosis?
majority autosomal dominant
218
what are 6 presentation of hereditary spherocytosis?
jaundice anaemia gallstones splenomegally haemolytic crisis - triggered by infection Aplastic crisis - usually triggered by parvovirus
219
what are 2 investigations for hereditary spherocytosis?
EMA binding test Cryohaemolysis test not needed if there is FHx and typical features
220
what is the management of hereditary spherocytosis?
phototherapy or exchange transfusion - neonates with jaundice folic acid supplementation Splenectomy
221
what are 3 complications of hereditary spherocytosis?
gallstones aplastic crisis bone marrow expansion
222
what are 4 precipitators of G6PD deficiency?
Fava beans soy red wine infections
223
what does the urine look like in G6PD deficiency?
tea coloured
224
what is aplastic anaemia?
stem cell disorder characterised by pancytopenia
225
what are 4 causes of aplastic anaemia?
radiation and toxins drugs infections fanconi's anaemia
226
what condition are Howell-jolly bodies seen in?
Sickle cell
227
what is a histological sign go sickle cell disease?
Howell-jolly bodies
228
what is the most common cause of osteomyelitis in sickle cell crisis?
salmonella
229
what do Reed-Sternberg cells look like?
owl eye nuclei
230
what is found in the urine in multiple myeloma?
bench-jones proteins - Ig light chains
231
what is found on the serum electrophoresis in multiple myeloma?
monoclonal paraprotein band
232
what is the treatment of severe malaria?
Artemisinin combination therapy (ACT) | quinine
233
what is the inheritance pattern for haemophilia?
X-linked recessive
234
which factor is there a deficiency in in haemophilia A?
VIII - 8
235
which factor is there a deficiency in in haemophilia B?
IX - 9
236
what are 7 clinical manifestations of haemophilia?
Abnormal, prolonged bleeding Easy bruising (ecchymosis) Spontaneous haemorrhage Muscular haematomas Hemarthrosis Epistaxis GI symptoms
237
what is needed for diagnosis of haemophilia?
Bleeding scores coagulation factor assays genetic testing
238
what clotting time will be prolonged in haemophilia?
activated partial thromboplastic time - aPTT Prothrombin time (PT) will be normal
239
what are 3 differentials for haemophilia?
Von Willebrand disease Platelet dysfunction disorders Scurvy
240
what is the management for haemophilia?
IV infusion of clotting factor desmopressin in mild haemophilia A as promotes vWF Lifestyle changes, RICE for MSK, analgesia, physio
241
what medications should be avoided in haemophilia?
Aspirin and NSAIDs - increase bleeding risk
242
what are 3 complications of haemophilia?
Intracranial haemorrhage Compartment syndrome arthropathy from haemarthrosis
243
what chromosome codes for von willebrand factor?
chromosome 12
244
what are the 3 different types of von willebrand disease?
1 - partial deficiency 2 - reduced function of vWF 3 - complete deficiency
245
what does von willebrand factor do?
Mediated platelet adhesion Stabilises factor VIII
246
what is the presentation of von Willebrand disease?
Bleeding gums epistaxis easy bruising menorrhoagia heavy bleeding FAMILY HISTORY
247
what is the most common bleeding disorder?
Von Willebrand disease
248
what can be used to treat von willebrand disease?
1 - Desmopressin - stimulated release of vWF from endothelial cells OR Tranexamic acid 2 - von Willebrand factor infusion OR Factor VIII + vWF infusion Hysterectomy may be required in very severe menstrual bleeding
249
what is thalassaemia?
a genetic defect in the protein chains that make up haemoglobin leading to haemolytic anaemia
250
what is the inheritance of thalassaemia?
Autosomal recessive
251
what demographic is alpha thalassaemia most common in?
asian and African descent
252
what demographic is beta thalassaemia most common in?
SE asian, Mediterranean and Middle Eastern descent
253
what are 6 manifestations of thalassaemia?
Microcytic anaemia Anaemia symptoms - fatigue, pallor, SOB, palpitations Neonatal Jaundice Gallstones Splenomegaly poor growth and development Chipmunk faces - compensatory extramedullary haematopoiesis in skull
254
what are 4 investigations for thalassaemia?
