Haematology Flashcards
(246 cards)
1
Q
What is blood made up of?
A
Plasma (liquid of the blood) contains RBC, WBC and platelets ALSO contains clotting factors e.g. fibrinogen
2
Q
Once clotting factors are removed from blood what is left?
A
Glucose
Electrolytes e.g. sodium and potassium
Proteins e.g. immunoglobulins and hormones
3
Q
Where is bone marrow found?
A
Pelvis
Vertebrae
Ribs
Sternum
4
Q
Label the following:
A
5
Q
Name some pleuipotent haematopoietic stem cells?
A
Undifferentiated cells with potential to transform:
Myeloid stem cells
Lymphoid stem cells
Dendritic cells
6
Q
What doe RBCs develop from? How long do they last?
A
Reticulocytes (myeloid stem cells)
Survive 3 months
7
Q
What are platelets made from? How long do they last?
A
Megakaryocytes (live for 10 days) - clump together and plug gaps
8
Q
What are the WBC?
A
Myeloid stem cells (become promyelocytes, then:)
- Monocytes then macrophages
- Neutrophils
- Eosinophils
- Mast cells
- Basophils
Lymphoid stem cells (become lymphocytes)
- B cells (mature in bone marrow to become plasma cells and memory B cells)
- T cells (mature in thymus gland to become CD4 T helper cells, CD8 cytotoxic T cells, natural killer cells)
9
Q
What are some key blood film findings and when are they seen?
A
Anisocytosis - variation in size of blood cells (myelodysplastic syndrome)
Target cells - iron deficiency anaemia, post-splenectomy
Heinz bodies - blobs of denatured globin seen in G6PD and alpha-thalassaemia
Howell-Jolly bodies blobs of DNA (post-splenectomy and severe anaemia - body is regenerating cells quickly)
Reticulocytes - immature RBC, slightly larger than RBC with RNA material in (reticular - mesh like appearance), 1% of reticulocytes is normal, increases in haemolytic anaemia
Schistocytes - fragments of RBC - indicate damaged RBC from networks of clots e.g. haemolytic uraemic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenia purpura, metallic heart valves and haemolytic anaemia
Sideroblasts - immature RBC with blobs of iron (bone marrow is unable to incorporate iron in to Hb molecules) - myelodysplastic syndrome
Smudge cells - ruptures WBC due to fragile cells during blood film - chronic lymphocytic leukaemia
Spherocytes - spherical RBCs without normal bi-concave disk space - autoimmune haemolytic anaemia or hereditary spherocytosis
10
Q
What is anaemia?
A
Low level of Hb in the blood
11
Q
What are the normal Hb ranges in men and woman?
A
Women = 120-165
Men = 130-180
MCV = 80-100
12
Q
What are the causes of microcytic anaemia?
A
T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia
13
Q
What are the causes of normocytic anaemia?
A
A – Acute blood loss
A – Anaemia of Chronic Disease
A – Aplastic Anaemia
H – Haemolytic Anaemia
H – Hypothyroidism
14
Q
What are the two types of macrocytic anaemia?
A
Megaloblastic = impaired DNA synthesis - rather than dividing it keeps growing (caused by vitamin deficiency)
Normoblastic
15
Q
Give examples of causes of megaloblastic and normoblastic macrocytic anaemia?
A
Megaloblastic = B12 deficiency, folate deficiency
Normoblastic macrocytic anaemia = alcohol, reticulocytosis (usually from haemolytic anaemia / blood loss), hypothyroidism, liver disease, drugs e.g. azathioprine
16
Q
What are the symptoms of anaemia?
A
Tiredness
SoB
Headaches
Dizziness
Palpitations
Worsening of other conditions e.g. angina, HF, PVD
17
Q
Which symptoms are specific to iron deficiency anaemia?
A
Pica - dietary cravings for abnormal things
Hair loss
18
Q
What are some generic signs of anaemia?
A
Pale skin
Conjunctival pallor
Tachycardia
Raised RR
19
Q
What are some signs of specific causes of anaemia?
A
Koilonychia is spoon shaped nails and can indicate iron deficiency
Angular chelitis can indicate iron deficiency
Atrophic glossitis is a smooth tongue due to atrophy of the papillae and can indicate iron deficiency
Brittle hair and nails can indicate iron deficiency
Jaundice occurs in haemolytic anaemia
Bone deformities occur in thalassaemia
Oedema, hypertension and excoriations on the skin can indicate chronic kidney disease
20
Q
What are some inital investigations of anaemia?
A
Hb
MCV
B12
Folate
Ferritin
Blood film
21
Q
What are some further investigations for anaemia?
A
Oesophago-gastroduodenoscopy (OGD) and colonoscopy to look for GI cause of iron deficiency - on urgent referral for GI cancer
Bone marrow biopsy - if cause is unclear
22
Q
When can iron deficiency occur?
A
Insufficient dietary intake
Requirements increase (e.g. pregnancy)
Iron is lost (e.g. bleeding from colon cancer)
Inadequate absorption
23
Q
Where is iron mainly absorbed?
A
Duodenum
Jejunum
24
Q
What can cause problems with iron absorption?
A
Medications that reduce stomach acid e.g. proton pump inhibitors (lansoprazole and omeprazole) as acid is need to keep iron in soluble ferrous Fe2+ form
25
Which **conditions** can cause **inadequate iron absorption**?
Coeliacs
Crohns
26
What are some **causes** of **iron deficiency anaemia**?
**Blood loss**
**Dietary** insufficiency
**Poor** iron **absorption**
Increased **requirements** e.g. pregnancy
27
What can cause **iron deficiency** anaemia due to **blood loss**?
Women = menorrhagia (heavy periods)
Men = GI tract (oesophagitis, gastritis, IBD)
28
How does **iron travel around the body**?
**Ferric ions** (Fe3+) bound to carrier protein **transferrin**
29
What is **total iron binding capacity**?
Total space on the **transferrin** molecule for the iron to bind
30
What is the **formula** for **proportion of transferrin molecules** that are bound to iron?
Transferin saturation - serum iron / TIBC
31
What is **ferritin**?
Form iron takes with stored in cells
32
When is extra ferritin released? What if it's low?
Inflammation (infection / cancer)
If low = iron deficiency
33
Can a **patient with normal ferritin have iron deficiency**?
Yes
34
Does serum iron vary? Is it a good measure?
Yes, rises after iron containing meals, not useful on its own
35
How do TIBC and transferrin levels change?
**Increase in iron deficiency**
**Decrease in iron overload**
36
What can give the **impression** of **iron overload**?
Supplementation
Acute liver damage
37
What are the **investigations** for **iron deficiency anaemia**?
