Haematology Flashcards
(133 cards)
1
Q
Haemophilia A + B have which inheritance pattern?
A
X-linked recessive
2
Q
Is haemophilia A or B more common?
A
A
3
Q
Haemophilia A is a deficiency of which clotting factor?
A
Factor 8
4
Q
Haemophilia B is a deficiency of which clotting factor?
A
Factor 9
5
Q
How is mild haemophilia defined
A
Haematomas following severe trauma
6
Q
How is moderate haemophilia defined
A
Haematomas following mild trauma
7
Q
How is severe haemophilia defined
A
Spontaneous haematomas
8
Q
Haemophilia causes ? PT and ? APTT
A
Normal PT
Prolonged APTT
9
Q
What special test can you do with blood to confirm a diagnosis of haemophilia?
A
Mixing study
Mixing patients blood with external plasma will correct the APTT
10
Q
Besides replacing factor 8, what else can be used to treat haemophilia A?
A
Desmopressin (vasopressin triggers the release of factor VIII from endothelial cells)
11
Q
What is the most common inherited bleeding disorder?
A
Von Willebrand Disease
12
Q
In VWD what happens to bleeding time, PT and APTT?
A
Bleeding time increased
PT normal
APTT sometimes normal, sometimes raised
13
Q
VWD causes high/low factor 8 levels?
A
Low
Factor VIII levels are a good assessment for vWD because this factor is found bound to vWF in blood, in which state it is protected from rapid breakdown
14
Q
How do you manage VWD?
A
Recombinant VWF
Desmopressin (stimulated VWF release from endothelial cells)
Factor 8 concentrate
15
Q
Which type of VWD won’t respond to Desmopressin?
A
Type 3
16
Q
What is the inheritance pattern for type 1 VDW?
A
Autosomal dominant
17
Q
What is the inheritance pattern for type 2 VWD?
A
Autosomal dominant
18
Q
What is the inheritance pattern for type 3 VWD?
A
Autosomal recessive
19
Q
How is type 1 VWD described?
A
A quantitative deficiency of VWF
20
Q
How is type 2 VWD described?
A
A qualitative defect in VWF
21
Q
How is type 3 VWD described?
A
Complete absence of VWF
22
Q
What are the two main types of acute leukaemia?
A
Acute lymphocytic leukaemia
Acute myeloid leukaemia
23
Q
Does ALL more commonly affect children or adults?
A
Children
24
Q
Does AML more commonly affect children or adults?
A
Adults
25
Which cells develop from the lymphocytic cell line?
Natural killer cells
B cells --> plasma cells
T cells
26
Which cells develop from the myeloid cell line?
Basophils
Eosinophils
Neutrophils
27
Describe the pathophysiology of acute leukaemia
Acquired gene mutations affect how bone marrow cells divide and mature
Either lymphoid/myeloid stem cells proliferate but differentiation/maturation stops in the early stages
This results in accumulation of abnormal and dysfunctional blasts
This disrupts normal haematopoiesis --> leukopenia, thrombocytopenia, anaemia
Abnormal cells also infiltrate organs such as liver, spleen, nodes, skin
28
Risk factors for ALL
Risk factors = often none, HTLV (Human T-cell lymphotropic virus), Down syndrome, neurofibromatosis type 1, ataxia telangiectasia
29
Risk factors for AML
Risk factors = pre-existing haematopoietic disorder (myelodysplastic syndromes, aplastic anaemia, myeloproliferative disorders), previous chemotherapy or radiation, smoking, benzene exposure, Down syndrome, Fanconi anaemia
30
Clinical features of acute leukaemia
Sudden onset, rapid progression over days/weeks
Fatigue, pallor, weakness
Bleeding, petechiae, purpura
Frequent infections, fever
31
Features that are more common/specific to AML rather than ALL
Leukaemia cutis/myeloid sarcoma (blue/puruple/grey nodular skin lesions)
Gingival hyperplasia
Extremely high WBC counts that can cause leukostasis
32
Features that are more common/specific to ALL rather than AML
```
Fever and constitutional sx
Lymphadenopathy
Hepatosplenomegaly
Bone pain
Mediastinal involvement
CNS involvement
```
33
Are Auer rods seen in ALL or AML
AML
34
What is 'leukaemic hiatus' on blood film?
A way to differentiate AML from CML
| In CML myeloid cells of all stages are present whereas in AML its mainly blasts and mature (no in-between).
35
What are the nuclei of lymphoblasts like in ALL
Large and irregular
36
What are the nuclei of myeloblasts like in AML
Round or kidney shape
37
Is myeloperoxidase positive or negative in ALL and AML
Negative in ALL
| Positive in AML
38
What symptoms/problems can leukostasis cause?
