Haematology Flashcards

(81 cards)

1
Q

Causes of microcytic anaemia

A

iron deficiency - diet, malabsorption or blood loss
thalassaemia

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2
Q

causes of normocytic anaemia

A

chronic disease
acute blood loss
renal failure - EPO deficient
RBC destruction

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3
Q

causes of macrocytic anaemia

A

B12 def
Folate def
pregnancy
alcohol
hypothyroidism
aplastic anaemia
myeloma
liver disease

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4
Q

what can cause haemolytic anaemias

A

note these are rare
- congenital; membrane, enzyme (G6PD) of Hb disorders of RBCs
- acquired; wilson’s disease, autoimmune, drugs

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5
Q

management of beta thal major

A

long term blood transfusions + iron chelation

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6
Q

tests for haemolysis

A

Coomb’s test:
1. indirect antiglobulin test - to cross match blood
2. Direct Antiglobulin Test - detect patients own antibodies to their red cells

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7
Q

mechanism of action and reversal of dabigatran

A

direct thrombin inhibitor
idarucizumab to reverse

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8
Q

universal FFP donor

A

AB RhD negative

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9
Q

what is lymphoma? how does it normally present

A

normal lymphoid structure replaced by malignant cells
bimodal ages; younger then in older age
Smooth, firm lymph nodes, dry cough, tiredness and B symptoms (weight loss, night sweats and fever)

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10
Q

types of lymphoma

A

hodgkins; reed-sternberg cells

non-hodgkins:
- diffuse large B cell (high grade)
- follicular NHL (low grade)
- T cell

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11
Q

lymphoma staging system

A

Ann Arbour
stage 1 - one group of nodes
stage 2 - 2 or more groups but one side of the diaphragm
stage 3 - both sides of the diaphragm
stage 4 - organ involvement (bone, liver, lungs, spleen)

A or B is also assigned based on whether B symptoms present

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12
Q

types of leukaemia

A

Myeloid - acute or chronic
Lymphocytic - acute or chronic

acute - ‘blasts’ on blood film. Pt is anaemic +/- thrombocytopenic. bone marrow failing

chronic - mature cells seen in excess numbers but are malignant and abnormal

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13
Q

what is multiple myeloma?
what symptoms and signs?

A

malignancy arising from the antibody producing plasma B cells
Calcium raised with normal phosphate and ALP
Renal impairment
Anaemia
Bone pain - often back pain. osteolytic lesions in bone

bence jones proteins in urine and plasma

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14
Q

what does prothrombin time measure? what factors affect it?

A

extrinsic pathway
factors, II, V, VII X
warfarin also affects it

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15
Q

what does activated partial thromboplastin time (APTT) measure? what factors/things affect it?

A

intrinsic pathway
factors VIII, IX, XI, XII
also heparin and von willebrand deficiency

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16
Q

what causes both PT and APTT to be prolonged?

A

vitamin K deficiency
disseminated intravascular coagulation

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17
Q

what is the premise of the 50:50 plasma test?

A

mix 50% patients plasma and 50% normal
if problem is a factor deficinecy then clotting times will correct
if an inhibitor is present then it wont correct

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18
Q

Haemophilia A and B
what are they?
management?

A

A = factor VIII deficiency
B = factor IX deficiency

A; give factor VIII 3x daily. amount depends on severity
also can give desmopressin

B; give factor IX 1x daily

both can give tranexamic acid in a bleed

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19
Q

CLL complications

A
  • anaemia
  • hypogammaglobulinaemia leading to recurrent infections
  • warm autoimmune haemolytic anaemia in 10-15% of patients
  • transformation to high-grade lymphoma (Richter’s transformation)
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20
Q

management of essential thrombocytosis

A

hydroxyurea

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21
Q

what is von willebrands disease?
symptoms
types

A

inherited bleeding disorder of reduced vWF which is a carrier for factor VIII and promotes platelet adhesion
epistaxis and menorrhagia are common symptoms

type 1 - partial reduction (80% of patients)
type 2 - abnormal vWF
type 3 - no vWF