**Oesophago-gastroduodenoscopy** (OGD) and **colonoscopy** look for cancer of GI tract (if no clear cause e.g. menorrhagia / pregnancy)
38
What are the **management** options for **iron deficiency anaemia**?
**Blood transfusion**
**Iron infusion** e.g. cosmofer (small risk of anaphylaxis, avoided in sepsis as iron 'feeds' bacteria)
**Oral iron** e.g. **ferrous sulfate** 200mg TDS (causes constipation and black coloured stools - unusable if malabsorption problems)
Expect rise of 10g/L per week
39
What is **pernicious anaemia**?
Cause of **B12 deficiency anaemia**
40
What is **intrinsic factor** and **where is it made**?
Needed for **absorption of vitamin B12** in the **ileum** made in the **parietal cells**
41
What happens in **pernicious anaemia**?
**Antibodies** form against **parietal cells** or **intrinsic factor**
42
What results from **vitamin B12 deficiency**?
**Neurological symptoms**:
* **Peripheral neuropathy** with numbness / paraesthesia
* Loss of vibration sense or proprioception
* Visual changes
* Mood / cognitive changes
43
Which **antibodies** are **tested for** in **pernicious anaemia**?
**Intrinsic factor antibody** (first line)
**Gastric parietal cell antibody** (less helpful)
44
What is the **management** of dietary deficiency of B12/ **pernicious anaemia**?
**Dietary deficiency** = oral replacement with **cyanocobalamin** (unless deficiency is severe)
**Pernicious anaemia** = 1mg IM **hydroxycobalamin** 3 times weekly for 2 weeks then every 3 months
45
How to treat **combined folate and B12 deficiency**?
**Treat B12 deficiency first** before correcting folate deficiency (if giving folic acid first = **subacute combined degeneration of the cord**)
46
What is **haemolytic anaemia**?
Destruction of **RBC** leading to anaemia
47
List some **inherited haemolytic anaemias**?
Hereditary **spherocytosis**
Hereditary **elliptocytosis**
**Thalassaemia**
Sickle cell anaemia
G6PD deficiency
48
List some **acquired haemolytic anaemias**?
**Autoimmune haemolytic anaemia**
**Alloimmune haemolytic anaemia** (transfusions reactions and haemolytic disease of newborn)
**Paroxysmal nocturnal haemoglobinuria**
**Microangiopathic haemolytic anaemia**
**Prosthetic valve** related haemolysis
49
What are the **features** of **haemolytic anaemia**?
**Anaemia** - due to reduction in circulating RBCs
**Splenomegaly** - filled with destroyed RBCs
**Jaundice** - bilirubin is released during destruction of RBCs
50
What are the **investigations** of **haemolytic anaemia**?
**FBC** (normocytic anaemia)
**Blood film** (schistocytes - fragments of RBCs)
**Direct coombs test** (positive in autoimmune haemolytic anaemia)
51
What is the most common inherited haemolytic anaemia in northern europeans?
**Hereditary spherocytosis** (autosomal dominant)
Sphere shaped RBCs - fragile and easily break down
52
How does **hereditary spherocytosis present**?
**Jaundice**
**Gallstones**
**Splenomegaly**
**Aplastic crisis** (in presence of parvovirus)
53
How is **hereditary spherocytosis** diagnosed?
**EMA binding assay**
FH
Clinical features
**Spherocytes** on **blood film**
54
What does the **FBC** reveal for **haemolytic anaemia**?
**MCHC** (mean corpuscular haemoglobin concentration) is raised
**Reticulocytes** raised
55
What is the **treatment** of **hereditary spherocytosis**?
**Folate supplementation**
**Splenectomy**
**Cholecystectomy** (removal of gallbladder for gallstones)
56
What is **hereditary elliptocytosis**?
What is the **pattern of inheritance**?
**Managment**?
Same as hereditary spherocytosis except RBC are ellipse shaped
Autosomal dominant
Presentation and management the same
57
What is **G6PD deficiency**?
Who is it more common in?
What is the mode of inheritance?
Defect in the RBC enzyme G6PD
Common in **mediterranean** and **african patients**
**X-linked recessive**
58
What can trigger the **crises** in **G6PD deficiency**?
**Infections**
**Medications**
**Fava beans** (broad beans)
59
How does **G6PD** **present**?
**Jaundice** (neonatal period)
**Gallstones**
**Anaemia**
**Splenomegaly**
**Heinz bodies** on blood films
60
How can a **diagnosis** of **G6PD** deficiency be made?
**G6PD enzyme assay**
61
Which **medications** trigger **haemolysis** in **G6PD deficiency**?
**Primaquine** (an antimalarial)
**Ciprofloxacin**
**Sulfonylureas**
**Sulfasalazine**
**Sulphonamide** drugs
(Sulph-group)
62
What is **autoimmune haemolytic anaemia**?
What are the two types?
How can it be tested for?
**Antibodies** are created against the patient's **RBC**
**Warm type / cold type** autoimmune haemolytic anaemia
Positive **direct Coombs test**
63
What is **warm type autoimmune haemolytic anaemia**?
What is it associated with?
More common, **haemolysis** occurs at **normal** or **above normal** temperatures (usually **idiopathic**) arising without a clear cause
CLL, lymphoma, SLE
64
What is **cold type autoimmune haemolytic anaemia**? What is it also known as?
What is it associated with?
Lower temps (\< 10oC) = antibodies against RBC attach and cause them to **clump together** (**agglutination**) causing **destruction of RBC** as immune system is activated against them
Also known as **cold agglutinin disease**
Associated with **lymphoma** and presents with Raynaud's phenomenon
65
What can **cold type AIHA** occur secondarily to?
**Lymphoma**
**Leukaemia**
**Systemic lupus erythematosus**
**Infections** = mycoplasma, EBV, CMV, HIV
66
What is the **management** of **autoimmune haemolytic anaemia**?
Blood **transfusions**
**Prednisolone** (steroids)
**Rituximab** (monoclonal antibody against B cells)
**Splenectomy**
67
What are the **two scenarios** where **alloimmune haemolytic anaemia** occurs?
**Transfusion reactions**
## Footnote
**Haemolytic disease of the newborn**
68
What happens in **haemolytic transfusion reactions**?
Immune system produces **antibodies** against **antigens** on foreign RBC
69
What is **paroxysmal nocturnal haemoglobinuria**?
Rare condition - genetic mutation in **haematopoietic stem cells** in **bone marrow** occurs during patients lifetime.
Loss of **protein** on the surface of **RBC** which usually inhibit the **completment cascade** - causes destruction of RBCs
70
How does **paroxysmal nocturnal haemoglobinuria** present?
