Haematology Flashcards
1
Q
What is haematopoeisis?
A
The formation of different types of blood cells
Too much (hyperfunction) can result in splenomegaly
2
Q
What are B symptoms?
In what do they occur?
A
- Fever
- Night sweats
- Weight loss >10% body weight
Occurs in lymphoma and some leukaemias
3
Q
How can you measure heparin and what reversal agent may be used?
A
APTT - raised (intrinsic pathway)
Protamine can reverse it
4
Q
Give some causes of an elevated APTT?
A
- Heparin
- Haemophilia
- VWD
- Antiphospholipid syndrome
5
Q
What part of the coagulation cascade does heparin act on?
A
Intrinsic pathway - can monitor it by measuring APTT
Heparin activate antithrombin, which activates thrombin and Xa. Unfractionated heparin has a short half-life.
(LMWH is subcut, can monitor through anti Xa level)
6
Q
What are the four different types of leukaemia?
A
- Acute myeloid leukaemia
- Acute lymphoblastic leukaemia
- Chronic myeloid leukaemia
- Chronic lymphoblastic leukaemia
7
Q
What is the most common type of leukaemia in adults and children respectively?
A
Adults - CLL
Children - ALL
8
Q
Which leukaemia is associated with the Philadelphia chromosome and what is the genetic fault?
A
CML
9;22 translocation
Can occur in ALL in which it indicates a poor prognosis
9
Q
In the different types of leukaemia, are there any specific cells that are abnormal?
A
AML - auer rods and neutrophils on blood film, increased in blast cells
ALL - lymphocytes
CLL - lymphocytes
10
Q
What is Richter transformation?
A
When CLL turns into non-Hodgkin’s Lymphoma
11
Q
What is your drug of choice for CML?
A
Imatinib
12
Q
What do auer rods indicate on blood film?
A
AML
13
Q
What symptoms might someone with an acute leukaemia present with?
A
- Malaise
- Anorexia
- Fever
- Anaemia
- Pallor
- Lymphadenopathy
- Neutropenia
- Hepatosplenomegaly
14
Q
What is a Blast Crisis and in what does it occur?
A
It occurs in CML
Is is an increase in lymphoblast cells in the blood or bone marrow resulting in a fever, bone pain, fatigue and severe anaemia
15
Q
What investigations should you do to investigate a potential leukaemia?
A
Bloods: raised WCC, blood film with blast cells
CXR: mediastinal widening
Bone marrow aspirate
16
Q
What are the 5 phases of chemotherapy treatment in leukaemias?
A
- Induction
- Consolidation and CNS protection
- Interim maintenance
- Delayed intensification
- Continuing maintenance
17
Q
What are some defining features of chronic lymphocytic leukaemia?
A
- elderly
- lymphocytosis (increased WCC)
- smudge/smear cells on blood film
18
Q
What are the two main types of thalassaemia?
A
Alpha - 4 types
Beta - 3 types
19
Q
What are the 4 kinds of alpha thalassaemia?
A
Depends on how many alpha chains are normal.
4 normal = fine
3 normal = asymptomatic, borderline if they have any symptoms
2 normal = slightly anaemic
1 normal = HbH, very anaemic, microchromic, splenomegaly, bone changes
0 normal = HbBarts, incompatible with life, hydrops fetalis
20
Q
How is thalassaemia treated and the side effects managed?
A
- regular blood transfusions
- iron chelation (desferrioxamine)
21
Q
What investigations can you do?
A
Hb electrophoresis
22
Q
What are your INR targets for AF, VTE?
A
AF - 2.5
VTE 2.5 (3.5 if recurrent)
INR<2
23
Q
How is warfarin reversed?
A
Stop warfarin
Vitamin K (takes 4-24hrs)
FFP
Human prothrombin complex (beriplex, takes 1hr)
24
Q
What blood result gives your best indicator of warfarin other than INR?
A
PT - extrinsic pathway
25
What investigation do you do for hereditary spherocytosis and what do you see?
