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Haematology Flashcards

1
Q

findings in iron studies for iron deficiency anaemia?

A

microcytic
hypochromic
low ferritin
high TIBC

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2
Q

what are haemoglobinopathies?

A

Affect either:
1. quality and structure of Hb-HbS, HbO, HbE

  1. quantity of Hb- thalassaemia
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3
Q

what is normal adult hb?

A

HbA

2 alpha chains and 2 beta chains

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4
Q

what is foetal Hb?

A

HbF

2 alpha chains and 2 gamma

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5
Q

causes of iron deficiency anaemia?

A

blood or GI loss
insufficient dietary intake
malabsorption e.g. coeliac

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6
Q

blood film for iron deficiency anaemia?

A

target cells

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7
Q

signs of iron deficiency anaemia?

A

koilonychia (spoon-shaped nails)
atrophic glossitis
angular stomatitis
post-cricoid webs

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8
Q

tx of iron deficiency anaemia?

A

ferrous sulphate
continue for at least 3 months after Hb is normal

GI investigation

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9
Q

causes of anaemia of chronic disease?

A 
chronic infection
vasculitis
RA
malignancy
renal failure
etc
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10
Q

Ix of all anaemia?

A

FBC, TFTs, U&Es
haematinics- B12 and folate, ferritin, serum iron, TIBC
scoping
CT

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11
Q

causes of normocytic anaemia?

A 
bleeding
aplastic anaemia
haemolytic anaemia
sickle cell disease
pregnancy
chronic disease
hypothyroidism
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12
Q

what is aplastic anaemia?

A

failure of bone marrow development that causes pancytopenia and hypoplastic bone marrow

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13
Q

what is haemolytic anaemia?

A

breakdown of RBCs before their normal life-span

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14
Q

hereditary causes of haemolytic anaemia?

A

hereditary spherocytosis
G6PD deficiency
sickle-cell
thalassaemia

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15
Q

acquired causes of haemolytic anaemia?

A

immune- warm/cold antibody type, transfusion reaction, rhesus disease

non-immune- malaria, dapsone (drug to treat dermatitis herpetiformis), malignancy, DIC, pre-eclampsia

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16
Q

signs of haemolytic anaemia?

A

bilirubin and LDH raised due to breakdown of cells

MCV high because reticulocytes so big

urinary urobilinogen

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17
Q

what is G6PD deficiency?

A

red blood cell enzyme defect

X linked, males, Mediterranean

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18
Q

precipitants of G6PD deficiency?

A

drugs- antimalarials, ciprofloxacin, sulphonamides
infections
fava beans

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19
Q

features of G6PD deficiency?

A

neonatal jaundice
intravascular haemorrhage
gallstones
splenomegaly

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20
Q

blood film of G6PD deficiency?

A

Heinz bodies

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21
Q

what is hereditary spherocytosis?

A

normal biconcave disc shape of RBC is replaced by a sphere- shaped one
AD inheritance

cells are destroyed by the spleen

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22
Q

Ix of hereditary spherocytosis??

A

blood film-spherocytes

EMA binding test

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23
Q

features of hereditary spherocytosis?

A

failure to thrive
jaundice, gallstones
splenomegaly
aplastic crisis- precipitated by parvovirus

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24
Q

Tx of hereditary spherocytosis?