**Red urine** in the morning containing **haemoglobin** and **haemosiderin** causing anaemia
71
What are patients with **paroxysmal nocturnal haemoglobinuria** predisposed to?
**Thrombosis** (e.g. DVT, PE and hepatic vein thrombosis)
**Smooth muecle dystonia** (e.g. oesophageal spasm and erectile dysfunction)
72
What is the **management of paroxysmal nocturnal haemoglobinuria**?
**Eculizumab** (monoclonal antibody that targets completment component 5, C5 - suppressing complement system) or **bone marrow transplantation** (can be curative)
73
What is **microangiopathic haemolytic anaemia** (MAHA)?
**Small blood vessels** have **structural abnormalities** causing **haemolysis of blood cells**
74
What can **microangiopathic haemolytic anaemia** usually secondary to?
**Haemolytic uraemic syndrome** (HUS)
**Disseminated intravascular coagulation** (DIC)
**Thrombotic thrombocytopenia purpura** (TTP)
**Systemic lupus erythmatosus** (SLE)
Cancer
75
What is a **key complication** of **prosthetic heart valves**? (both bioprosthetic and metallic)
**Haemolytic anaemia** - due to turbulence
76
What is the **management** of **prosthetic valve haemolysis**?
**Monitoring**
**Oral iron**
**Blood transfusion** if severe
**Revision surgery** in severe cases
77
What does **normal haemoglobin** consist of?
2 **alpha** and 2 **beta-globin chains**
78
What is **thalassaemia**?
**Genetic defect** in **protein chains** that make up Hb
79
What are the **two types of thalassaemia**?
What is the pattern of inheritance?
**Alpha thalassaemia**
**Beta thalassaemia**
Autosomal recessive
80
What are the **features** of **thalassaemi****a**?
RBC = fragile and break down
**Splenomegaly**
**Bone marrow expands** = susceptibility to fractures, pronounced forehead, malar eminences (cheekbones)
81
What are the **potential signs and symptoms** of **thalassaemia**
**Microcytic anaemia** (low mean corpuscular volume)
**Fatigue**
**Pallor**
**Jaundice**
**Gallstones**
**Splenomegaly**
**Poor growth and development**
**Pronounced forehead** and **malar eminences**
82
How is a **diagnosis** of **thalassaemi****a** made?
**FBC** showing microcytic anaemia
**Haemoglobin electrophoresis** to diagnose **globin** abnormalities
**DNA testing** to look for genetic abnormality
Pregnant women are offered screening at booking
83
How to **monitor** for **iron overload** in **thalassaemia**?
**Monitor serum ferritin**
84
How to manage iron overload in thalassaemia?
Limit transfusions
Iron chelation
85
What are the **features** of **iron overload in thalassaemia**?
**Fatigue**
Liver **cirrhosis**
**Infertility** and impotence
**Heart failure**
**Arthritis**
**Diabetes**
**Osteoporosis** and joint pain
86
On what chromosome is the defect in **alpha-thalassaemia**?
**Chromosome 16** (codes for alpha-globin chains)
87
What is the **management** of **alpha thalassaemia**?
**Monitor FBC**
Monitor for **complications**
**Blood transfusions**
**Splenectomy**
**Bone marrow transplant** can be curative
88
On which chromosome is the defect in **beta-thalassaemia**?
**Chromosome 11** (gene coding for beta-globin chains)
89
What are the three types of **beta thalassaemia**?
**Thalassaemia minor**
**Thalassaemia intermedia**
**Thalassaemia major**
90
What are the **features** and **treatment** of **thalassaemia minor**?
Carrier of an abnormally functioning beta globin gene (one **abnormal** and **one normal**)
Causes **microcytic anaemia** (require only monitoring and no active treatment)
91
What are the **features** and **treatment** of **thalassaemia intermedia**?
**Two abnormal copies** of the **beta-globin gene** (either two defective or one defective and one deletion)
Requires **monitoring** and **occasional blood transfusions** (may require iron chelation to prevent overload)
92
What are the **features of thalassaemia major**?
What is the treatment?
**Homoxygous** for the **deletion gene** (no functioning beta-globin genes at all)
Presents with **severe anaemia** and **failure to thrive** in **early childhood**
Causes:
* Severe microcytic anaemia
* Splenomegaly
* Bone deformities
Management:
* Regular **transfusions**
* Iron **chelation**
* **Splenectomy**
* Bone marrow transplant can be curative
93
What is **sickle cell anaemia**?
**Genetic condition** causes **sickle** (crescent) shaped **RBCs** (making them fragile = **haemolytic anaemia**)
94
What happens in **sickle cell disease**?
Have **abnormal variant** called **haemoglobin S** (HbS) causing RBC to be sickle shaped
95
When is **fetal haemoglobin** (HbF) usually replaced by **haemoglobin A** (HbA)?
Around **6 weeks**
96
What is the pattern of **inheritance** for **sickle cell disease**?
What are the 2 different kinds?
**Autosomal recessive** (abnomal gene for beta-globin on chromosome 11)
One copy = **sickle cell trait**
Two copies = **sickle cell disease**
97
Why is **sickle cell disease** more common in pts from areas affected by malaria (e.g. africa, india, middle east, and caribbean) ?
Being **sickle cell trait** reduces the severity of malaria
98
When is **sickle cell disease** routinely tested for?
**Pregnant woman** at risk of being carriers
**Newborn screening heel prick test** (5 days old)
99
What are some **complications** of **sickle cell disease**?
**Anaemia**
Increased **risk of infection**
**Stroke**
**Avascular necrosis** in large joints such as the hip
**Pulmonary hypertension**
Painful and persistent penile erection (**priapism**)
**Chronic kidney disease**
**Sickle cell crises**
Acute **chest syndrome**
100
What is the **management** of **sickle cell anaemia**?
Avoid dehydration and other triggers
Ensure **vaccinations** up to date
**Antibiotic prophylaxis** to protect against infection with **pen V**
**Hydroxycarbamide** to stimulate production of **HbF**
**Blood transfusion** (for severe)
**Bone marrow transplant** can be curative
101
What is a **sickle cell crisis**?
What causes them?
How to treat?
**Spectrum of acute crises** related to condition
**Causes** = infection, dehydration, cold / significatn life events
**Supportive management** = low threshold for admission, treat any infection, keep warm, keep well hydrated (IV fluids), simple analgesia e.g. paracetamol / ibuprofen, penile aspiration in priapism
**NSAIDs** avoided where there is renal impairment
102
What is a **vaso-occlusive crisis** (aka painful crisis)?
Clogginf of **capillaries** causing **distal ischaemia** associated with **dehydration** and raised **haematocrit**
103
What are the **symptoms** of a **vaso-occlusive crisis**?
