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Haematology Flashcards

1
Q

what is the mode of inheritance for G6PD deficiency?

A

X-linked recessive

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2
Q

what is the role of G6PD enzyme?

A

protects red blood cells from oxidative stress

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3
Q

what is thrombocytopenia?

A

deficiency of platelets in blood

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4
Q

triad of haemolytic uraemic syndrome

A

AKI, anaemia and thrombocytopenia

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5
Q

commonest cause of haemolytic uraemic syndrome in children?

A

E.coli 0157

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6
Q

what is haemolytic anaemia?

A

premature destruction of red blood cells

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7
Q

hereditary haemolytic anaemia conditions

A

sickle cell, thalassaemias, hereditary spherocytosis

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8
Q

acquired haemolytic anaemia conditions

A

autoimmune disorders, infections e.g. malaria, medication reactions or trauma

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9
Q

Describe intravascular haemolysis and the pathophysiology of it

A

Haemolysis occurs in bloodstream
leads to release of free haemoglobin
-excess dealt with:
combo with haptoglobin/ albumin
lost in urine
stored in epithelial cells

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10
Q

Describe extravascular haemolysis and the pathophysiology of it

A

haemolysis taking place outside bloodstream
-mainly in spleen and liver -> RBCs= phagocytosed
splenomegaly and hepatomegaly are typical

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11
Q

what type of anaemia does anaemia of chronic disease present with

A

microcytic or normocytic

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12
Q

what are pencil cells associated with?

A

iron deficiency anaemia
(mainly when caused by coeliac disease)

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13
Q

what are Howell-Jolly bodies?

A

remnants of RBC nuclei
* they’re Jolly cause they have extra stuff to others *

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14
Q

what do Howell-Jolly bodies indicate?

A

functional hyposplenism - spleen not functioning or true - splenectomy

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15
Q

What is DIC? and what do blood results look like?

A

disseminated intravascular coagulation
consumption of clotting factors
blood results
- thrombocytopenia
- schistocytes
- raised d-dimer (fibrin degradation product)
- clotting profile = increased prothrombin time and APTT, decreased fibrinogen

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16
Q

what is ferritin?

A

acute phase protein- increases in response to infection

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17
Q

iron studies of anaemia of chronic disease

A

low serum iron - inhibition of iron absorption
high ferritin -
low transferrin - reduced ability

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18
Q

what is the blood universal donor?

A

O-

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19
Q

What blood can rhesus +ve patients receive?

A

Rh -ve AND +ve

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20
Q

fatigue, headache and itchy after hot bath?

A

polycythaemia rubra vera

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21
Q

what should be given to patient on warfarin experiencing significant bleed?

A

Prothrombin complex concentrate (PTCC)- reverses anticoagulation and contains factors 2, 7, 9 and 10

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22
Q

which test confirms the diagnosis of Haemophilia A?

A

factor VIII assay

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23
Q

NHS management of neutropenic sepsis?

A

IV piperacillin with tazobactam (broad spectrum asap)

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24
Q

diagnosis of chronic lymphocytic leukaemia

A

elevated white cell count with absolute lymphocytosis
-smudge cells on blood film