EMA binding assay
spherocytes
26
What is the name for too many and too few platelets?
Thrombocytosis - too many
| Thrombocytopenia - too many
27
What are your management options for myeloma?
- bone marrow transplant
- bisphosphonates
- blood transfusion
28
What investigations would be abnormal in myeloma? What other investigations would you want to do?
- IgG, IgA - raised
- serum electrophoresis + urine (Bence-Jones Protein)
- proteinaemia
- bone marrow biopsy
- U&E check kidney function due to light chain depositions
- calcium - raised in myeloma
29
What investigations are used to diagnose Beta-thalassaemia?
FBC - microcytic hypochromic anaemia
Haemoglobin electrophoresis
DNA testing
30
What is your management of beta thalassaemia major?
What is a problem with this?
Regular blood transfusions
Iron induced oxidative stress of liver, heart, brain, requires iron chelation therapy
31
EMA binding assay is for…?
Hereditary spherocytosis
32
What is the only thing the indirect Coombs test is used for?
Rhesus haemolytic disease of the newborn
33
How might a myeloma present?
Back pain
Bone pain, bone destruction
Haematuria, hypercalcaemia
Systemic - malaise, anaemia, fever, recurrent infections
34
What is a myeloma a malignancy of?
Bone marrow plasma cells
35
What is CRAB an acronym for and which condition is it applicable?
Calcium
Renal (creatinine raised)
Anaemia
Bone (lytic lesions)
36
What will you see on the investigations performed in a myeloma patient?
Raised calcium
Raised urea
Urine protein electrophoresis - immunoglobulin light chains (Bence-Jones protein)
24-hour urine immunofixation - paraproteinemia, raised IgG, IgA
Blood film - rouleaux formation
'Pepperpot skull' on CXR/PET
37
How are myelomas managed?
Thalidomide (NOT IN PREGNANCY)
Transplantation
Chemo + Radiotherapy
38
What supportive treatment is given in myelomas?
Bisphosphonates
Blood transfusions for anaemia
Prophylactic antibiotics
39
What are the three types of beta thalassaemia?
Minor - asymptomatic carriers, the trait is present
Intermedia - less severe anaemia
Major (Cooley's anaemia) transfusion dependent
40
In thalassaemia patients on regular transfusions what is a major complication and a way to combat this?
Iron deposition and overload
| Treat with chelation (desferrioxamine)
41
What are some inherited and acquired causes of thrombophilia?
Inherited: Factor V Leiden
Antithrombin deficiency
Acquired: antiphospholipid syndrome, cancer, Behcet's disease, liver disease or nehrotic syndrome
42
What are some risk factors for Lymphoma?
Hodgkin's Lymphoma - EBV
| NHL - Toxins, Family History
43
What indicates a worse prognosis in Hodgkin's Lymphoma?
Lymphocyte depleted
44
What is your main medical management of sickle cell disease?
Hydroxycarbamide
Definitive treatment is stem cell transplant
45
What are some potential complications of polycythaemia?
Gout
| Thrombosis
46
What can cause polycythaemia?
Dehydration
| COPD and sleep apnoea
47
How do you manage polycythaemia?
Venesection
| Aspirin
48
What are the main causes of microcytic anaemia?
Iron deficiency
| Chronic disease
49
What are the main causes of normocytic anaemia?
Blood loss
Chronic disease
Malignancy
50
What are the main causes of macrocytic anaemia?
B12 & Folate deficiency
Alcohol
Hypothyroidism
51
What is the genetic issue in haemophilia?
A - factor VIII deficiency
| B - factor IX deficiency
52
How is G6PD inherited?
X-linked inheritance
53
How is von willebrands disease inherited?
Autosomal dominant
54
How do you treat Von Willebrand's disease?
Transexamic acid
Desmopressin
AVOID aspirin and NSAIDs due to bleeding risk
55
What can trigger discovery of G6PD?
Antimalarials
| Infections
56
What should be done in patients with a raised INR/PT on warfarin?