Pain
Fever
Those of triggering infection
**Priapism** (blood trapping in penis - treated urgentky with aspiration)
104
What is a **splenic sequestration crisis**?
What is the **management**?
RBCs blocing blood flow in the spleen = **acutely enlarged and painful spleen** causing **severe anaemia** and **circulatory collapse** (hypovolaemic shock)
Considered an **emergency**
Management = supportive with blood transfusions and fluid resus
**Splenectomy** prevents
105
What is an **aplastic crisis**?
**Temporary loss** of **creation** of **new blood cells** (commonly triggered by **infection with parvovirus B19**) leading to **significant anaemia** - management is **supportive** (blood transfusions if necessary)
Usually **resolve spontaneously in a week**
106
What is **acute chest syndrome** in sickle cell disease?
Diagnosis =
**Fever / resp symptoms** with
New **infiltrates** on **CXR**
107
What causes **acute chest syndrome**?
**Infection** = pneumonia or bronchiolitis
**Non-infective causes** = pulmonary vaso-occlusion or fat emboli
108
What is the **treatment** of **acute chest syndrome** (emergency)?
**Abx** or **antivirals** for infections
Blood **transfusions** for anaemia
**Incentive spirometry** using a machine to encourage effective and deep breathing
**Artificial ventilation** with NIV or intubation
109
What is **leukaemia**?
Cancer of a **particular line** of the **stem cells** in the **bone marrow** (causing unregulated production of certain cells)
Can be **chronic** or **acute** and affect **myeloid** or **lymphoid** cell lineage
110
What are the **four main types of leukaemia**?
Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia
111
Name another rarer leukaemia (unlikely to come up in exams)?
**Acute promyelocytic leukaemia**
112
What can whe excessive production of a single type of cell lead to?
**Pancytopaenia** (anaemia, leukopaenia, thrombocytopaenia)
113
What ages do the various leukaemias occur?
**ALL** **C**e**LL**mates have **C**o**M**mon **AM**bitions
Under 5 and over 45 = ALL
Over 55 = CLL
Over 65 = CML
Over 75 = AML
114
How does **leukaemia** present?
**Non-specific** (get urgent FBC if leukaemia on differentials)
* Fatigue
* Fever
* Failure to thrive (children)
* Pallor due to anaemia
* Petechiae and abnormal bruising due to thrombocytopaenia
* Abnormal bleeding
* Lymphadenopathy
* Hepatosplenomegaly
115
What are the **differential diagnosis** of **petechiae**?
**Leukaemia**
**Meningococcal septicaemia**
**Vasculitis**
**Henoch-Schonlein Purpura**
**Idiopathic Thrombocytopenia Purpura (ITP)**
**Non-accidental injury**
116
How is **leukaemia** diagnosed?
**FBC** (within 48 hrs if suspected leukaemia, if petechiae / hepatosplenomegaly then referred immediately to hospital)
**Blood film** to look for abnormal cells / inclusions
**LDH** (can be raised in other cancers)
**Bone marrow biopsy** (main investigation for diagnosis)
**CXR** (infection / mediastinal lymphadenopathy)
**Lymph node biopsy** (assess lymph node involvement / investigate for lymphoma)
**LP** (CNS involvement)
**CT**, **MRI**, and **PET** scans for staging ans assessing for lymphoma / other tumours
117
What are the different types of **bone marrow biopsies**?
**Aspiration** = liquid sample of cells from bone marrow
**Trephine** = solid core sample for better assessment of cells
**Biopsy** = from iliac crest, with local anaesthetic and specialist needle (examined straight away, trephine sample takes a few days of preparation)
118
What is **ALL**?
Malignant change in a **lymphocyte precursor cell** causing proliferation of single type of lymphocyte (usually **B-cells**) leading to **pancytopaenia**
Peaks at 2-4 years old and most common cancer in children
Associated with **Down's syndrome**
119
What does a **blood film** show on **ALL**?
**Blast cell****s**
120
What **genetic defect** is **ALL** associated with?
**Philadelphia chromosome** (t(9:22) translocation) in 30% of adults and 3-5% of children
121
What is **chronic lymphocytic leukaemia**?
**Chronic** proliferation of single type of well differentiated lymphocyte (usually B-lymphocytes)
122
How does **CLL** present?
Often **asymptomatic** but can present with **infections** e.g. anaemia, bleeding and weight loss
Can cause **warm autoimmune haemolytic anaemia**
123
What can CLL transform into? What is this called?
**High-grade lymphoma** called **Richter's transformation**
124
What does a **blood film** show for **CLL**?
**Smear** or **smudge cells** (occur during process of preparing blood film where fragile WBC rupture)
125
What are the **three phases** to **chronic myeloid leukaemia**?
**Chronic phase** = 5 years, asymptomatic, incidental diagnosis from raised WCC
**Accelerated phase** = abnormal blast cells take high proportion of cells in bone marrow and blood (10-20%), more symptomatic, anaemic, thrombocytopaenic, immunocompromised
**Blast phase** = high proportion of blast cells and blood (\>30%) severe symptoms and pancytopaenia, often **fatal**
126
What **cytogenic change** is **characteristic of CML**?
**Philadelphia chromosome:** translocation of genes between **chromosome** **9** and **22**
**t(9:22)** translocation
127
What is **acute myeloid leukaemia**?
**Most common** acute leukaemia in **adults**
Can result from **myeloproliferative disorder** e.g. **polycythaemia ruby vera** or **myelofibrosis**
Blood film shows high proportion of **blast cells** which have **rods** inside their **cytoplasm** and are named **auer rods**
128
What are the causes of **massive splenomegaly**?
**CML**
Myelofibrosis
Malaria
Gaucher's syndrome
129
What is helpful in exams to remember for:
ALL
CLL
CML
AML?
**ALL** = children and downs
**CLL** = most common leukaemia in adults overall, associated with warm haemolytic anaemia, Richter's transformation, smudge / smear cells
**CML** = three phases including 5 year asymptomatic chronic phase, philadelphia chromosome
**AML** = most common adult leukaemia, result of transformation from myeloproliferative disorder, associated with Auer rod
130
What is the **management** of **leukaemia**?
_MDT_
Treatment mainly **chemo** and **steroids**
Other treatments = RT, bone marrow transplant, surgery
131
What are some **complications** of **chemo**?
**Failure**
**Stunted growth** / development in children
**Infections** due to **immunodeficiency**
**Neurotoxicity**
**Infertility**
**Secondary malignancy**
**Cardiotoxicity**
**Tumour lysis syndrome**
132
What is **tumour lysis syndrome** caused by?