FBC Iron studies Haemoglobin electrophoresis DNA testing
255
why is there iron overload in thalassaemia?
Increased iron absorption in GI tract Blood transfusions
256
what are 6 complications of iron overload?
Liver cirrhosis Hypogonadism Hypothyroidism Heart failure Diabetes Osteoporosis
257
defects on what chromosome cause alpha thalassaemia?
chromosome 16
258
what can all 4 alpha globulin chains in alpha thalassaemia cause?
in utero death
259
what are the 3 different severities of beta thalassaemia?
thalassaemia minor (trait) thalassaemia intermedia thalassaemia major
260
what is the clinical picture in beta thalassaemia minor?
carriers with mild microcytic anaemia - may have microcytosis without anaemia
261
what is the clinical picture in beta thalassaemia intermedia?
2 defective or 1 defective and 1 deletion gene leads to significant microcytic anaemia - may need occasional blood transfusion
262
what is the clinical picture in beta thalassaemia major?
homozygous for deletion genes Severe anaemia and failure to thrive in early childhood May have bone changes
263
what are 4 bone changes in thalassaemia major?
Frontal bossing enlarged maxilla depressed nasal bridge protruding upper teeth
264
what can be given to thalassaemia patients to prevent iron overload?
deferoxamine
265
what are 5 complications of thalassaemia?
Heart failure hypersplenism aplastic crisis iron overload gallstones
266
when are people screened for thalassaemia?
all women in pregnancy
267
what can reverse heparin overdose?
protamine sulphate
268
what is the antidote to warfarin?
vitamin K1
269
What is the Philadelphia chromosome?
Translocation of a part of chromosome 9 to chromosome 22
270
is primaquine safe in pregnancy?
NO
271
what is given as tumour lysis prophylaxis in AML?
allopurinol
272
what are the histological signs of G6PD?
bite cells Heinz bodies reticulocytes
273
what antibiotic is contraindicated in G6PD?
trimethoprim
274
What leukaemia is tumour lysis syndrome most common in?
AML
275
what cells are seen in CLL?
smudge cells
276
what cells are seen in ALL?
blast cells
277
what is antiphospholipid syndrome?
acquired autoimmune disease that causes thrombophilia - blood to be hypercoagulable. increases risk of PE, DVT, Stroke, MI and MISCARRIAGE
278
what autoimmune condition is associated with antiphopholipid syndrome?
SLE - 30% of people with SLE have antiphospholipid antibodies
279
what skin finding can be seen in antiphospholipid syndrome?
livedo reticularis lacey, net like discolouration - like mottling
280
what 3 antibodies cause antiphospholipid syndrome?
anticardiolipin antibodies Lupus anticoagulant Anti-beta-2 glycoprotein I antibodies
281
what is seen on clotting profile in antiphospholipid syndrome?
normal PT paradoxical prolonged APTT thombocytopenia is also common
282
what is the management of antiphospholipid syndrome?
Primary prophylaxis - low dose Aspirin Secondary prevention - Lifelong warfarin INR 2-3 LMWH + high dose Aspirin in pregnancy If thromboembolic events despite warfarin - target increased to 3-4
283
what is the inheritance pattern of haemochromatosis?
autosomal recessive mutations of HFE gene on both copies of chromosome 6
284
what is haemochromatosis?
genetic condition due to mutation of human haemochromatosis protein gene on chromosome 6 causing iron overload
285
what are 8 presentation so of haemochromatosis?
Chronic tiredness Joint pain Pigmentation of skin testicular atrophy erectile dysfunction amenorrhoea cognitive symptoms hepatomegaly usually presents >40 years can be later in women
286
what are 6 causes of raised serum ferritin?
haemochromatosis infection chronic alcohol consumption non-alcoholic fatty liver disease hepatitis c cancer
287
what 2 investigations can be used for haemochromatosis?
Iron studies - transferrin saturation raised, raised ferritin, low total iron binding capacity Genetic testing for HFE mutation
288
what can be used to establish iron concentration in liver in haemochromatosis?
liver biopsy with perl's stain - helps stage fibrosis and exclude other liver pathology
289
what are 7 complications of haemochromatosis?
secondary diabetes liver cirrhosis endocrine and sexual problems - hypogonad, ED, amenorrhoea, reduced fertility Cardiomyopathy and heart failure Hepatocellular carcinoma Hypothyroidism Chondrocalcinosis - calcium pyrophosphate deposits in joints
290
what is the management of haemochromatosis?