Not bleeding – withhold warfarin until INR <5
Bleeding – medical emergency – seek senior help, discuss with haematologist, vitamin K 5mg STAT IV, consider prothrombin complex concentrate (Beriplex), withhold warfarin until INR <5.
57
How might haemophilia present?
Haemarthrosis – bleeding joints
| Usually in childhood
58
What are some causes of increased INR/PT? (increased bleeding risk)
Overcoagulation with warfarin
DIC
Acute liver failure
59
A patient presents with shortness of breath, tachycardia and isolated anaemia on bloods - suggest some differentials?
What are some important questions to ask about?
- PE
- HF
- COPD
- Anaemia
- Chest infection
- Pulmonary fibrosis
Ask about: DH (methotrexate will cause anaemia), menorrhagia, bleeding (GI, stool)
Investigations: Fe deficiency, B12/Folate
60
What is the difference between haematinics and a full iron profile?
Haematinics will only give ferretin which is an acute stage protein. A full iron profile will give you iron binding capacity etc.
61
What are some differentials for splenomegaly?
- malaria
- CML (myeloproliferative disorder)
- myelofibrosis
- lymphoproliferative disorders - CLL, lymphoma
- [sickle cell crisis only in sequestration, usually the spleen decreases in size]
- haemolytic disorders - anaemia, hereditary spherocytosis
EBV (infectious mononucleosis, glandular fever, monospot test)
- HIV, CMV, endocarditis
- RA/SLE
- Liver disorders - portal hypertension
62
What associated symptoms may occur in a patient presenting with splenomegaly?
- weight loss
- night sweats
- travel history
- fever
- sore throats
- FB
- bruising
- bleeding
- myalgia
63
What investigations would you want to do on someone with splenomegaly?
- blood film
- FBC
- monospot test
- CT abdomen
- LFT
- BBV screen
64
Acquired haemophilia is a deficiency of what?
Factor VIII
| - presents with PR bleed, sheek bleeding (abdominal bleeding)
65
What do you do with an abnormal clotting result?
- REPEAT sample
- check chronicity
- check anticoagulation
- liver disease/DIC
- request lupus anticoagulant screen - this is an autoantibody that interferes with the test
- Long APTT - request factor levels, but discuss before doing this with a haematologist
66
What factors are part of the extrinsic pathway?
PT
| VII
67
What factors are part of the intrinsic pathway?
VIII, IX, XI, XII
| APTT
68
What type of anaemia is iron deficiency anaemia?
Microcytic hypochromic
69
What does hypochromic mean?
pale, low mean cell haemoglobin
70
What is HbF?
Foetal haemoglobin, two alphas, two gammas
71
How is Sickle Cell Disease inherited?
Autosomal recessive
72
What chromosome is it on and what is the mutation?
Single amino acid, chromosome 11
73
What is the lifespan of a sickle cell?
20-30 days
74
What is seen on blood film for SCD?
Reticulocytes (due to increased production, RBC precursors with nuclear components)
75
What is a risk for sickle cell trait?
Sudden death in extreme exertion or severe hypoxia (anaesthetic, deep sea diving)
76
What are some classic presentation features of sickle cell disease?
Splenomegaly (multiple splenic infarcts and autosplenectomy), anaemia, increased infection risk (mainly pneumococcal)
77
How is a vaso-occlusive crisis managed?
Analgesia, O2 high flow, hydration, blood flow
78
What are some causes of iron deficiency anaemia?
Malnutrition, poor absorption, poor diet, menstruation, GI bleed, coeliac disease
79
What is a sickle cell crisis?
Vaso-occlusive (acute chest syndrome), aplastic crisis
80
What is a vaso-occlusive crisis?
Hb becomes deoxygenated at once. Cold, dehydration, hypoxia, lots of sickling and occlusion of vessels. There is pain due to oxygen deprivation. Swollen joints, dactylitis, acute abdomen (if mesenteric ischaemia or renal papillary necrosis).