Release of **uric acid** from cells being destroyed in chemo (potassium and phosphate is also released)
133
What can result from **high uric acid**?
Forms **crystals** in the **interstitial tissue** and **tubules** of the **kidneys** causing **AKI**
134
Which medications are used to **reduce high uric acid levels**?
**Allopurinol** or **rasburicase**
High phosphate can cause low calcium so this is also monitored
135
What are **lymphomas**?
Cancers affecting **lymphocytes** inside the **lymphatic system** causing cancerous cells to proliferate in **lymph nodes**
136
Where may the **enlarged lymph nodes** be found in **lymphomas**?
**Cervical**
**Axillary**
**Inguinal**
137
What are the **two main categories** of **lymphoma**?
**Hodgkin's lymphoma**
**non-Hodgkin's lymphoma**
138
What amount of lymphomas are Hodgkin's?
What age do people get it?
1 in 5
20 and 75 years
139
Whar are the **risk factors** for **Hodgkin's lymphoma**?
**HIV**
**EBV**
**Autoimmune conditions** e.g. RA and sarcoidosis
**FH**
140
How does **Hodgkin's** **lymphoma** present?
**Lymphadenopathy** (non-tender and rubbery)
Sometimes pain in lymph nodes when drinking alcohol
**B symptoms** (fever, weight loss, night sweats)
Other symptoms = fatigue, itching, cough, SoB, abdo pain, recurrent infections
141
What are the **investigations** for **lymphomas**?
**LDH** - often raised in Hodgkin's lymphoma (not specific)
**Lymph node biopsy** (key diagnostic test - looking for **Reed-Sternberg** cell = abnormally large B cell with multiple nuclei and nucleoli inside)
**CT, MRI** and **PET** can be used for diagnosing and staging lymphoma
142
What staging system is used for HL and NHL? What are the stages?
**Ann Arbor**
Stage 1: confined to one region of lymph nodes
Stage 2: more than one region but same side of diaphragm
Stage 3: above and below diaphragm
Stage 4: widespread involvement including non-lymphatic organs e.g. lungs or liver
143
What is the **management** of **lymphomas**? What are the **associated risks**?
**Chemotherapy** = leukaemia and infertility
**RT** = cancer, damage to tissues, hypothyroidism
144
What are a few **notable non-Hodgkin's lymphomas**?
**Burkitt lymphoma** = associated with EBV, malaria and HIV
**MALT lymphoma** = affects the **mucosa-associated lymphoid tissue** usually around the **stomach**, associated with H. pylori infection
**Diffuse large B cell lymphoma** = rapidly growing painless mass in patients over 65 years
145
What are some **risk factors** for **non-Hodgkin's lymphoma**?
HIV
EBV
H. pylori (MALT lymphoma)
Hepatits B or C infection
Exposure to pesticides and specific chemical called **trichloroethylene** used in several industrial processes
**FH**
146
How does NHL present?
Similarly to HL (only differentiated when lymph node is biopsied)
147
What is the **management** of **lymphoma**?
Watchful waiting
Chemotherapy
Monoclonal antibodies e.g. **rituximab**
RT
Stem cell transplantation
148
What is **myeloma**?
Cancer of the **plasma cells** (type of B lymphocyte that produce **antibodies**)
149
What is **multiple myeloma**?
Myeloma affects **multiple areas of the body**
150
What is **monoclonal gammopathy of undetermined significance**?
Excess of a **single type of antibosy** / antibody components without other features of myeloma (incidental finding in otherwise healthy person, significance is unclear, routinely followed up)
151
What is **smouldering myeloma**?
Progression of **MGUS** with higher levels of **antibodeies** or antibody components
**Premalignant** and **more likely to progress to myeloma** than MGUS
152
What is **Waldenstrom's macroglobulinemia**?
Type of **smouldering myeloma** with excessive **IgM** specifically
153
What are **antibodies**? What are they also called?
What happens to antibodies in myeloma?
**Immunoglobulin** - molecules made up from **two heavy chains** and **two light chains** arranged in a Y shape
There are **5 main types**: A, G, M, D and E
In **myeloma** one of these will be significantly abudant (mostly **IgG**) - this single type of antibody is called a **monoclonal paraprotein**
154
What is the **Bence Jones protein**?
Found in the **urine** of many patients with **myeloma** (part of a **subunit** of antibody called **light chains**)
155
What happens when **cancerous plasma cells** invade the bone marrow? What is this also called?
**Bone marrow infiltration** (anaemia, neutropenia, thrombocytopenia)
156
What is **myeloma bone disease**?
Result of **increased osteoclast** activity and suppressed **osteoblast** activity - more bone is reabsorbed then constructed
Caused by **cytokines** released from **plasma cells** and **stromal cells** (other bone cells) when incontace tiwh plasma cells
157
Where does **myeloma bone disease** typically affect?
**Skull**
**Spine**
**Long bones**
**Ribs**
Thin bone described as **osteolytic** leading to **pathological fractures** e.g. **vertebral body** in spine may collapse
158
What is another result of **osteoclast** activity?
**Hypercalcaemia**
159
What tumours can patients with myeloma develop?
**Plasmacytomas** - individual tumours made up of **cancerous plasma cells** (occur in bones, replacing normal bone tissue)
160
Why does **myeloma renal disease** occur?
High levels of **immunoglobulins** (antibodies) blocking flow through tubules
**Hypercalcaemia** impairs renal function
Dehydration
Medications used to treat myeloma e.g. **bisphosphonates** can be **harmful to kidneys**
161
What happens to the **viscosity** of **blood** in **myeloma**?
**Hyperviscous** due to large amounts of immunoglobulins
162
What can **raised** plasma viscosity cause?
Easy **bruising**
Easy **bleeding**
Reduced or **loss of sight** due to vascular disease in eye
**Purple discolouration to extremities** (purplish palmar erythma)
**Heart failure**
163
What **4 features** of **myeloma** to remember for exams?
**CRAB**
**C**alcium (elevated)
**R**enal failure
**A**naemia (normocytic, normochromic) from replacement of bone marrow
**B**one lesions / pain
164
What are the **risk factors** for **myeloma**?
Older age
Male
Black African ethnicity
Family history
Obesity
165
When should **myeloma** be suspected? What investigations to order?
**FBC** (low WBC count)
**Calcium** (raised)
**ESR** (raised)
**Plasma viscosity** (raised)
166
What further tests if any initial results return positive for **myeloma**?
Urgent **serum protein electrophoresis**
Urgent **Bence-Jones** protein test
167
What are some more **specific** tests for **myeloma**?