1- Venesection - initially weekly - keep transferrin saturation <50% and serum ferritin <50 ug/l 2 - deferoxamine - iron chelation
291
what stem cell do all blood cells differentiate from?
Multipotential haematopoietic stell cell - hemocytoblast
292
what 2 cells does the multipotential haematopoietic stem cell differentiate into first?
Common myeloid progenitor Common lymphoid progenitor
293
what 2 cells does the common lymphoid progenitor differentiate into?
Natural killer cell - large granular lymphocyte small lymphocyte
294
what 2 cells does the small lymphocyte cell differentiate into?
T lymphocyte b lymphocyte
295
what do B lymphocytes differentiate into?
Plasma cells Memory b lymphocytes
296
what is the role of plasma cells?
Produce antibodies against specific antigens
297
what 4 cells does the common myeloid progenitor cell differentiate into?
Megakaryocyte Erythrocyte Mast cell Myeloblast
298
what do megakaryocytes differentiate into?
Platelets
299
what 4 cells do myeloblasts differentiate into?
Basophils Neutrophils Eosinophils Monocytes
300
what do monocytes differentiate into?
Macrophages
301
What are 6 blood transfusion reactions?
Non-haemolytic febrile reaction Minor allergic reaction Anaphylaxis Acute haemolytic reaction Transfusion associated circulatory overload (TACO) Transfusion related acute lung injury (TRALI)
302
what is acute haemolytic transfusion reaction?
Due to ABO mismatch Causes massive intravascular haemolysis due to RBC destruction by IgM antibodies Symptoms begin minutes after transfusion - fever, abdo and chest pain, agitation, hypotension
303
what is the management and 2 complication of acute haemolytic transfusion reaction?
management - stop transfusion - check patient identity and blood products - Repeat typing and cross matching - Send blood for direct coombs test - generous fluid resus - inform lab complications - DIC - renal failure
304
what causes non-haemolytic febrile reaction?
antibodies reacting with white cell fragments in blood products and cytokines that have leaked in storage causes fever and chills stop/slow transfusion give paracetamol monitor
305
what is the management of minor allergic reaction to blood transfusion?
Temporarily stop transfusion Antihistamines monitor
306
what causes anaphylaxis in blood transfusion?
patient with IgA deficiency who have anti-IgA antibodies
307
what is transfusion associated circulatory overload (TACO)?
Due to fluid overloading - causes pulmonary oedema may also be hypertensive
308
What is the management of transfusion associated circulatory overload?
Slow/stop transfusion Consider IV loop diuretics - furosemide O2
309
what is Transfusion related acute lung injury (TRALI)?
Non-cardiogenic pulmonary oedema secondary to increased vascualr permeability caused by host neutrophil activation by donated blood Causes hypoxia, pulmonary infiltrates on CXR, fever, hypotension - within 6 hours of transfusion
310
what is the management of transfusion related acute lung injury?
Stop transfusion Oxygen and supportive care
311
what blood product is most likely to be contaminated with bacteria?
Platelets as stored at room temp
312
Is prothrombin time affected by the intrinsic or extrinsic coagulation pathway?
extrinsic pathway PT - Play Tennis Outside = extrinsic INR is a standardised version of PT
313
is activated partial thromboplastin time (APTT) affected by the intrinsic or extrinsic coagulation pathway?
intrinsic pathway aPTT = Play Table Tennis inside = intrinsic
314
what factors are involved in the intrinsic coagulation pathway?
XII XI IX VIII 12, 11, 9, 8
315
what factors are involved in the extrinsic coagulation pathway?
III VII 3 + 7
316
what factors are involved in the common coagulation pathway?
X V II I XIII 10, 5, 2, 1, 13
317
what triggers the intrinsic coagulation pathway?
Contact with damaged endothelial surfaces
318
what triggers the extrinsic coagulation pathway?
Tissue factor III activation via endothelial tissue damage to IIIa
319
what is disseminated intravascular coagulation?
simultaneous coagulation and haemorrhage caused formation of thrombi which consume clotting factors (V, VIII) and platelets leading to bleeding
320
what are 5 causes of DIC?