A

folate replacement

splenomegaly

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25
what is sickle-cell anaemia?
recessive disorder causing production of abnormal beta globin chains HbS rather than HbA the sickled cells are fragine and easily destroyed therefore cause haemolytic anaemia
26
are reticulocytes increased or decreased in sickle cell disease?
increased due to bone marrow trying to keep up with increased destruction of red cells
27
what is sickle cell trait?
60% HbA 40% HbS Normally asymptomatic with normal FBC and no haemolysis provides some protection against malaria
28
how does sick cell present?
``` at age 6-12 months anaemia splenomegaly overwhelming infection crises ```
29
what are sickle cell crises?
- vaso- occlusive crisis (includes acute chest syndrome) - aplastic crisis - sequestration crisis
30
pathology of vaso-occlusive crisis?
``` lots of Hb become deoxygenated at once cold-dehydrated, hypoxia lots of sickling occlusion of small vessels sometimes of larger vessels ```
31
features of vaso-occlusive crisis?
- severe pain from oxygen deprivation of tissues and avascular necrosis of the bone marrow - swollen joints - dactylitis - acute abdomen
32
mx of vaso-occlusive crisis?
strong analgesia high flow O2 hydration blood transfusion
33
specific types of vaso-occlusive crisis?
thrombotic stroke acute chest syndrome priapism
34
features of acute chest syndrome?
new pulmonary infiltrate on XR and dyspnoea, pain, cough and fever
35
mx of acute chest syndrome?
``` oxygen chest physio may require NIV or I&v antibiotics transfusion/exchange transfusion ```
36
what causes an aplastic crisis?
parvovirus b19 infection | massive drop in Hb over 1 week
37
what is a sequestration crisis?
splenomegaly drop in Hb, elevated reticulocytes circulatory collapse, shock
38
mx of sickle cell disease?
- avoid cold, dehydration, hypoxia, exhaustion, alcohol and smoking - folic acid supplementation - oral penicillin V - full vaccinations plus pneumococcal, flu and hep B - iron chelation to prevent fe overload with repeated tx e.g. deferoxamine - hydroxycarbamide- increases HbF - annual transcranial USS
39
complications of sickle cell disease?
``` chronic pain pulmonary hypertension stroke infection gallstones retinopathy avascular necrosis of femoral or humeral head ```
40
what is beta thalassaemia trait?
BB,__ | mildly anaemic, asymptomatic
41
what is beta thalassaemia major?
__,__ severe anaemia chronic transfusion dependent bony deformities
42
what are the different types of alpha thalassaemia?
4 deletions- in utero death 3 deletions- very anaemic, hypochromic, microcytic, splenomegaly, variable bone changes 2 deletions= slightly anaemic 1 deletion- asymptomatic, borderline Hb
43
what is pernicious anaemia?
autoimmune condition with antibodies against parietal cells and intrinsic factor
44
what is normal b12 absorption?
B12 is bound by salivary R protein then bound to intrinsic factor which is produced by the parietal cells
45
features of pernicious anaemia?
macrocytic anaemia megaloblasts (big fragile immature red cells) paraesthesia, glossitis fatigue
46
mx of pernicious anaemia?
B12 injections (hydroxycobalamin) 1mg IM every other day for 2 weeks maintenance- 1mg every 3 months for life can take orally if dietary cause
47
what can maternal folate deficiency cause?
neural tube defects (spina bifida)
48
causes of folate deficiency?
``` poor diet increased demand- pregnancy, malignancy, IBD malabsorption-coeliac drugs- anti-epileptics alcohol ```
49
tx of folate deficiency?
oral folic acid | prophylaxis in pregnancy- 400mcg from conception until 12 weeks
50
what is intracapsular vs extracapsular haemolytic anaemia?
intravascular- complement fixation, trauma, DIC, enzymes extravascular (more common)- removed from circulation because of defective or antibodies on them
51
what is seen on blood film post-splenectomy?
Howell-jolly bodies increased risk from encapsulated bacteria e.g. strep pneumonia, HIB need regular vaccines and lifelong penicillin