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22
Q

investigation results in von willebrands disease
management

A

prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation

management: Tranexamic acid for bleeds, desmopressin, factor VIII concentrate

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23
Q

causes and blood results of Disseminated Intravascular Coagulation

A

causes:
- trauma
- sepsis
- obstetric complications
- malignancy

blood results:
decreased platelets and fibrinogen
increased APTT, PT

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24
Q

long term management of sickle cell disease

A

hydroxyurea
pneumococcal vaccine every 5 years

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25
management of a sickle cell crisis
analgesia e.g. opiates rehydrate oxygen consider antibiotics if evidence of infection blood transfusion exchange transfusion: e.g. if neurological complications
26
blood transfusion complications
-immunological: acute haemolytic, non-haemolytic febrile, -allergic/anaphylaxis -infective -transfusion-related acute lung injury (TRALI) -transfusion-associated circulatory overload (TACO) -other: hyperkalaemia, iron overload, clotting
27
typical presentation of CML
60-70 years old anaemia --> lethargy drenching night sweats massive splenomegaly weight loss leukocyte alkaline phosphatase thrombocytosis and lymphocytosis
28
treatments of: CML CLL NHL
CML = imatinib CLL = Fludarabine, cyclophosphamide and rituximab (FCR). second line is ibrutinib NHL = Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone (R-CHOP) is used in the treatment of Non-Hodgkin's lymphoma (NHL).
29
ALL presentation
typically 2-5 years old anaemia and splenomegaly increased lymphocytes, + neutropenia and thrombocytopenia
30
what is the preferred anticoagulant if renal function is impaired?
apixaban
31
Iron profile in haemochromatosis
Raised transferrin and ferritin. Low total iron binding capacity
32
threshold for transfusion in anaemia
gernerally <70g/l in ACS <80g/L
33
symptoms and treatment for immune thrombocytopenia
more common in females. presents with petechiae, epistaxis and bleeding gums first line treatment is oral prednisolone
34
test for autoimmune haemotlyic anaemia
direct coombs test
35
polycythaemia vera what is it risks treatment prognosis
myeloproliferative disorder caused by clonal proliferation of a marrow stem cell where all cell counts are raised particularly RBCs increased risk of thrombotic events - stroke and MI patients should be on low dose aspirin venesection to reduce Hb chemotherapy can be beneficial thrombotic events common 5-15% of patients progress to myelofibrosis 5-15% of patients progress to acute leukaemia
36
Which clotting factors are affected by heparin
Prevents activation of factors 2, 9, 10, 11
37
What clotting factors are affected by warfarin
2, 7, 9, 10
38
management of anti-phospholipid syndrome in pregnancy
low dose aspirin once pregnancy confirmed on urine testing LMWH once fatal heart on ultrasound then discontinue at 34 weeks
39
general features of autoimmune haemolytic anaemia
Normocytic anaemia Reticulocytosis Raised LDH and bilirubin
40
management of autoimmune haemolytic anaemia
warm; steroids +/- rituximab cold; responds less well to steroids
41
how does beta thal major present
in first year of life with failure to thrive and hepatosplenomegaly
42
what is desferrioxamine
iron chelation therapy
43
in what conditions are 'target cells' seen on blood film
Sickle-cell/thalassaemia Iron-deficiency anaemia Hyposplenism Liver disease
44
in what conditions are 'tear-drop' poikilycytes seen on blood film
myelofibrosis
45
in what conditions are 'spherocytes' seen on blood film
Hereditary spherocytosis Autoimmune hemolytic anaemia
46
in what conditions are 'basophilic stippling' seen on blood film
Lead poisoning Thalassaemia Sideroblastic anaemia Myelodysplasia
47
in what conditions are Howell-Joly bodies seen on blood film
hyposplenism
48
in what conditions are Heinz bodies seen on blood film
G6PD deficiency Alpha-thalassaemia
49
in what conditions are Schistocytes ('helmet cells') seen on blood film
Intravascular haemolysis Mechanical heart valve Disseminated intravascular coagulation
50
in what conditions are pencil poikilocytes seen on blood film
iron deficiency anaemia
51
in what conditions are Burr cells seen on blood film
Uraemia Pyruvate kinase deficiency
52
types of blood transfusion reaction
Immunological Infective Transfusion Related Lung Injury Transfusion Associated Circulatory Overload
53
Non Haemolytic febrile reaction to blood transfusion