Sepsis Malignancy Trauma - major surgery, burns, shock, AAA dissection Severe organ dysfunction - Liver disease Obstetric complications - abruption, HELLP syndrome
321
what is seen on bloods in DIC?
Low platelets Prolonged aPTT, PT and bleeding time Decreased fibrinogen D-dimer - raised
322
what is the management of DIC?
Tx underlying cause Anticoagulation - low dose heparin - due to microthrombi Blood product transfusion
323
what are 4 complications of DIC?
Intracranial bleed Life threatening haemorrhage Multi-organ failure Gangrene or digital loss - due to microthrombi
324
what is the most common cause of thrombocytopenia?
Immune thrombocytopenia (idiopathic thrombocytopenia purpura)
325
what is thrombocytopenia?
Platelets <150 x10^9/L
326
what is the normal range for platelets?
150-450 x10^9 /L
327
what are 8 causes of thrombocytopenia due to reduced platelet production?
Viral infections - EBV, CMV, HIV B12/folate deficiency Liver failure + alcohol abuse- reduced thrombopoietin production Bone marrow infiltration - leukaemia Myelodysplastic syndrome Chemotherapy Genetics - wiskott-aldrich syndrome, faconi anaemia
328
what are 6 causes of thrombocytopenia due to increased platelet destruction?
Medications - sodium valporate, methotrexate Alcohol Immune thrombocytopenic purpura Thrombotic thrombocytopenia purpura Heparin induced thrombocytopenia Haemolytic uraemic syndrome
329
What low platelet count is usually symptomatic?
<50 x10^9/L
330
At what platelet count is there high risk for spontaneous bleeding?
<10 x10^9/L
331
what are the top 4 differentials for abnormal bleeding?
thrombocytopenia von willebran disease Haemophillia Disseminated intravascular coagulation
332
what is wiskott-aldrich syndrome?
X-linked condition - eczema, thrombocytopenia, immunodeficiency
333
what is faconi aanemia?
inherited bone marrow failure syndrome, characterised by pancytopenia
334
what are 4 drugs that can induce thrombocytopenia?
Heparin Quinine Sulfonamides Naproxen
335
what is immune thrombocytopenia purpura?
Autoimmune condition where autoantibodies are created against platelets leading to their destruction and ultimately thrombocytopenia
336
what is the course of ITP usually in children?
Usually triggered by recent viral infection and resolves typically within 6 months but in adults is usually more chronic
337
what is primary versus secondary ITP?
Primary - no clear cause Secondary - antibody medicated due to SLE, CLL, Drugs, Viruses, Pregnancy
338
How is immune thrombocytopenia purpura diagnosed?
Diagnosis of exclusion 1 - FBC, peripheral blood smear Bone marrow aspiration, blood borne virus serology
339
what is the management of immune thrombocytopenia purpura?
1 - Prednisolone 1mg/kg OD reducing dose Adjunct - IV immunoglobulins With significant bleeding - Platelet transfusion 4-6 units of pooled platelets 2 - Splenectomy - if not responding to medical management Immunosuppression - rituximab
340
what is thrombotic thrombocytopenic purpura?
A microangiopathy where tiny thrombi develop in small vessels, consuming platelets and leading to thrombocytopenia
341
deficiency of what protein causes thrombotic thrombocytopenic purpura?
ADAMTS13
342
what is the role of ADAMTS13?
Inactivated von willebrand factor Reduces platelet adhesion to vessel walls reduces clot formation
343
what can cause ADAMTS13 deficiency?
Inherited genetic mutation Autoimmune disease against protein
344
what is the classic pentad of thrombotic thrombocytopenic purpura ?
Thrombocytopaenia purpura Microangiopathic haemolytic anaemia Neurological dysfunction Renal dysfunction Fever
345
what is the management of thrombotic thrombocytopenic purpura ?
1 - Plasma exchange 1 - orticosteroids 1 - Caplacizumab 2 - Rituximab
346
what is heparin induced thrombocytopenia?