**B** - Bence-Jones protein (requent **urine electrophoresis**)
**L** - Serum-free **L**ight-chain assay
**I** - Serum **I**mmunoglobulins
**P** - Serum **P**rotein electrophoresis
**Bone marrow biopsy** to confirm diagnosis of myeloma
**Imaging** (in order of preference) = whole body MRI, whole body CT, skeletal survey (x-ray images of full skeleton)
168
What are the **x-rays** signs of **myeloma**?
Punched out lesions
Lytic lesiosn
"**Raindrop skull**" caused by **many punched out** (lytic) lesions through skull
169
What is the **management** of **myeloma**?
**_MDT_**
* Chemo = bortezomid, thalidomide, dexamethasone
* **Stem cell transplantation** as part of clinical trial
* **VTE** prophylaxis with aspirin / LMWH when on certain chemos e.g. thalidomide as higher risk of developing thrombus
170
What is the **management** of **myeloma bone disease**?
**Bisphosphonates** (suppress osteoclast activity)
**Radiotherapy** to bone lesions for pain
**Orthopaedic surgery** to stabalise bones (e.g. inserting prophylactic intramedullary rod) or treating fractures
**Cement augmentation** - injecting cement into **vertebral fractures** or **lesions** to improve spine stability and pain
171
What are some **complications** of **myeloma**?
Infection
Pain
Renal failure
Anaemia
Hypercalcaemia
Peripheral neuropathy
Spinal cord compression
Hyperviscosity
172
When do **myeloproliferative disorders** occur? What are they considered as?
Uncontrolled **proliferation** of a single type of stem cell
Type of **bone marrow cancer**
173
What **myeloproliferative disorders** are there to **remember?**
**Primary myelofibrosis** = proliferation of haematopoietic stem cells
**Polycythaemia vera** = proliferation of erythroid cell line
**Essential thrombocytopaenia** = proliferation of **megakaryocytic cell line**
174
What can **myeloproliferative disorders** progress into?
**Acute myeloid leukaemia**
175
What arhe the **mutations** associated with **myeloproliferative disorders**?
JAK2
MPL
CALR
176
What can **myelofibrosis** result from?
**Primary myelofibrosis, polycythaemia vera** or **essential thrombocythaemia**
177
What happens in **myelofibrosis**?
Proliferation of cell line leads to **fibrosis** of the **bone marrow** (replaced by scar tissue) in response to **cytokines** that are released from **proliferating cells** (important cytokine = fibroblast growth factor)
Affects production of blood cells and leads to **anaemia** and low white blood cells (**leukopenia**)
178
Where else is haematopoiesis after **myelofibrosis**?
**Haematopoiesis** = liver and spleen (extramedullary haematopoiesis) - hepatomegaly and splenomegaly leading to **portal hypertension**
If occuring in spin = **spinal cord compression**
179
How do **myeloproliferative disorders** present?
**Systemic symptoms** = fatigue, night sweats, weight loss, fever
**Underlying condition signs** = anaemia (except in polycythaemia), splenomegaly (abdo pain), low platelets (bleeding and petechiae), **thrombosis** is common in polycythaemia and thrombocythaemia, **raised blood cells** (thrombosis and red face), **low white blood cells** (infections)
180
What are the **three key signs** on **examination** for **polycythaemia vera**?
**Conjunctival plethora** (redness in conjunctiva of eye)
**Ruddy** complexion
**Splenomegaly**
181
What are the FBC findings for the various myeloproliferative disorders?
**Polycythaemia vera** = raised Hb
**Primary thrombocythaemia** = raised platelet count
**Myelofibrosis** (due to primary MF or secondary to PV or ET) can give variable findings - **anaemia**, **leukocytosis** or **leukopenia** (high or low white cell count), **thrombocytosis** or **thrombocytopenia** (high or low platelet counts)
182
What will a **blood film** show in **myelofibrosis**?
**Teardrop-shaped RBC****s**, varying sizes of RBC (**poikilocytosis**) and immature red and white cells (blasts)
183
How are **myeloproliferative disorders** diagnosed?
**Bone marrow biopsy** (aspiration is normally 'dry' as bone marrow has turned to scar tissue)
184
What can help guide management in myeloproliferative disorders?
**JAK2**
**MPL**
**CALR** genes
185
What is the **management** of **primary myelofibrosis**?
If disease minimal then monitor with no active treatment
**Allogenic stem call tranplantation** (potentially curative but carries risk)
**Chemo** to control disease, improve symptoms and slow progression but not curative alone
**Supportive management** of anaemia, splenomegaly and portal hypertension.
186
What is the **management** of **polycythaemia vera**?
**Venesection** - keeps Hb in normal range (first line treatment)
**Aspirin** - reduces risk of developing blood clots (**thrombus formation**)
**Chemotherapy** - controls disease
187
What is the management of **essential thrombocytopenia**?
**Aspirin** reduces the risk of developing blood clots (thrombus formation)
**Chemo** to control disease
188
What is **myelodysplastic syndrome**?
Caused by **myeloid bone marrow cells** not maturing properly and not producing healthy blood cells
Causing low levels of:
**Anaemia**
**Neutropenia** (low neutrophil count)
**Thrombocytopenia** (low platelets)
189
When is **myelodysplastic syndrome** most common?
What is the risk associated?
Patients **above 60 years old** and previous treatment with **chemo** / **radio**
Risk it may turn into **acute myeloid leukaemia**
190
How may patients with myelodysplastic syndrome present?
Symptoms of **anaemia** (fatigue, pallor, SoB)
**neutropenia** (severe / frequent infections)
**thrombocytopenia** (purpura / bleeding)
191
How is **myelodysplastic syndrome diagnosed**?
**FBC** abnormal
**Blasts** on blood film
Diagnosis from **bone marrow aspiration** and **biopsy**
192
What are the **management options** for **myelodysplastic syndrome**?
**Watchful waiting**
**Supportive treatment** with **blood transfusions** if severely anaemic
**Chemotherapy**
**Stem cell transplantation**
193
What is **thrombocytopenia**? What is the **normal range**?
Low platelet count
Normal = between 150 and 450
194
What can cause **problems with production** of **platelets**?
**Sepsis**
**B12** or **folic acid deficiency**
Liver failure causing reduced **thrombopoietin** production in the liver
**Leukaemia**
**Myelodysplastic syndrome**
195
What can cause **problems with destruction** of **platelets**?
**Medications** (sodium valporate, methotrexate, irotretinoin, antihistamines, proton pump inhibitors)
## Footnote
**Alcohol**
**Immune thrombocytopenic purpura**
**Therombotic thrombocytopenic purpura**
**Heparin-induced threombocytopenia**
**Haemolytic-uraemic syndrome**
196
How does **thrombocytopenia** present on blood film?