81
What is acute chest syndrome?
vaso-occlusive crisis of lungs, new pulmonary infiltrate on CXR with dyspnoea, fever, pain, cough. Give O2, chest fever, NIV (if severe)
82
How is sickle cell disease managed?
regular management – avoid triggers (cold, dehydration, hypoxia, exhaustion, alcohol, smoking), folic acid supplements, oral pen V, full vaccinations
83
Give some complications of Sickle Cell Disease
Complications of SCD: chronic pain, PH, stroke, infection, gallstones, retinopathy, avascular necrosis of femoral or humoral heads
84
What are the 3 main causes of MASSIVE splenomagaly?
CML
Myelofibrosis
Malaria
85
What factor does rivaroxaban work on?
RivaroXaban - Xa
| Same as ApiXaban
86
What does dabigatran work on?
Direct thrombin inhibitor
87
What might you see on a blood film for hyposplenism (or post-splenectomy)?
Howell-Jolly bodies
| Target cells
88
Poikilocytes are seen in the blood films and may indicate what?
Iron deficiency anaemia
B12 deficiency
Myelofibrosis
89
Give some non-pathological causes of decreased Hb.
Pregnancy
Dehydration
IV fluid
90
What is the difference between thrombocytosis and thrombocytopaenia?
```
Thrombocytosis = increased platelets
Thrombocytopaenia = decreased platelets
```
91
What are classic symptoms of anaemia?
```
Fatigue
Palpitations
Anorexia
Sypnoea
Pallor (conjunctival)
Tachycardia
```
92
What are the three main pathophysiologies behind anaemia?
Impaired production
Increased red cell destruction
Blood loss
93
What are the different causes of microcytic anaemia?
Iron deficiency anaemia
Chronic disease
Thalassaemia
94
What are the different causes of normocytic anaemia?
Acute blood loss
Anaemia of chronic disease
Malignancy
95
What are the different causes of macrocytic anaemia?
B12/Folate deficiency
Alcohol
Hypothyroidism
96
What are some signs of iron deficiency anaemia?
Glossitis - inflammation of the tongue
Koilonychia - spoon shaped nails
Angular cheilitis - ulceration at the corner of the mouth
Post-cricoid webs - Plummer Vinson syndrome, characterised by difficulty swallowing and iron deficiency
97
What will be seen on a coagulation/iron screen in iron deficiency anaemia?
```
Low ferritin (transferrin, TIBC)
Low serum iron
```
98
What are your investigations of choice for iron deficiency anaemia?
Bloods: FBC, U&E, full iron
| Colonoscopy/OGD
99
What are some side effects of PO iron?
Black stools
Constipation
Upset stomach
Staining of teeth
100
What is Von Willebrands's disease?
A common inherited bleeding disorder, most are autosomal dominant
Results in prolonged bleeding time, prolonged APTT, reduced factor VIII
101
How do you manage Von Willebrand's Disease?
Transexamic acid - mild bleeding
Desmopressin - raises levels of vWF from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate
102
What is the main factor in cryoprecipitate and what are indications for transfusion?
Factor VIII
Massive haemorrhage
Uncontrolled bleeding due to haemophilia
Low fibrinogen level
103
What forms of anticoagulation can be used during pregnancy?
WARFARIN IS CONTRAINDICATED
| S/C LMWH - preferred to IV
104
When should a patient have an urgent OCG/colonoscopy?
Men with Hb under 110g/L
105
Haemarthrosis is a typical feature of what?
```
Haemophilia A and B
A tense, swollen joint, often knee
No history of trauma
No sign of injury
No fever
No fracture or arthritis on x-ray
```
106
What is the most common type of HL?
Nodular sclerosing
107
What is a lymphoma?
Malignant growth of WBC, occurs in lymph glands
108
What are the different types of HL?
Nodular sclerosing
Lymphocyte rich HL - high infiltrate
Mixed - worst prognosis, drenching sweats, fever, weight loss
Lymphocyte depleted - poor prognosis, Reed Sternberg cells
109
What are the two common types of NHL?