Chest pain, headache, altered mental status, priapism, DIC
39
What is tumour lysis syndrome?
Rapid destruction of tumour cells causing a massive release of intracellular components --> renal damage
40
What happens to K, Ca, PO4 and urea in tumour lysis syndrome?
High K
High PO4
Low Ca
High urea
41
What are some clinical features of tumour lysis syndrome?
Nausea, vomiting, diarrhoea, lethargy, haematuria, seizures, arrhythmias, tetany, muscle cramps, paraesthesia
42
What is the basic pathological difference between acute and chronic leukaemia?
In acute leukaemia cells dont mature
| In chronic leukaemia cells don't work effectively (CLL cells dont die as they should, CML cells divide too quickly)
43
What are the two main types of chronic leukaemia?
Chronic lymphocytic leukaemia
| Chronic myeloid leukaemia
44
Who tends to be affected by CLL?
Elderly men
45
What are some clinical features of CLL?
```
Constitutional/B symptoms
Painless lymphadenopathy
Recurrent infections
Sx of anaemia
Sx of thrombocytopenia
Chronic pruritus/urticaria
Hepatomegaly/splenomegaly
```
46
What would an FBC show in CLL?
Persistent lymphocytosis with high percentage of small mature lymphocytes
Granulocytopenia
Anaemia
Thrombocytopenia
47
What does blood film show in CLL?
Smudge cells (Gumprecht shadows)
48
Is CLL a low or high grade malignancy?
Low grade
49
What is Richter's transformation/syndrome?
Transformation of CLL into a high grade NHL
50
How can Richter's transformation/syndrome present?
Rapidly progressive lymphadenopathy
New onset of B symptoms
High LDH
51
What are the three distinct clinical phases of CML?
Chronic
Accelerated
Terminal/Blast crisis
52
Describe the chronic phase of CML
The chronic phase is characterized by nonspecific symptoms (fever, weight loss, night sweats) and splenomegaly and can persist for up to 10 years
53
Describe the accelerated phase of CML
The accelerated phase is characterized by complications secondary to the suppression of the other cell lines (thrombocytopenia, anemia, neutropenia, recurrent infections, leukostasis). Develop extreme splenomegaly in accelerated phase and this is often a sign of transition to terminal phase.
54
Describe the terminal phase/blast crisis of CML
The clinical picture of the terminal phase, blast crisis, resembles that of acute leukemia. Rapid progression of bone marrow failure causes pancytopenia and bone pain Blast crisis (progression of CML to AML) occurs when increased division of myeloid cells in CML leads to increased mutations resulting in totally immature cells being produced (AML).
55
Is hepatosplenomegaly more common in CML or CLL?
CML
56
What drug is really good at treating CML?
Imatinib (tyrosine kinase inhibitor)
57
What would FBC show in CML?
Extrememe leukocytosis
Basophilia
Thrombocytosis
58
What is lymphoma
A type of blood cancer that causes tumours to form in lymph nodes
59
What are the two broad types of Hodgkin lymphoma
Classical
| Nodular lymphocyte predominant
60
What are the four types of classical Hodgkin lymphoma?
Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted
61
Where does nodular sclerosing classical hodgkin lymphoma most commonly localise?
Mediastinal and cervical lymph nodes
62
Where does mixed cellularity classical hodgkin lymphoma most commonly localise?
Abdominal and splenic lymph nodes
63
Where does lymphocyte rich classical hodgkin lymphoma most commonly localise?
Cervical and axillary nodes
64
What are some risk factors for developing hodgkin lymphoma?
EBV mononucleosis
Immunodeficiency - HIV, meds, chemo
Autoimmune diseases
65
Clinical features of hodgkin lymphoma
```
Painless lymphadenopathy
Sx of mediastinal mass (chest pain, SOB)
B symptoms
Hepatosplenomegaly
Pel-Ebstein fever (temp for 1-2 weeks, normal for 1-2 weeks)
Alcohol induced pain
Pruritus
```
66
Reed-Sternberg cells are pathognomic for X?
Hodgkin Lymphoma
67
What is the name of the classification system for Hodgkin lymphoma?
Ann-Arbor classification
68
Describe the Ann-Arbor classification for HL
I. 1 lymph node area
II. 2+ but confined to one side of the diaphragm
III. 2+ on both sides of the diaphragm
IV. Disseminated spread into an extralymphatic organ. IVA if asymptomatic, IVB if B symptoms, IVX if bulky disease
69
Which chemo regimen is typically used for HL
ABVD - adriamycin (doxorubicin), bleomycin, vinblastine, dacarbazine)
70
Is HL or NHL more common?