**Asymptomatic** and found incidentally
**Below 50** x 109/L = easy / spontaneous bruiding / prolonged bleeding (nose bleeds, bleeding gums, heavy periods, blood in urine / stools)
**Below 10** x 109/L high risk of spontaneous bleeding (intracranial / GI)
197
What are some **differentials** for **abnormal** / **prolonged bleeding**?
**Thrombocytopenia** (low platelets)
**Haemophilia A** and **haemophilia B**
**Von Willebrand disease**
**Disseminated intravascular coagulation** (usually secondary to sepsis)
198
What are the other names for autoimmune thrombocytopenic purpura?
Idiopathic thrombocytopenic purpura
Primary thrombocytopenic purpura
199
What is **ITP**?
Condition where **antibodies** are created against **platelets**
Causing immune response and destruction
200
What are the **management options** for **ITP**?
**Prednisolone** (steroids)
IV **immunoglobulins**
**Rituximab** (a monoclonal antibody against B cells)
**Splenectomy**
201
What are the **extra** management steps for **ITP**?
Platelet count monitored
Education on **persistent headaches** and **melaena**
Careful control of **blood pressure** and suppressing **menstrual periods**
202
What is **thrombotic thrombocytopenic purpura**?
**Tiny blood clots** form in **small vessels** (microangiopathy) using up **platelets** and causing thrombocytopenia and bleeding under skin
203
How does TTP present?
**Fever**
**Neuro signs**
**Thrombocytopenia**
**Haemolytic anaemia**
**Renal failure**
204
Why does **thrombotic thrombocytopenic purpura** occur?
Problem with protein called **ADAMTS13** (normally **inactivates von Willebrand factor** and reduces **platelet adhesion** to vessel walls and **clot formation**)
Blood clots that form break up causing **haemolytic anaemia**
205
Why does a **deficiency** in **ADAMTS13** protein occur?
Inherited **genetic mutation**
**Autoimmune disease** (antibodies created against protein)
206
What is the **treatment** of TTP?
**Plasma exchange**
**Steroids**
**Rituximab** (monoclonal antibody against B cells)
207
What is **hepatin induced thrombocytopenia** (HIT)?
Development of **antibodies** against platelets in response to **exposure to heparin** (specifically target protein on the platelets called **platelet factor 4** (PF4)
These are **anti-PF4** / **heparin antibodies**
208
What is the **action** of the **HIT antibodies**?
Bind to platelets and **acitvate clotting mechanisms** causing **hypercoagulable state** leading to **thrombus** (also break down platelets and cause **thrombocytopenia** (patient on heparin with low platelets forms blood clots)
209
How is a **diagnosis** of **HIT** made?
What is the management?
Testing for **HIT antibodies**
Stopping heparin and using **alternative anticoagulant** guided by specialist
210
What is **Von Willebrand disease** (VWD)?
Most common **inherited** cause of **abnormal bleeding** (**haemophilia**) many **different genetic causes** most of which are **autosomal dominant**- causing deficiency / absence / malfunctioning **glycoprotein** called **von Willebrand factor** (VWF)
Three types based on underlying cause, type 3 = most severe
211
How to patients with VWD present?
**Bleeding gums** with brusing
Nose bleeds (**epistaxis**)
Heavy menstrual bleeding (**menorrhagia**)
Heavy bleeding during surgical operations
**Family history** of VWD is **very relevant**
212
How is a **diagnosis** of **VWD** made?
History of **abnormal bleeding**, **family history**, **bleeding assessment tools** and lab investigations
Due to all underlying causes = **no easy test** = diagnosis is challenging
213
What is the **management** of **VWD**?
Usually **no day to day treatment**, management = for controlling major bleeding / prep for operations
**Desmopressin** can stimulate release of VWF
**VWF** can be infused
**Factor VIII** infused along with plasma-derived VWF
Control of **heavy periods** (tranexamic, mefanamic acid, norethisterone, COCP, mirena coil)
**Hysterectomy** in severe cases
214
What are **haemophilia A** and **haemophilia B**?
**Inherited severe bleeding disorders**
Haemophilia A = deficiency in **factor VIII**
Haemophilia B (aka christmas disease) = deficiency in **factor IX**
215
What is the **pattern of inheritance** of **haemophilia A and B**?
**X linked recessive**
men only require one abnormal copy as they only have one X chromosome, women require both (if one = carrier)
Haemophila A and B almost exclusively affect males
216
What are the **signs and symptoms** of **haemophilia A** and **B**?
Severe bleeding disorders (bleed excessively in response to minor trauma and risk of **spontaneous haemorrhage**)
Presentation in neonates = **intracranial haemorrhage**, **haematomas** and **cord bleeding**
Spontaneous bleeding into **joints** (**haemoathrosis**) and **muscles** are a classic feature (untreated = joint damage and deformity)
217
Where can **abnormal bleeding** occur in **haemophilia**?
**Gums**
**GI tract**
**Urinary tract** = haematuria
**Retroperitoneal** space
**Intracranial**
**Following procedures**
218
How is **haemophilia** diagnosed?
**Bleeding scores**
**Coagulation factor assays**
**Genetic testing**
219
What is the **management** of **haemophilia**?
**Affected clotting factors** (VIII or IX) replaced by **IV infusion** either **prophylactically** or in **response to bleeding** (complication = **antibodies** against the **clotting factor** - treatment will be unaffective)
**_Acute episodes of bleeding_**
* **Infusion** of the affected factor (VIII or IX)
* **Desmopressin** to stimulate the release of **von Willebrand factor**
* **Antifibrinolytics** e.g. **tranexamic acid**
220
How can a **VTE** cause a **stroke**?
Hole in heart (**atrial septal defect**)
221
What are the **risk factors** for **VTE**?
**Immobility**
Recent **surgery**
**Long haul flights**
**Pregnancy**
Hormone therapy with **oestrogen** (COCP and HRT)
**Malignancy**
**Polycythaemia**
**SLE**
**Thrombophilia**
222
What are **thrombophilia****s**? Give some**examples**?
Conditions which **predispose patients** to **blood clots**
e.g:
**Antiphospholipid syndrome**
Antithrombin deficiency
Protein C or S deficiency
Factor V Leiden
Hyperhomocysteinaemia
Prothrombin gene variant
Activated protein C resistance
223
What is the **VTE** prophylaxis if a patient is deemed at risk on admission?
What are some contraindications?
**LMWH** (e.g. enoxaparin)
Contraindications = active bleeding, existing anticoagulation with **warfarin** or a **NOAC**
**Antiembolic compression stockings** (unless peripheral arterial disease)
224
How does **DVT present**?