Fever and chills slow or stop the transfusion, paracetamol and monitor
54
minor allergic reaction to blood products
pruritus and urticaria temporarily stop the transfusion, give antihistamines and monitor
55
anaphylaxis reaction to blood products
hypotension, dyspnoea, wheezing, angioedema stop the transfusion, IM adrenaline and A-E support
56
Acute haemolytic reaction to blood products
ABO incompatibility - human error Fever, abdominal pain, hypotension Stop transfusion , supportive care
57
Transfusion associated circulatory overload
Excessive rate of transfusion More likely in pre existing heart failure Pulmonary oedmea and hypertension Slow or stop transfusion. Consider IV loop diuretic
58
Transfusion-related acute lung injury
Hypoxia, pulmonary infiltrates on CXR, fever, hypotension Stop transfusion. Oxygen and supportive care
59
Red blood cell transfusion thresholds and targets
Without ACS: threshold 70g/L Target 70-90 With ACS: threshold 80 g/L Target 80-100
60
Richter’s transformation presentation.
CLL transformation to a high grade non-Hodgkin lymphoma Presents with rapidly growing lymph node, fever, weight loss, night sweats, nausea, abdo pain
61
CLL investigation findings
Leukocytosis Anaemia Thrombocytopenia Smudge/smear cells on blood film Immunophenotyping is done
62
Next steps if DVT likely on two level wells score
Ultrasound the leg - if DVT present start anticoagulation If USS negative do a D-dimer If negative DVT unlikely If positive then re scan in a few days and monitor If can’t USS within 4 hours then anticoagulate in the meantime
63
Next steps if DVT unlikely on two level wells score (1 point or less)
D dimer within 4 hours - if positive then USS leg within 4 hours (anticoagulate if longer) - if negative DVT unlikely
64
Choice of anticoagulant in DVT
DOACs from start in most patients (rivaroxaban or apixaban first line) If not suitable LMWH followed by warfarin E.g. in antiphospholipid syndrome or severe renal impairment
65
acute haemolytic reaction to blood products
ABO incompatibility - human error fever, abdominal pain and hyptension stop transfusion
66
Factor V Leiden
Activated protein C resistance Most common form of inherited thrombophilia
67
G6PD deficiency - summary of condition - features - diagnostic test
Most common red blood cell enzyme defect Most common in people of Mediterranean and African descent X linked Can be precipitated by some drugs, infection, and broad/fava beans Intravascular haemolysis Neonatal jaundice Gallstones common Heinz bodies on film Measure enzyme activity of G6PD
68
Hereditary spherocytosis - summary - features - test
Autosomal dominant inheritance More common in people of Northern European descent Neonatal jaundice Chronic symptoms of haemoylsis Gallstones Splenomegaly Spherocytes on film Diagnose with EMA binding test
69
Features of haemophilia
X linked Haemarthroses Haematoma Prolonged bleeding after trauma or surgery Prolonged APTT Bleeding time, PT, thrombin time all normal
70
Immune thrombocytopenia in adults
Immune mediated reduction in the platelet count More common in older females May be detected on routine blood tests or present with petechiae, purpura, bleeding Management is oral prednisone or IVIG if active bleeding
71
Iron studies in iron deficiency anaemia
Hb low Ferritin low (but inflammation can raise it so high ferritin does not rule out IDA) TIBC/transferrin high Transferrin saturation low
72
Management IDA
Identify cause - may need further investigations e.g. endoscopy (new onset in older people always rule out malignancy) oral ferrous sulphate until iron restored and then for 3 more months Iron rich diet
73
Iron studies anaemia of chronic disease
Serum iron low but not as low as IDA TIBC low Transferrin saturation low Ferritin high
74
What is a decrease in haptoglobin associated with
Intravascular haemolysis
75
Platelet transfusion risk
Highest risk of bacterial contamination
76
DVT or PE in pregnancy
Warfarin contraindicated Give SC LMWH
77
Sideroblastic anaemia
Microcytic hypochromic anaemia High iron ferritin and transferrin Can be congenital or acquired
78
Causes of massive splenomegaly
Myelofibrosis CML Malaria Gauchers syndrome
79
What is hydroxycobalamin
Vitamin B12 replacement Given IM
80
Causes of neutropenia
Viral Drugs e.g. cytotoxics, carbimazole Benign ethnic neutropenia - common in black African or Afro Caribbean Haematological malignancy Rheumatological conditions Severe sepsis
81
Correcting a mixed B12 and folate deficiency
Correct B12 first with IM injections then start oral folic acid once normal