NHL
71
How are non-hodgkin lymphomas categorised?
B cell or T cell
| High grade or low grade
72
Are B-cell or T-cell NHL more common?
B cell
73
What are the low grade B-cell non-hodgkin lymphomas?
```
Chronic lymphocytic leukaemia
Hairy cell leukaemia
Waldenstroms macroglobulinaemia
Follicular lymphoma
MALT lymphoma
```
74
What is the most common low-grade non-hodgkin lymphoma?
Follicular lymphoma
75
What are the high-grade non-hodgkin lymphomas?
Burkitt lymphoma
Diffuse large B cell lymphoma
Precursor B cell lymphoblastic lymphoma
Mantle cell lymphoma
76
What is the most common NHL in adults?
Diffuse large B cell lymphoma
77
What is the low grade T cell NHL?
Mycosis fungoides (cutaneous lymphoma)
78
What are the high grade T cell NHLs?
Adult T cell lymphoma
Cutaneous T cell lymphoma
Precursor T cell lymphoblastic lymphoma
79
What are the risk factors for developing NHL
```
Chromosomal translocations
EBV
HIV
HTL
HCV
H.pylori (MALT)
Autoimmune disease
Immunodeficiency
Benzene exposure
Radiation
```
80
Clinical features of NHL
```
Painless lymphadenopathy (insidious onset in low grade, generalised in high grade)
Splenomegaly
Hepatomegaly
GI/skin/thyroid/CNS involvement
B symptoms
Fatigue/weakness
Anaemia
Bleeding
Infections
```
81
FBC findings in NHL
Anaemia
High/low WBC
Thrombocytopenia
High LDH
82
All NHL is treated with a curative approach apart from which category?
Advanced low-grade
83
Multiple myeloma is a malignant disease causing uncontrolled proliferation of which type of cell?
Plasma cells
84
Clinical findings of multiple myeloma
```
Bone pain (back, spontaneous fractures)
Sx of hypercalcaemia
B symptoms
Weakness and anaemia
Recurrent infection
Petechial bleeding
Foamy urine (caused by Bence Jones proteinuria)
```
85
Blood test findings in multiple myeloma
```
Anaemia
Thrombocytopenia
Leukopenia
Low reticulocyte count
Raised ESR
High Ca
High creatinine
High total protein levels
Raised beta-2-microglobulin
```
86
Diagnostic criteria for multiple myeloma
```
Marrow biopsy showing >10% monoclonal plasma cells plus one of:
Hypercalcaemia
Renal insufficiency
Anaemia
Bone lesions
>60% monoclonal plasma cells in marrow
```
87
What is TTP?
Thrombotic thrombocytopenic purpura
| A thrombotic microangiopathy
88
Who typically gets affected by TTP?
Previously healthy adults
89
What caused TTP?
Acquired autoantibodies that result in accumulation of von Willebrand factor
90
What are the 5 key clinical findings in TTP?
1. Fever
2. Neurological signs/symptoms
3. Thrombocytopenia
4. Haemolytic anaemia
5. Impaired renal function
91
How do you manage TTP
Treat without waiting to confirm diagnosis
Fluid status, electrolytes, acid base
Plasma exchange/FFP
Prednisolone
92
Who is typically affected by HUS?
Children <5 after 5-10 days of diarrhoea (usually bloody)
93
What is HUS?
Haemolytic uraemia syndrome
| A thrombotic microangiopathy
94
What causes HUS?
Usually secondary to E.coli 0157:H7 producing shiga-like toxin
95
What are the 3 key clinical findings of HUS?
1. Thrombocytopenia
2. Haemolytic anaemia
3. Renal insufficiency
96
What is the management for HUS?
Treat without waiting to confirm diagnosis
Avoid antibiotics and anti-motility agents
Fluid status
Electrolytes
Acid-base balance
BP
97
When should you suspect ITP?
Isolated thrombocytopenia
98
Who gets affected by ITP?
Children after a viral illness (acute ITP)
| Women of childbearing age (chronic, insidious onset)
99
What is the management of ITP?
Usually just watch and wait - spontaneously resolves within 6 months for most children
In adults, if platelet count is <30,000 can give corticosteroids
100
Causes of microcytic anaemia
```
IDA (late phase)
Chronic disease (late phase)
Sideroblastic anaemia
Lead poisoning
Thalassaemia
```
101
What does blood film show in sideroblastic anaemia?
