Haematology Flashcards
(137 cards)
1
Q
What is Waldenstroms Macroglobulinaemia
A
IgM paraprotein !!!
Lymphoplasmacytoid Malignancy
2
Q
How does Waldenstroms Macroglobulinaemia Present?
A
Systemic Upset - weight loss lethargy Hepatosplenomegally Raynauds Hypercoag state - Strokes etc NO lytic lesions
3
Q
Waldenstroms vs Multiple Myeloma
A
Multiple Myeloma is IgG Bence Jones and presence of Lytic Bone Lesions
4
Q
At what Hg level can you offer a transfusion in an otherwise healthy patient?
A
70
5
Q
What target Hg is aimed for post transfusion in a healthy patient?
A
70-90
6
Q
In a patient with ACS what level Hg is needed for a transfusion?
A
80
7
Q
What is the Hg target in a patient with ACS receiving a transfusion?
A
80-100
8
Q
What can be used to reveres Dabigatran?
A
Indarocuzimab
9
Q
Side Effects of Heparin
A
Heparin Induced Thrombocytopenia - Bruise like rash itchy swollen
10
Q
If someone cant tolerate Heparin what should be used?
A
DOAC
11
Q
Commonest Hodgkins Lymphoma
A
Nodular Sclerosing
Good Prognosis
Lacunar Cells
12
Q
Best Prognosis in Hodgkins Lymphoma.
A
Lymphocyte Predominant
13
Q
Worst Prognosis in Hodgkins Lymphoma
A
Lymphocyte Depleted
Any tumour with B cell symptoms - fever night sweats weight loss etc
14
Q
> 65 plus new iron deficiency anaemia
A
Referral for Colonoscopy +/- endoscopy
15
Q
What does someone require post splenectomy?
A
Antibiotic prophylaxis - Penicillin V or Erithromycin
Pneumococcal Vaccine - Every 5 years
Influenza - Once a year
Haemophilus Influenza and Men C - Once off
16
Q
Irradiated Blood Products protects against what?
A
Graft Versus Host
17
Q
In immune Thrombocytopaenic Purpura what medication can be used in the treatment?
A
Oral Prednisilone
If you need quick results for example to allow a invasive surgery to occur. Immunoglobulin can be used as it has a quicker response.
18
Q
INR >8 + Major Bleed
or Major bleed regardless of INR
A
Stop Warfarin
IV Vitamin K
Infusion of prothrombin complex or FFP
19
Q
INR >8 + Minor Bleeding
A
Stop Warfarin
IV vitamin K
Repeat INR in 24hrs - <5.0 restart
20
Q
INR >8
A
Stop warfarin Vitamin K oral
Check INR in 24 hours - <5.0 start again
21
Q
INR 5.0 - 8.0
A
Withold 1 0r 2 doses and reduce maintenance dose
22
Q
INR 5.0 - 8.0 + Minor Bleeding
A
Stop Warfarin + IV Vitamin K
Check INR restart after 24 hours in <5.0
23
Q
Who is the universal Plasma donor?
A
AB blood donors as their plasma has no circulating antibodies
24
Q
Beta thalassaemia major - blood results
A
Absent HbA - normal Adult Haemoglobin
Increased HbA2 and HbF - fetal haemaglobin and delta chain haemaglobin
25
ITP management
No treatment - platelets >30 and asymptomatic
Oral steroids - Symptomatic or platelets <30
Immunoglobulins can be used in more severe cases
resistant - splenectomy
Platelets only given in severe bleeding
26
What is elevated in tumour lysis syndrome?
Products of cell destruction
K+
PO4
Urea
27
Low Hb, MCV , MCH
Microcytic hypochromic
Target Cells
Extravascular haemolysis - jaundice and haemochromatosis
HbH disease
| Alpha Thalassaemia 3 genes missing
28
Target Cells
Increased ferritin, Fe, Hbf and HbA2
Extravascular haemolysis - jaundice and haemochromatosis
Beta Thalassaemia
29
Describe the types of beta thalasaemia
The genes can be entirely deleted or altered so they are left effective.
Minor - 1 deleted gene
Intermedia - 2 genes affected
Major - 2 gene deleted
30
What is sideroblastic anaemia
Inability to properly convert Fe2+ into Haem
31
Sideroblastic anaemia - causes
Congenital - X linked ALAS2 mutation
| Aquired - alcohol excess, deficiency in Vitamin B6, Lead poisoning
32
```
Pappenheimer bodies
Basophilic stippling
Haemochromatosis symptoms
MCV is normal or low
Increased Fe2+, Ferritin
Decreased TIBC
```
Sideroblastic anaemia
33
Decreased serum iron and transferrin
Normal TIBC
Increased ferritin
Anaemia of chronic disease
34
Heinz body and bite cells
Decreased RBC and Haptoglobulin
Increased LDH Reticulocytes Bilirubin
Negative Coombs test
G6PD deficiency
| Intravascular haemolysis due to oxidative stress
35
Common oxidative stressors in G6PD deficiency
```
Fava beans
Aspirin
NSAIDs
Parvovirus
Sulfonamides
```
36
What is the definitive diagnostic test in G6PD deficiency ?