52
what are the myeloproliferative disorders?
RBC- polycythaemia rubra vera WBC- CML Platelets- essential thrombocytopenia Fibroblasts- myelofibrosis
53
what are primary and secondary polycythaemia?
``` primary= polycythaemia vera secondary= hypoxia, renal carcinoma, smoking, COPD ```
54
what is polycythaemia rubra vera?
neoplastic proliferation of haemopoietic cells in the bone marrow
55
mutation causing polycythaemia rubra vera?
JAK2 mutation
56
risk of polycythaemia rubra vera?
``` hyperviscosity -> thrombosis prutitis after hot bath splenomegaly haemorrhage plethoric appearance ```
57
tx of polycythaemia rubra vera?
aspirin venesection bone marrow suppression- hydroxycarbamide
58
complications ofpolycythaemia rubra vera?
2-8% transform to myelofibrosis and 1-3% transform to AML
59
causes of massive splenomegaly?
CML myelofibrosis malaria
60
what is ITP?
immune thrombocytopenic purpura
61
causes of ITP in adults vs children?
children- follows viral infection, benign, self-limiting adults- insidious and chronic usually self-limiting and as easy bruising/ mucosal bleeding
62
tx of ITP?
``` stop any NSAIDS or aspirin and observe any platelet count prednisolone IVIG anti-D avoid contact sports ```
63
what is TTP?
thrombotic thrombocytopenic purpura | MEDICAL EMERGENCY
64
pathology of TTP?
MICROANGIOPATHIC THROMBOSES -> HAEMOLYSIS AND PLATELET COMSUMPTION congenital deficiency or autoimmune attack
65
features of TTP?
``` anaemia and haemolysis thrombocytopenia fever purpura cerebral dysfunction ```
66
mx of TTP?
plasma exchange
67
indications for platelet transfusion?
platelet count <30 with clinically sigf bleeding or <100 and bleeding and critical sites e.g. CNS
68
what is haemophilia?
X linked disorder of coagulation
69
types of haemophilia?
A- factor VIII deficiency | B- (Christmas disease)- factor IX deficiency
70
features of haemophilia?
haemoarthroses haematomas prolonged bleeding after surgery or trauma (long APTT)
71
tx of haemophilia?
A- factor VIII replacement | B- factor IX replacement
72
which anticoagulants work at which pathways?
intrinsic pathway- heparin (affects APTT) | extrinsic pathway- warfarin (PT- measured by INR)
73
how is warfarin reversed?
immediately- beriplex (4 factor concentrate- 2,7,9,10) over 12 hours- vitamin K
74
how does warfarin work?
inhibits production of the vitamin K dependent clotting factors
75
how does heparin work?
activates antithrombin-> which inactivates thrombin and Xa
76
what is the difference between unfractionated heparin and LMWH?
unfractionated heparin- IV, short half life, monitored by APTT LMWH- sub cut, longer half life, monitor with anti Xa level
77
how is heparin reduced?
protamine
78
``` how do the DOACs - rivaroxaban - apixaban -dabigatran work? ```
rivaroXAban and apiXAban are anti XA dabigatron is a direct thrombin inhibitor
79
what is a clotting profile of DIC?
low platelets, prolonged PT/APTT, prolonged bleeding time
80
what is the most common inherited bleeding disorder?
von Willebrand's disease | AD
81
feature of VWD?
mucosal bleeding- epistaxis, menorrhagia
82
mx of VWD?
tranexamic acid for mild bleeding desmopressin-raises levels of vWF factor VIII concentrate
83
name some transfusion reactions?
GOT A BAD UNIT Graft vs host disease Overload Thrombocytopenia Alloimmunisation Blood pressure unstable Acute haemolytic reaction Delayed haemolytic reaction Urticaria Neutrophilia Infection TRALI
84
What is hyper viscosity syndrome?
an increase in whole body viscosity due to raised immunoglobulins produced by malignant clonus of plasma cells (myeloma) or WBCs
85
what is raised ESR and osteoporosis until proven otherwise?
myeloma
86
what is myeloma?
malignancy of plasma cells | produce a monoclonal paraprotein (IgA or IgG- abnormal serum electrophoresis and urinary bence jones protein)
87
features of myeloma?
``` CRABI Hypercalcaemia Renal impairment Anaemia Bone pain Infection ```
88
Ix of myeloma