Basophilic stippling
| Ringed sideoblasts
102
Causes of normocytic anaemia
```
Sickle cell disease
Pyruvate kinase/G6PD deficiency
Hereditary spherocytosis
Autoimmune haemolytic anaemia
Microangiopathic haemolytic anaemia (TTP, HUS)
Blood loss
Aplastic anaemia
CKD
IDA (early phase)
Chronic disease (early phase)
```
103
Causes of megaloblastic macrocytic anaemia
Vitamin B12 deficiency
Folate deficiency
Medication - Phenytoin, trimethoprin, hydroxyurea, MTX
Fanconi anaemia
104
Causes of non-megaloblastic macrocytic anaemia
```
Liver disease
Myelodysplastic syndrome
Diamond Blackfan anaemia
Hypothyroidism
Multiple myeloma
```
105
How can you differentiate megaloblastic and non-megaloblastic anaemia on blood film?
Megaloblastic has hypersegmented neutrophils, non-megaloblastic doesn't
106
What can be used to reverse the effects of unfractionated heparin?
Protamine sulphate
107
How long does Warfarin take to reach therapeutic range?
48-72 hours
108
What is Virchow's triad?
Virchows triad = the three main pathophysiological components of thrombus formation
1. Hypercoagulability – increased platelet adhesion, increased clotting tendency (thrombophilia)
2. Endothelial damage – inflammatory, traumatic
3. Venous stasis – varicose veins, immobilisation, local heat application
109
Risk factors for DVT
```
History of VTE
Immobilisation
Age >60
Malignancy
Hereditary thrombophilia (Factor V Leiden)
Pregnancy
Estrogen use
Obesity
Smoking
```
110
Clinical features of DVT
Unilateral
Swelling
Heavy/tight feeling
Progressive tenderness/dull pain - worse when walking, better when leg elevated
Homan sign (pain on ankle dorsiflexion)
Superficial vein distention
Calf circumference >3cm difference (mark 10cm from tibial tuberosity)
111
DVT differentials
```
Post-traumatic swelling
Haematoma
Lymphoedema
Venous insufficiency
Ruptured popliteal cyst
Cellulitis
Compartment syndrome
```
112
How id D-dimer used in the assessment of VTE?
Used to rule it out if Wells score is low probability
113
What is the minimum length of anticoagulation prophylaxis after VTE?
3 months
114
Clinical features of PE
```
Acute onset
Dyspnea
Tachypnea
Sudden onset pleuritic chest pain
Cough
Haemoptysis
Tachycardia +/- hypotension
JVP distention
Co-existing DVT
```
115
What may an ABG show in PE?
Respiratory alkalosis with hypocapnia
116
What is a massive PE?
A large clot which affects perfusion of the right side of the heart
117
A Wells score of ? makes DVT likely
2 or more
118
A Wells score of ? makes PE likely?
More than 4
119
Which coagulation pathway does PT measure?
Extrinsic
120
Which coagulation pathway does APTT measure?
Intrinsic
121
What can we used to measure the common coagulation pathway?
Fibrinogen
| TT, PT and APTT all affected
122
What can cause a prolonged PT
```
Warfarin
Factor 7 deficiency
Factor 2 deficiency
Factor 5 deficiency
Factor 10 deficiency
```
123
What can cause a prolonged APTT
```
Heparin
Factor 8 deficiency
Factor 9 deficiency
Von Willebrands Disease
Factor 11 deficiency
Factor 12 deficiency (but no bleeding)
```
124
Mouth slits on RBCs on a blood film indicates what disease?
Hereditary stomatocytosis
125
What kind of cells are seen on blood film in G6PD deficiency?
Bite cells
126
What kind of cells are seen on blood film in pyruvate kinase deficiency?
Sputnik cells with protruding spikes
127
What is the inheritance pattern for thalassaemia?
Autosomal recessive
128
What is splenic sequestration crisis in sickle cell disease?
Occlusion of splenic artery causes blood to pool and get trapped in the spleen causing LUQ pain, anaemia, reticulocytosis and hypotension
129
What causes aplastic crisis in sickle cell disease?
Parvovirus B19 infection
130
Which medication can be used to try and prevent sickle cell crisis?
Hydroxyurea
131
What can trigger sickle cell crisis?
```
Reduced oxygen tension/hypoxia
Dehydration
Stress
Infection
Pregnancy
Acidosis
```
132
How do you manage acute sickle cell crisis?
```
Hydration
Analgesia
Nasal oxygen
VTE prophylaxis
Bed rest
Exchange transfusions if severe
```
133
Difference in blood test results between Von Willebrands disease and Haemophilia A
Both with have prolonged APTT
VWD will have prolonged bleeding time
HA will have normal bleeding time