Enzyme Assay
37
G6PD deficiency - demographic
X linked autosomal recessive disease - men
| African and Mediterranean origin
38
Autoimmune Haemolytic Anaemia - Types
Warm - IgG extravascular, occurs at body temperature, mainly idiopathic
Cold - IgM extravascular occasionally intravascular (raynauds), occurs at cold temperatures
39
Direct Coombs test results in Haemolytic anaemia
Warm - IgG and C3D
| Cold - C3D
40
Common causes of warm haemolytic anaemia
```
Viral
SLE
Lymphoma
Leukamia
Beta lactam antibiotics
```
41
Normocytic anaemia
Raised reticulocytes
Positive Coombs test
Haemolytic anaemia
42
Elevated 2,3 BGP
Negative Coombs test
Spiky dehydrated cells -echinocytes
Mainly extravascular haemolysis - raised bilirubin + Fe
Some intravascular - decreased haptoglobulin
Pyruvate Kinase deficiency
43
Extravascular haemolysis
Spherocytes
Reticulocytes
Hereditary Spherocytosis
44
What is use to diagnose Hereditary Spherocytosis
Osmotic fragility test
45
Hereditary Spherocytosis inheritance
75% AD
| 25% AR
46
Hodgkins Lymphoma - treatment
Adriamycin + Bleomycin + Vinblastine + Dacarbazine ABVD 2-6 cycles
+ Radiotherapy
47
Painless lymphadenopathy
Alcohol causes pain
Reed Sternberg Cells
Hodgkins Lymphoma
48
Non Hodgkins Lymphoma - Treatment
```
Rituximab - CD 20
Cyclophosphamides
Hydroxydaunorubicis
Oncovin
Prednisolone
```
49
Nocturnal Haemoglobinuria
Phosphatidylinosite Glycan A defect
50
Symptoms of Nocturnal Haemoglobinuria
```
Anaemia
Venous Thromboembolism
Oesophageal spasm
Erectile dysfunction
Dark urine especially in the morning
```
51
Treatment for Paroxysmal Nocturnal Haemoglobinuria
Eculizumab
52
Sickle Cell Admission Criteria
Everyone is admitted apart from.
Well adult + mild/moderate pain + Temp <38*c
Well child + mild/moderate pain + No temp
53
Transfusion Reactions
| Fever + Chills
Non haemolytic febrile reaction
| Slow stop transfusion + Paracetamol
54
Transfusion Reactions
| Pruritus + Urticaria
Minor allergic reaction
| Stop + Antihistamine
55
Transfusion Reactions
| Fever Abdominal Pain Hypotension
Acute Haemolytic Reaction
Stop + Confirm patient + Bloods to labs + Coombs test
Fluid Resus
56
Transfusion Reactions
| Hypoxia + Infiltrates on chest xray
Acute Lung Injury
| Stop + Supportive therapy
57
Transfusion Reactions
| Pulmonary Oedema Hypertension
Circulatory Overload
Slow/Stop
Loop Diuretics
58
Common causes of aplastic anaemia?
Autoimmune destruction is commonest
Indomethacin, Propythiouracil, Chloramphenicol
EBV HIV
59
Common signs of Aplastic anaemia
Pancytopenia
| Bone Marrow dry tap
60
Fanconis anaemia
Congenital aplastic anaemia
| Short, microcephaly, cafe au lait, absent or small thumbs
61
If only RBC are absent or low but Platelets and WBC are normal what can be a cause?
Diamond Blackfan Anaemia
62
ITP - cause
IgG binds to GPIIb/IIIa which then allows the spleen to destroy platelets
63
ITP - Types
Acute - 2 weeks post virus - spontaneous resolution - generally children
Chronic - women of reproductive age - idiopathic or Hep C HIV Lupus
64
ITP - Diagnosis
```
Diagnosis of exclusion
USS for splenomegaly
FBC
HIV
Hep C screen
```
65
Thrombolitic Thrombocytopenic Purpura - presentation
Reduced platelets - bleeds, purpura
| Neurological issues - confusion, speech, coma
66
TTP - cause
Von Willenbrand factor isn't broken down so micrclots develop everywhere.