``` serum protein electrophoresis- monoclonal antibodies IgA or IgG Urine- bence jones protein Bone marrow biopsy- high plasma cells FBC- anaemia, thrombocytopenia XR-pepper pot skull CT/MRI ```
89
what can be seen on blood film in myeloma?
roleaux formation (stacking of RBCs)
90
mx of myeloma?
``` stem cell transplants chemo zoledronic acid for pathological fracture prevention Annual influenza vaccine VTE prophylaxis Analgesia ```
91
features of Hodgkin's lymphoma?
young adults rubbery, painless lymphadenopathy B symptoms alcohol-induced lymph node pain
92
causes of Hodgkin's lymphoma?
unknown infections- EBV, helicobacter pylori immunodeficiency- HIV, transplant patients autoimmune disorders
93
findings on lymph node biopsy for Hodgkin's lymphoma?
Reid sternberg cells
94
staging for Hodgkin's lymphoma?
Ann Arbor staging
95
4 types of Hodgkin's lymphoma?
Nodular sclerosing- most common mixed cellularity lymphocyte depleted- worst prognosis lymphocyte rich- best prognosis
96
features of non-Hodgkin's lymphoma?
commonest are follicular and diffuse large B cell (latter has worst prognosis) Present with painless lymphadenopathy with or without B symptoms hepatosplenomegaly
97
extra-nodal disease of NHL?
gastric MALT small bowel lymphomas non-MALT gastric lymphoma
98
what is Burkitt's lymphoma?
associated with EBV, rapid proliferation
99
what cancer does myelofibrosis increase the risk of?
myelofibrosis
100
what cells are seen on blood film of myelofibrosis?
tear drop cells
101
features of AML?
ADULTS | presents with pancytopenia (bruising, infection, mouth sores, anaemia)
102
what is a variant of AML?
acute promyelocytic myeloid leukaemia
103
what is the prognosis of AML?
POOR- 20% 3 year survival with chemotherapy
104
causes of CML?
adults, sometimes incidental finding Philadelphia chromosome t9,22 forms BCR ABL fusion gene, makes tyrosine kinase pathway always on
105
features of CML?
B symptoms | chronic -> accelerated phase -> blast transformation (transformation to acute leukaemia)
106
Ix of CML?
high WCC, low Hb, variable platelets, high urate, high B12 bone marrow biopsy Ph Chr
107
tx of CML?
Tyrosine kinase inhibitor e.g. dasatinib, imatinib
108
who does ALL affect?
children | Down's syndrome, ionising radiation
109
features of ALL?
``` Anaemia bruising infection hepatosplenomegaly lymphadenopathy bone pain joint pain ```
110
blood film of ALL?
blast cells
111
tx of ALL?
``` Blood/platelet transfusion IV fluids Allopurinol (prevents tumour lysis syndrome) infection management chemo ```
112
features of CLL?
COMMONEST older adults asymptomatic or non-specific systemic symptoms or B symptoms
113
blood film of CLL?
smudge cells
114
staging for CLL?
binet staging
115
prognosis for CLL?
1/3 never progress 1/3 progress slowly 1/3 progress actively
116
what is the level for anaemia in men and women?
men <135g/L | women <115g/L
117
what can CLL transform to and what is it called?
Richter's transformation | high grade lymphoma
118
what is seen on blood film of AML?
auer rods
119
what does TIBC measure?
the amount of transferrin in the blood (binds to iron) | Both TIBC and transferrin levels increase in iron deficiency and decrease in iron overload.
120
what is ferritin?
Ferritin is the form that iron takes when it is deposited and stored in cells. Extra ferritin is released from cells in inflammation, such as with infection or cancer. If ferritin in the blood is low it is highly suggestive of iron deficiency. If ferritin is high then this is difficult to interpret and is likely to be related to inflammation rather than iron overload. A patient with a normal ferritin can still have iron deficiency anaemia, particularly if they have reasons to have a raised ferritin such as infection.
121
why is it important to treat B12 deficiency before folate deficiency?
Treating patients with folic acid when they have a B12 deficiency can lead to subacute combined degeneration of the cord.

3b specialties (7 decks)

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