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25
mot common hereditary thrombophilia
Factor V leiden
26
acquired thrombophilias
anti-phospholipid syndrome
27
what is myelofibrosis
myeloproliferative disorder often presenting with pancytopenia
28
signs and symptoms of myelofibrosis
weight loss night sweats splenomegaly marrow failure signs: poikilocytes, dry tap on bone marrow aspiration
29
management of major bleed and INR>8
stop warfarin, give vit K and prothrombin complex concentrate
30
complication of essential thrombocytosis
ischaemic stroke
31
investigations for tumour lysis syndrome
ECG, U&E, calcium, uric aid
32
primary part of coagulation that heparin exerts action on
activation of antithrombin III - inactivation of thrombin -> inhibits formation of stable blood clots
33
type of reaction associated with immediate, severe reaction following blood transfusion
type II hypersensitivity - involves IgG/ IgM
34
what factor initiates the coagulation cascade?
factor XII
35
deficiency in what factor results in Haemophilia A
factor VIII
36
what feature may be present in patient with excessive loss of plasma proteins
increased susceptibility to infection
37
what blood type can receive donations from all blood types
AB positive
38
multiple myeloma symptoms
CRAB - high Calcium, Renal insufficiency, Anaemia and Bone lesions
39
high total iron binding capacity with low ferritin and low serum iron
iron deficiency anaemia
40
low total iron binding capacity with normal/high ferritin and normal serum iron
anaemia of chronic disease
41
management of essential thrombocythemia
low risk - (<40 y/o AND no history of thrombosis, no cardiovascular risk factors, platelet count <1500) = aspirin only high risk - (> 60 OR previous history of thrombosis, diabetes, hypertension, platelet count >1500) = hydroxycarbamide and aspirin
42
what are typical findings for myelofibrosis?
blood film analysis- tear drop poikilocytes (blood cells squeeze out of fibrotic marrow) bone marrow aspiration- dry tap
43
which clotting factor is most likely affected in patient with Von Willebrand disease?
factor 8 - Von Willebrand promotes platelet adhesion to damage blood vessels and stabilises factor 8 *remember - V III = V W *
44
what is a common finding following acute traumatic event e.g. surgery ?
neutrophilia
45
Massive splenomegaly and weight loss
chronic Myeloid leukaemia
46
management of chronic myeloid leukaemia?
Imatinib - tyrosine kinase inhibitor -> inhibits the activity brought on by the Philadelphia chromosome
47
prolonged PT is most likely caused by which factor deficiency?
factor VII
48
APTT measures speed of clotting via which pathway? what factors are involved?
intrinsic and common pathways Intrinsic = Factors VII, IX, XI and XII common = I, II, V and X *remember- APTT = play table tennis INSIDE = intrinsic *
49
PT measures the speed of clotting via which pathway? what factors are involved?
extrinsic and common pathway Extrinsic= III and VII common = I, II, V and X *remember - PT = play tennis OUTSIDE = extrinsic*
50
typical findings for antiphospholipid syndrome
miscarriage, leg rash, raised APTT, low platelets anticardiolipin antibodies
51
typical presentation of fever, headache, thrombocytopenia, haemolytic anaemia and renal failure with schistocytes on blood film
thrombotic thrombocytopenic purpura
52
Stage 1 Hodgkin lymphoma
single lymph node involvement
53
stage II Hodgkin's lymphoma
2 or more lymph node/ regions on the same side of the diaphragm
54
stage III Hodgkin's lymphoma
nodes on both sides of the diaphragm
55
stage IV Hodgkin's lymphoma
spread beyond the lymph nodes
56
rubbery lymph nodes and 'starry sky' appearance on biopsy
Burkitt's lymphoma - type of non-hodgkin's lymphoma
57
most worrying sign of blood transfusion reaction
hypotension - indicating anaphylaxis, acute haemolytic reaction or transfusion-related acute lung injury
58
What conditions does polycyteamia rubra vera have potential to transform into
Myelofibrosis or Acute Myeloid Leukaemia
59
Duration of anticoagulation treatment following a provoked or unprovoked VTE
provoked = 3 months unprovoked = 6 months
60
how to distinguish between intramedullary and extramedullary haemolysis
extramedullary- leads to release of protoporphyrin (unconjugated bilirubin) symptoms = jaundice and gall stones intramedullary - leads to fee Hb in circulation => reduced haptoglobin symptoms= pink urine
61
examples of intramedullary haemolysis
G6PD deficiency alloimmune haemolysis
62
H.pylori eradication treatment
omeprazole, amoxicillin and vancomycin (associated with MALT - a type of Non-Hodgkin's lymphoma)
63
what is the definitive diagnostic test for sickle cell anaemia
haemoglobin high performance liquid chromatography of whole blood lysate (HPLC)
64
management of polycythemia vera
low risk - aspirin high risk - hydroxycarbamide
65
what is factor V leiden most likelt to present with
pulmonary embolism or DVT (they are LAIDen down with coagulants )
66
Immunoglobulin involved in warm autoimmune haemolytic anaemia
IgG (warm weather is Great)
67
Immunoglobulin in cold autoimmune haemolytic anaemia
IgM (cold weather is Miserable)
68
disproportionate microcytic anaemia
think beta thalassaemia trait
69
what does neutrophilia tend to indicate?
bacterial infection other main causes: - trauma - surgery - necrosis - haemorrhage less commonly - corticosteroid use - myeloproliferative disease
70
what are protein C and S
serum anticoagulants
71
what does warfarin do to protein C and S
reduces its concentration - period of time (1-2 days) after starting warfarin that patient is procoaguable - particularly if have Protein C or S deficiency
72
what virus can precipitate an aplastic crisis in a patient with sickle cell disease
parvovirus B19 infection -causes slapped cheek syndrome
73
what is most common process that causes pernicious anaemia
autoimmune destruction of parietal cells in stomach that secrete intrinsic factor -> unable to absorb vitamin B12 in ileum
74
investigation findings for osteomalacia
low calcium , low phosphate and high ALP
75
what is the most appropriate long-term prophylactic in a patient with sickle cell anaemia who has repeated painful crises
daily oral hydroxyurea
76
new onset sever microcytic anaemia at 3-9 months old with frontal bossing
beta thalassaemia major
77
history of petechiae, thrombocytopenia, normal APTT and PT
typical of Immune thrombocytopenic purpura
78
most common inherited thrombophilia
factor V Leiden
79
what blood test result would indicate that heparin treatment needs to be withdrawn
drop in platelets by more than 30% - heparin induced thrombocytopenia
80
presentation of beta thalassaemia trait
usually asymptomatic - microcytic anaemia - target cells on blood film patient usually from Mediterranean countries or middle eastern
81
smudge cells
chronic lymphocytic leukaemia
82
schistocytes
intravascular haemolysis
83
what cells cause graft vs host disease
caused by T cells in donor tissue
84
tumour lysis syndrome
PUKE calcium phosphate uric acid potassium (K) = all elevated calcium = decreased
85
indications for blood transfusion
symptomatic anaemia and haemoglobin <8 g.dl
86
heparin monitoring and reversal
APT - for unfractionated, not much monitoring needed for LMWH reversal = protamine sulphate
87
warfarin monitoring and reversal
INR and vitamin K
88
mechanism of action of clopidogrel
anti-platelet - ADP receptor antagonist
89
mechanism of action of abciximab
anti platelet - GP IIb/IIIa inhibitors
90
mechanism of action of heparin
anti-coagulant - potentiates anti thrombin effect
91
mechanism of action of warfarin
anti-coagulant - vitamin K antagonist
92
mechanism of action of aspirin
irreversibly blocks COX in platelets inhibits TXA2 synthesis and PGI2
93

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