Aquired - autoimmune
Congenital - Autosomal recessive from birth
67
TTP - Treatment
Dialysis if AKI
| Plasmapheresis or Rituximab
68
Von Willenbrand Disease - Cause
Reduced platelet adhesion +
VWF usually protects VIII from protein C and S
= Bleeds
69
Von Willenbrand Disease - Treatment
Minor bleed = Desmopressin analogues
| Major Bleeds = Exogenus VWF and Factor VIII
70
Factor V leiden
Commonest Hyper coagulable state
71
Hodgkins lymphoma - Presentation
Nodal Disease
| Painless cervical lymphadenopathy
72
Hodgkins Lymphoma - Cells and Immunology
B cells
CD15 and CD30
'Owl eyes' - reed-sternberg cells
73
Hodgkins Lymphoma - Nodular Lymphocytic predominant
Generally affects men
CD20 CD45
Lobulated popcorn cells
Rituximab can be used
74
Diagnosis of lymphomas
CT - staging
| Lymph node biopsy
75
If there is mediastinal adenopathy what lymphoma is it likely to be?
Nodular Sclerosing Hodgkins Lymphoma
76
Non Hodgkins Lymphoma - Presentation
Nodal + Extranodal disease - hepatosplenomegally, mucosal tissues
77
Whats the commonest Non Hodgkins cell lymphoma ?
Diffuse Large B cel
| Agressive course
78
What non Hodgkins cell lymphoma is considered indolent?
Follicular
79
Burkitt Lymphoma - histology
Starry Sky appearance
80
Burkitt Lymphoma - presentation
African - lymphoma affecting Jaw
| European - Ileocaecal junction
81
Burkitt lymphoma - causes
HIV
| EBV
82
Commonest T cell lymphoma
Adult T cell lymphoma
| Caused by human T lymphotropic virus
83
Is MALT - Hodgkins or non hodgkins?
Non Hodgkins
84
CML - Immunology and cytology
Philadelphia Chromosome t(9,22)
| BCR ABL - tyrosine kinase
85
CLL - Immunology and cytology
CD23 CD19 CD5
| Smudge cells on blood film
86
What can be used to reverse a bleed in a patient using Rivaroxaban or Apixiban?
Andexanit Alfa
87
What can be used to reverse a bleed in a patient using heparin?
Protamine Sulfate
88
Multiple myeloma on skull xray
Raindrop skull
89
What is the commonest inherited bleeding disorder?
Von Willenbrand disease
Autosomal Dominant Disorder
Acts like a platelet disorder - less haemarthrosis
90
Sickle Cell Patient -
| Dysopnoea Chest Pain Pulmonary Inflitrates on X-Ray
```
Acute Chest Syndrome
# cause of death after childhood
```
91
Acute Chest syndrome management
Pain Relief
Respiratory support
Antibiotics
Transfusion
92
Sickle Cell -
Parvovirus infection
Reduced Hb and reticulocytes
Bone marrow suppression
Aplastic Crisis
93
Sickle Cell-
Worsening anaemia
Increased Reticulocyte
Hepatosplenomegally
Sequestration crisis
94
```
Sickle Cell
Painful vasoocclusive
Dehydrated
Infection
Deoxygenated
```
Thrombotic crisis
95
Recent heparin use
Prothrobotic state
+ve for heparin platelet factor 4 complex
Heparin induced thrombocytopenia
96
How long should a non urgent RBC transfusion take place over?
90-120 minutes
97
What affect can a packed RBC infusion have on a ECG?
Increased K+ leading to ECG changes related to this
98
Chemotherapy used in AML
Anthracycline
| Cytarabin
99
What genetic conditions put you at an increased risk of acute leukaemia
Down syndrome
NF1
Klineflelters
Fanconis anaemia
100
What is one of the earliest signs in myelofibrosis
Isolated neutropenia
| As spleen and liver can take up the slack in RBC and platelet production
101
Platelet transfusion
Theshold is <10
| Unless bleeding or surgery is planned
102
Contraindications for a platelet transfusion
Autoimmune Thrombocytopenia
Heparin induced thrombocytopenia
TTP
Chronic Bone marrow failure - as body develops antibodies as platelets so rare
103
In a CLL patient presenting with acute onset B cell symptoms what is likely to have happened?
Richters transformation - non hodgkins lymphoma with a very aggressive course
104
Scott Coutts
Smile King
105
Elevated methaemoglobinaemia
| Hypoxia not responding to oxygen therapy
Amyl Nitrate
| Poppers usage
106
In post thrombotic syndrome what is the management?