Follicular - Low grade
| Diffuse large B-cells - High grade
110
What is the classical presentation of lymphoma?
Painless lymphadenopathy (asymmetrical)
Weight loss, sweating, fever
Splenomegaly/Hepatomegaly
111
What may be seen on X-ray in Lymphoma?
Mediastinal widening or mass
112
What is a hallmark finding on biopsy of HL?
Reed-Sternberg cells, 'owl eyes'/popcorn cells
113
What is the staging criteria used for HL?
Ann Arbor
114
What are the drugs used in HL chemotherapy treatment?
```
ABVD
Adriamycin (doxorubicin)
Bleomycin
Vinblastine
Dacarbazine
```
115
What is myelofibrosis?
A myeloproliferative disorder
Anaemia symptoms, massive splenomegaly
Pokilocytes
116
What is an acute haemolytic transfusion reaction and what should be done?
Reaction to transfusion
Fever, rigors, decreasing BP, chest pain, dizziness
Terminate the transfusion, give fluid resuscitation with saline and inform the lab
117
What is seen on a biopsy in Burkitt's Lymphoma?
'Starry sky' densely packed basophilic cells interspersed with white areas containing cellular debris
118
What are symptoms of polycythaemia rubra vera?
Itching
Increased risk of thrombosis
Increased gout risk
Rosacea
119
How do you treat polycythaemia?
Aspirin
Venesection
Bone marrow suppressive drugs
120
What are some secondary causes of polycythaemia?
Hypoxia - COPD, altitude, obstructive sleep apnoea
121
What does a rouleaux formation on a blood film indicate?
Myeloma
122
Give five differentials of bone pain tenderness?
```
Myeloma
Sickle cell anaemia
Paget's disease of the bone
Hyperparathyroidism
Metastatic lung cancer
```
123
What kind of inheritance is Glucose-6-phosphate dehydrogenase deficiency?
X-linked
124
How can G6PD present?
Oxidative stress if infection, acidosis, drugs (nitrofurantoin), dietary requirements (fava beans)
Haemolytic anaemia - red blood cell breakdown - jaundice, red cell fragments on blood film, Heinz bodies on blood film
125
What does the clotting screen look like in DIC?
Low platelets
High PT
High APTT
Low fibrinogen
126
What medication can be used in sickle cell disease?
Hydroxycarbamide - increased foetal haemoglobin
Can offer stem cell transplant if frequent crises
127
What is the genetic defect in haemophilia?
X-linked recessive
Occurs only in boys
A - Factor VIII deficiency
B - Factor IX deficiency
128
What symptoms occur in haemophilia?
Prolonged bleeding, muscle bleeds and joint bleeds
| Easy bruising, mouth bleeds
129
What kind of inheritance is von Willebrand Disease?
Autosomal Dominant
130
What is ITP?
Immune Thrombocytopenic Purpura
Immunological thrombocytopaenia caused by antiplatelet autoantibodies resulting in immune destruction of platelets
Occurs acutely post-illness, commonly in children
131
What is tumour lysis syndrome?
Breakdown of malignant cells leading to a high uric acid, potassium, phosphate levels and low calcium.
Causes arrythmias, seizures or sudden death
132
What is amyloidosis?
Systemic amyloid deposits around the body, can occur primary or secondary to another disease
133
What are features of amyloidosis?
Racoon eyes
Unexplained HF
Unexplained weight loss
Renal symptoms
134
What are some acute and chronic reactions to blood transfusions?
Febrile reaction
Acute haemolytic reaction – ABO incompatibility, stop transfusion, tell haematologist and saline
Anaphylaxis – bronchospasm, cyanosis – stop transfusion and maintain airway
Bacterial contamination – low BP and rigors
TRALI
Non-haemolytic febrile transfusion reaction
135
Features of pancytopenia?
Infection, bleeding, bruising, pallor, fatigue, SOB