Development of autoantibodies to platelets in response to heparin (either UH or LMWH) Heparin induced autoantibodies target platelet factor 4 protein on platelets
347
what is the presentation of heparin induced thrombocytopenia?
5-10 days post starting heparin HIT antibodies bind to platelets and activate clotting system Hypercoagulability - DVT, PE Thrombocytopenia - bleeding
348
How is heparin induced thrombocytopenia diagnosed?
Testing serum heparin induced thrombocytopenia antibodies
349
what anticoagulation can be used as an alternative to heparin in heparin induced thrombocytopenia?
Fondaparinux or argatroban
350
what is the MOA of aspirin?
COX-1 (and 2) inhibitor Cyclooxygenase is responsible for prostaglandin, prostacyclin and thromboxane synthesis - blocking thromboxane formation in platelets reduces ability to aggregate
351
what is the secondary prevention dose of aspirin?
75mg OD
352
what is the loading dose of aspirin in MI/Stroke?
300mg
353
what are 4 examples of P2Y12 inhibitors?
Clopidogrel Parasugrel Ticagrelor Ticlopidine
354
what medications can reduce the efficacy of clopidogrel?
PPIs - especially omeprazole and esomeprazole
355
what is the reversal agent for ticagrelor?
Bentracimab
356
what is the MOA of warfarin?
Vitamin K antagonist
357
what is the normal warfarin target?
Between 2-3 Aim 2.5
358
what are 5 contraindications to warfarin?
liver disease p450 enzyme inhibitors/inducers cranberry juice drugs which displace warfarin from plasma albumin - NSAIDs Drugs that inhibit platelet function
359
what are 7 cytochrome P450 inducers?
Antiepileptics - phenytoin, carbamezapine Barbiturates -phenobarbitone Rifampicin St johns wart chronic alcohol intake griseofulvin Smoking
360
what are 11 p450 inhibitors?
Antibiotics - ciprofloxacin, erythromycin Isoniazid Omeprazole Amiodarone Allopurinol Imidazoles - ketoconazole, fluconazole SSRIs Ritonavir sodium valporate quinupristine
361
what is the MOA of LMWH and heparin?
Activates antithrombin III - forms complex that inhibits factor Xa
362
what are 3 adverse effects of LMWH and heparin?
Thrombocytopenia osteoporosis hyperkalaemia
363
what is the antidose for LMWH and heparin?
Protamine sulphate
364
how is anticoagulation with heparin monitored?
activated partial thromboplastin time - APTT
365
what is the reversal agent for dabigatran?
Idarucizumab
366
what is the reversal agent for DOACs?
AdeXanet alfa Xa - what DOACs inhibits
367
what is the MOA of fondaparinux?
Activates antithrombin III which inhibits coagulation factor Xa
368
what is the MOA of DOACs?
Direct factor Xa inhibitors
369
What is the MOA of Dabigatran?
direct thrombin inhibitor
370
what increases the efficacy of riveroxaban?
taking it with food
371
what are 3 scenarios where dose reduction may be required in DOAC prescribing?
Elderly - >75-80 years Reduced body weight Severe renal impairment - 15-50 ml/min creatinine clearance
372
what is pancytopenia?
Reduction in all 3 major cellular components of blood RBCs <120 (F) OR <130 M WBCs <4 Platelets <150
373
what are 6 production causes of pancytopenia?
Vitamin deficiency - severe B12, folate or iron deficiency Aplastic anaemia Myelodysplastic syndromes Bone marrow infiltration - cancers Viral infections - HIV, parvovirus B19, CMV, EBV
374
what are 2 causes of pancytopenia due to increased destruction?
Splenic sequestration - sickle cell, cirrhosis, malaria, TB Autoimmune conditions - SLE, RhA
375
what are 8 risk factors for pancytopenia?
Age - children and older adults Male Autoimmune disease Recent viral infection Alabsorption syndromes FHx of aplastic anaemia Hx of cancer drugs/toxins
376
what are 5 presentations of pancytopenia?
Fatigue B symptoms recurrent infections epistaxis Pallor petechia splenomegaly
377
what is aplastic anaemia?
Bone marrow hypocellularity secondary to primary haematopoietic failure Causes pancytopenia - Normochromic normocytic anaemia Thrombocytopenia leukopenia
378
what is the most common hereditary cause of aplastic anaemia?