Compression stockings
107
Polcythaemia rubra Vera - first line investigation
JAK2 mutation
| Blood film
108
```
Increased Hb +/- neutrophil and platelet
Pruritic after bath
Splenomegaly
Bleeds
low ESR
```
PRV
109
EPO infusion side effects
```
Hypertensive encephalopathy
Bone aches
Flu like symptoms
Urticaria
Pure red cell aplasia
Thrombosis
Iron deficiency
```
110
Chemotherapy in CLL
Fludarabine
Cyclophosphamide
Rituximab
111
Management of antiphospholipid syndrome
Primary prevention - Aspirin
Secondary Prevention - Lifelong warfarin IN 2-3
- recurrent despite treatment - Aspirin + INR 3-4
- arterial thrombosis - INR 2-3
112
Only anticoagulant safe in pregnancy.
LMWH
113
If someone has an IgA deficiency what transfusion complication are they most at risk of?
Anaphylactic reaction
114
In a suspected G6PD defficiency when should enzyme assays take place?
At time or presentation
| 3 months
115
If someones INR is found to be below 2 what should be initiated?
LMWH cover and increase maintenance dose of warfarin
116
Age range for immediate same day referral for investigation if unexplained hepatosplenomegaly or petechia?
0-24 years old
117
If you have a Iron deficiency anaemia Iron study but the FBC shows an increased RDW on a background of crohns or coeliac what should you consider?
A mixed anaemia picture
| RDW means there is an increased variation in size of RBC
118
Management of warm haemolytic anaemia
Steroids
| +/- rituximab
119
If someone has a minor allergic reaction to a blood transfusion how is this managed?
Temporarily stop infusion
Antihistamine
Temperature rise shouldn't be more than 2 degrees
120
What is the commonest primary antibody deficiency? And how does it present?
Selective IgA deficiency
Recurrent URTI and sinus infections
Linked to coeliac disease
Risk of severe anaphylaxis post blood transfusion
121
Recurrent Pneumonia and abscess
| Staph Aureus and Fungal infections
Chronic Granulomatous Disease - Neutrophil disorder
122
Recurrent bacterial infections
No pus at sight of infection or wound
Desloughing of umbilical cord was delayed
Leucocyte adhesion
123
Child
Partial albinism
Peripheral neuropathy
Recurrent bacterial infections
Chediak Higashi syndrome - neutrophil deficiency
124
Hypogammaglobulinaemia
Risk of Autoimmune disorders
Risk of Lymphoma
Common variable immunodeficiency
125
X linked recessive condition
No B cells
Reduced immunoglobulins
Bruton X linked
126
```
Congenital heart disease - tetralogy of falot
Learning difficulties
Hypocalcaemia
recurrent viral an fungal infections
Cleft palate
```
DiGeorge syndrome - T cell deficiency
22q11.2 deletion
No thymus
127
Commonest x linked deficiency
| Increased bacterial fungal and viral infections
Severe combined immunodeficiency
| Stem cell is required
128
LMWH vs unfractionated heparin
LMWH - Antithrombin III and Xa inhibitor
- Monitored with Anti Xa assay - not routinely needed during surgery
- Prolonged duration - protamine sulphate isn't as affective
- First line VTE and ACS
- Less risk of HIT and osteoporosis
Unfractionated Heparin - Antithrombin III Xa IXa XI and XIIa inhibitor
- Monitored with APTT required if used during surgery.
- Shorter duration of action - preferred if increased bleed risk and fully reversible.
129
When would you suspect neutropenic sepsis and how is this managed?
Cause of neutropenia identified + Temperature >38 + RR >20 + Presumed or confirmed infection
Give Piperacillin + Tazobactan (Tazocin) and send blood cultures - don't wait for results to initiate sepsis 6.
130
Management of Polycythaemia Rubra Vera
Aspirin and venesection - first line
Hydroxyurea
Ruxotinib - JAK2 inhibitor
131
How long should a blood transfusion be transfused if the patient has HF?
3 hours
132
Inheritance of haemophilia
X linked
133
```
Fever
Neurological symptoms
Thrombocytopenia
Haemolytic anaemia
Renal failure
```
TTP
134
Auer rods
AML
135
Dabigatran MOA
Direct thrombin inhibitor
136
Apixiban or rivoroxaban
Direct Xa inhibitor
137
Ann Arbor staging for lymphoma
Grade 1 = Single nodal group
Grade 2 = 2 or more nodal groups
Grade 3 = Nodal groups on both sides of the diaphragm
Grade 4 = Extra lymphatic organs involved
```
A = Asymptomatic
B =B symptoms
X = bulky nodes
S = splenic involvement
E = extra nodal
```