Calf / leg **swelling** (measure circumference of cald 10cm below tibial tuberosity \> 3cm difference is significant)
**Dilated superficial veins**
**Tenderness** to **calf** (particularly over site of deep veins)
**Oedema**
**Colour changes to the leg**
Ask **questions** incase of **PE**
225
What is **Wells score**?
Risk of patient with symptoms actually **having DVT** or **pulmonary embolism** (takes into account recent surgery, clinical findings)
226
What **other conditions** may cause a raised D-dimer?
Pneumonia
Malignancy
Heart failure
Surgery
Pregnancy
227
How is a VTE diagnosed?
**DVT** = ultrasound doppler (repeat in 6-8 days if negative and positive D-dimer with Wells score suggesting DVT)
**Pulmonary embolism** = diagnosed with **CT pulmonary angiogram** or **ventilation**-**perfusion** (VQ) scan
228
What is the **initial management** of **VTE**?
Treatment dose **LMWH** (started immediately) e.g. **enoxaparin / dalteparin**
229
What is the **long term anticoagulation** in **VTE**?
**Warfarin** (target INR = 2-3, continue LMWH for 5 days when switching or INR is 2-3 for 24 hours, whichever longer)
**NOAC** / **DOAC** (oral anticoagulation that aren't warfarin, require less monitoring, originally called '**novel oral anticoagulants**' changed to '**non-vitamin K oral anticoagulants**' because no longer novel - changing to **direct acting oral anticoagulants** e.g. **apixaban**, **dapifatran** and **rivaroxaban**
**LMWH** is first line in pregnancy / cancer
**LMWH**
230
How long to **continue** anticoagulation for in **VTE**?
**3 months** = obvious reversible cause (then review)
**Beyond 3 months** = cause unclear, recurrent VTE, irreversible underlying cause e.g. thrombophilia (review at 6 months)
**6 months** in active cancer (then review)
231
What are **inferior vena cava filters**?
Devices fitted into **inferior vena cava** to filter blood (used in **recurrent PEs** or those that are unsuitable for anticoagulation)
232
How to **investigate unprovoked DVT**?
Investigate for **possible cancer**:
* History and examination
* CXR
* Bloods (FBC, calcium and LFTs)
* Urine dip
* **CT abdo and pelvis** in patients over 40
* **Mammogram** in women over 40
Investigate for **antiphospholipid syndrome** by checking for **antiphospholipid antibodies**
If **family history** then test for **hereditary thrombophilias**:
* Factor V Leiden (most common hereditary thrombophilia)
* Prothrombin G20210A
* Protein C
* Protein S
* Antithrombin
233
What is **Budd-Chiari syndrome**?
Where blood clot (thromobosis) develops in the **hepatic veins** blocking the outflow of blood (associated with **hyper-coagulable state**)
Causes **acute hepatitis**
Classic **triad**:
* Abdo pain
* Hepatomegaly
* Ascites
**Management** = anticoagulation (heparin / warfarin) investigating for underlying cause of hyper-coagulation and treating hepatits
234
What are **irradiated blood products**?
Who should recieve them?
Blood **depleted of T-lymphocytes** used to avoid transfusion associated graft versus host disease
* Granulocyte transfusions
* Intra-uterine transfusions
* Neonates (up to 28 post expected dates of delivery)
* Bone marrow / stem cell transplants
* Immunocompromised
* Hodgkin's disease
235
Name some **transfusion complications**?
**Immunological** = acute haemolytic, non-haemolytic febrile, allergic / anaphylaxis
**Infective**
**TRALI** (transfusion related acute lung injury)
**TACO** (transfusion related circulatory overload)
Other (hyperkalaemia, iron overload, clotting)
236
What causes **non-haemolytic febrile reaction**?
How does it present?
How is it managed?
**Antibodies** react to WBC fragments and cytokines (often the result of sensitisation by previous pregnancies)
**Presents** as chills, fever
**Manage** by slowing / stop transfusion and **paracetamol**
237
What causes **minor allergic reaction**?
How does it present?
What is the management?
Caused by **foreign plasma proteins**
**Presents** with pruritus and urticaria
**Managed** by **temporarily stopping transfusion** and **antihistamine**
238
What can cause **anaphylaxis** during blood transfusion?
How does it present?
How is it managed?
Thought to be caused by **patients with IgA deficiency** and **high anti-IgA antibodies**
Presents with **hypotension**, **dyspnoea**, **wheezing and angiooedema** within minutes
**Managed by stopping** transfusion, IM adrenaline and ABC support (oxygen, fluids) also consider corticosteroids or bronchodilators
239
Why does **acute haemolytic reaction** occur in blood transfusions?
How does it present?
How to manage?
What are some complications?
**ABO incompatibility** - IgM mediated (stop transfusion)
**Presents** as fever, abdo pain and hypotension **within minutes**
**Manage** by stopping transfusion, confirm pt ID, **DCT,** fluid (generous with saline) and resus
**Complications** = DIC and renal failure
240
How does TACO (transfusion associated circulatory overload occur?
How does it **present**?
How is it **managed**?
Caused by **excessive rate of transfusion** and / pre-existing heart failure
**Presents** as pulmonary oedema and **hypertension!!**
**Managed** with slowing / stopping transfusion and IV loop diuretics e.g. furosemide and oxygen
241
What causes **TRALI** (transfusion related acute lung injury)?
How does it **present**?
What is the **management**?
**Non-cardiogenic pulmonary oedema** (due to increased vascular permeability caused by host neutrophils)
**Presents** as hypoxia, pulmonary infiltrates on CXR, fever, hypotension within **6 hours**
**Managed** by **stopping the transfusion** and **oxygen and supportive care**
242
What is the **MOA** of dabigatran, rivaroxaban and apixaban?
**Rivaroxaban** and **apixaban** = Direct factor Xa inhibitor
**Dabigatran** = Direct thrombin inhibitor
243
What class of drug is **tranexamic acid**?
When is it given?
**Antifibrinolytic** given for menorragia and trauma (within 3 hrs as IV bolus followed by slow infusion)
244
What are some contraindications to platelet transfusions (even below 10)?
Chronic **bone marrow failure**
**Autoimmune thrombocytopenia**
**Heparin-induced thrombocytopenia**
**Thrombotic thrombocytopenic purpura**
245
What factors carry a worse prognosis for Hodgkin's lymphoma?
Male
\>45
B symptoms
Hb \<10.5 g/dL
**Lymphocyte depleted**
246
What are the indications for NOACs?
**Prevention of VTE following hip/knee surgery**
Treatment of **DVT and PE**
**Prevention of stroke in non-valvular AF**