Fanconi anaemia
379
what is the inheritance of fanconi anaemia?
autosomal recessive
380
what is the most common cause of aplastic anaemia?
idiopathic
381
what are 6 drugs that can cause aplastic anaemia?
Carbimazole Carbamezapine and phenytoin Chloramphenicol Chemo Benze chemicals Radiation
382
what is seen on bone marrow biopsy in aplastic anaemia?
Hypocellular one marrow with fat cells and fibrosis replacing normal bone marrow
383
what is the management of aplastic anaemia?
blood products and prevention and treament of infections Anti-thymocyte globulin and anti-lymphocyte globulin Stem cell transplant
384
what is the difference between petechiae, purpura and ecchymosis?
petechiae = <3mm - burst capillaries Purpura = 3-10mm Ecchymosis = >1cm
385
what 4 electrolyte disturbances are seen in tumour lysis sydrome?
High uric acid Hyperkalaemia Hyperphosphatemia Hypocalcaemia
386
Treatment of what cancers can cause tumour lysis syndrome?
Leukaemia Lymphomas
387
what can be used as prevention for tumour lysis syndrome?
IV fluids Allopurinol or rasburicase
388
what is clinical tumour lysis syndrome?
Lab finding of tumour lysis syndrome PLUS increased serum calcium, cardiac arrythmia/sudden death or seizure
389
what 4 things are present in cryopricipitate?
Factor VIII Factor XIII Fibrinogen vWF
390
what is a normal neutrophil count?
2-7.5 x10^9
391
what is classes as neutropaenia?
<1.5
392
what is classed as mild neutropenia?
1-1.5
393
what is classed as moderate neutropenia?
0.5-1
394
what is classed as severe neutropenia?
<0.5
395
what are 8 causes of neutropenia?
Viral - HIV, EBV, hepatitis Drugs Benign ethnic neutropenia Haematological malignancy - myelodysplastic, aplastic anaemia SLE RhA - hypersplenism Severe sepsis Haemodialysis
396
what is the most common cause of neutropenic sepsis?
Coagulase negative, gram positive bacteria - staphylococcus epidermis most common
397
what is neutropenic sepsis?
neutrophils <0.5 usually in a patient 7-14 days post chemo with a temp >38 and other presentations of sepsis
398
what antibiotics are used as neutropenic sepsis prophylaxis?
fluoroquinolones - ciprofloxacin
399
what is the management of neutropenic sepsis?
start Abx immediately IV - piperacillin with tazobactam Alternative abx if still pyrexial at 48 hours - meropenem, vancomycin If still unresponsive, investigate fungal infection
400
what is the most common cause of thrombophilia?
factor V leiden
401
what are 7 examples of thrombophilias?
Factor V leiden - most common Prothrombin gene mutation - second most common Protein C deficiency Protein S deficiency Antithrombin III deficiency Antiphospholipid syndrome - acquired
402
what is factor V leiden also known as?
activated protein c resitance
403
what is the pathophysiology of factor V leiden?
Mis-sense mutation in activated factor V leading to inactivation to be 10x slower than normal by activated protein C
404
what are the 2 different types of factor V leiden?
heterozygous - more common, 4-5x more likely VTE homozygous - less common - 10x more likely VTE
405
What additional treatment needs to be given in p.ovalae and p. Vivax malaria?
Primaquine - to prevent liver hypnozoites and therefore prevent relapse
406
What additional treatment needs to be given in p.ovalae and p. Vivax malaria?
Primaquine - to prevent liver hypnozoites and therefore prevent relapse
407
what kind of mosquitoes transmit dengue?
Aedes aegypti mosquito
408
what is the incubation period for dengue?
7 days
409
what is the presentation of dengue fever?
Fever Headache - retro-orbital Myalgia, bone pain, arthralgia - break bone fever Pleuritic pain facial flushing maculopapular rash haemorrhagic manifestation - petechia, purpura May also have abdo pain, hepatomegaly, vomiting
410
what is the severe form of dengue?
dengue hemorrhagic fever DIC - thrombocytopenia, spontaneous bleeding
411
what is the diagnosis of dengue fever?
Dengue serology NAAT for viral RNA NS1 antigen test

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