Haematology Flashcards
(143 cards)
1
Q
What is myeloma?
A
Cancer of differentiated B lymphocyte plasma cells
2
Q
Name 4 key features of myeloma
A
-Accumulation of malignant plasma cells in bone marrow
-Characteristic paraprotein
-Kidney failure
-Bone disease + hypercalcaemia
3
Q
What is the mnemonic for myeloma?
A
C-Ca >0.25mmol/l
R-Renal impairment
A-Anaemia
B-Bone lesions + pain
4
Q
What is the most common complication of myeloma?
A
Anaemia
5
Q
What are some of the most common sites for myeloma bone disease?
A
-Skull
-Spine
-Long bones
-Ribs
6
Q
What causes myeloma bone disease?
A
Increased osteoclast activity + suppressed osteoblast activity-imbalanced bone metabolism, more bone reabsorbed
7
Q
What causes fractures in patients with myeloma?
A
Myeloma bone disease forms osteolytic lesions-thin patches of bone which fracture easily
8
Q
What is MGUS?
A
Monoclonal gammopathy of undetermined significance
-Production of a specific paraprotein without other features of myeloma
9
Q
What is the chance of progression from MGUS to myeloma?
A
1%
10
Q
What is multiple myeloma?
A
Myeloma affecting multiple bone marrow areas
11
Q
What is smouldering myeloma?
A
Abnormal plasma cells + paraproteins but no organ damage/symptoms
12
Q
What is the chance of progression from smouldering myeloma to myeloma?
A
10%
13
Q
How do paraproteins damage kidneys?
A
Form protein casts in renal tubules
14
Q
What are the Ig classes of myeloma?
A
-IgG - 2/3 cases
-IgA - 1/3 cases
-IgD + IgM - rare
15
Q
What conditions can myeloma cause?
A
-Anaemia
-Thrombocytopenia
-Renal failure
-Infection
16
Q
What investigations would you order for suspected myeloma?
A
-Bone marrow biopsy
-Whole-body MRI
-X-ray
-Protein electrophoresis of blood + urine for paraprotein band
-FBC
-Calcium
-ESR
-Plasma viscosity
-U+E
17
Q
How is myeloma managed?
A
*Chemotherapy
*Stem cell transplant (<65yrs)
*Bisphosphonates
-Radiotherapy
-Orthopaedic surgery
-Cement augmentation
-Pain killers
-Antibiotics
18
Q
What is the prognosis for myeloma?
A
-5-year survival 35%
-High dose chemo + stem cell transplant = 4.5yrs average survival
19
Q
What are the aims of myeloma Tx?
A
-Reduce myeloma cells
-Reduce symptoms + complications
-Improve QofL + length of life
20
Q
What is HSCT?
A
Haematopoietic stem cell transplant
Any procedure where haematopoietic stem cells are given to a recipient with intention of repopulating/replacing haematopoietic system
21
Q
What is normally done before HSCT?
A
Chemotherapy to control cancer
22
Q
What are the 2 types of stem cell transplant?
A
Autologous
Allogeneic
23
Q
What is autologous stem cell transplant?
A
Where stem cells are obtained from the patient
24
Q
What is allogeneic stem cell transplant?
A
Where stem cells come from a donor
25
What loci is looked at for transplant matching?
HLE-human leukocyte antigen loci
A,B,C,DP,DQ,DR
26
What is a full match for transplants?
10/10
27
What is a half match for transplants?
5/10
28
What is a mismatch for transplants?
8 or 9/10
29
What does HLA do?
Presents peptides to T cells, for elimination of foreign particle + recognition of self
30
What are the components of a pre-transplant evaluation?
-Medical Hx, frailty factors
-Cardiac assessment
-Pulmonary assessment
-Renal + liver function
-Infectious disease markers
-Psychosocial evaluation
-Bone marrow biopsy-disease status
-Fertility counselling + preservation if relevant
31
What is conditioning chemotherapy + what is its purpose?
Chemo given just before stem cell infusion
Aim to suppress host immune system
32
What is GvHD?
Graft versus host disease, can be mild->life-threatening, rejection of donor lymphocytes
33
What is first line therapy for acute GVHD?
Corticosteroids
34
What is VOD/VOS?
Veno-occlusive disease/sinusoidal obstruction syndrome
Reaction to stem cell transplant
35
What are the key presenting features of VOD/VOS?
-Jaundice
-Tender hepatomegaly
-Fluid accumulation-rapid weight gain/ascites
36
What is prophylaxis for VOD/SOS?
-Heparin
-LMWH
-Ursodiol
37
What is lymphoma?
Neoplastic, clonal proliferation of lymphoid cells, typically affects lymph nodes
38
What are the classifications for lymphomas?
-Hodgkin vs non-Hodgkin lymphoma
-Non-H splits into aggressive and indolent
39
What is the most common type of cell that lymphomas come from?
B cells
40
What are the characteristics of indolent lymphoma?
-Slow growing
-Advanced at presentation
-'Incurable'
41
What are some risk factors for indolent lymphoma?
-HIV
-Trabsplant recipients
-Infection e.g. EBV, Helicobacter pylori
-Autoimmune disorders
-Family history
42
What is the main presentation for indolent lymphomas?
Painless lymphadenopathy (node enlargement)
43
What are B-symptoms?
Systemic symptoms of lymphoma e.g.
Fever
Weight loss
Night sweats
44
What is the main diagnostic investigation for lymphoma?
Lymph node biopsy
45
What is the key biopsy finding for Hodgkin's lymphomas?
Reed-Sternberg cells-cancerous B lymphocytes
46
What are additional investigations for lymphoma?
-CT
-MRI
-PET scan
47
What is the staging system for lymphomas?
Lugano-stages 1-4
48
What are the main Txs for non-H lymphoma?
-Watchful waiting
-Chemo
-Radiotherapy
-Monoclonal antibodies
-Stem cell transplant
Tx depends on grading
49
What are the Lugano stages?
1-1 node group
2-more than 1 groups but 1 side of diaphragm
3-both sides of diaphragm
4-widespread, includes non-lymphatic organs
50
Describe the bimodal age distribution with Hodgkin's lymphoma
Most common in:
-20-25
-80
51
What are the risk factors for Hodgkin's?
-HIV
-EBV
-Autoimmune conditions e.g. rheumatoid arthritis
-FH
52
What are Reed-Sternberg cells?
Large cancerous B lymphocytes with 2 nuclei + prominent nucleoli-owl w/ large eyes
53
What is Hodgkin's lymphoma?
When B lymphocytes mutate + create Reed-Sternberg cells + Hodgkin cells
54
What are the 2 types of Hodgkin's?
-Classical (95% cases)
-Nodular
55
What are the 4 subclasses of classical Hodgkin's?
-Nodular sclerosis
-Mixed cellularity
-Lymphocyte-rich
-Lymphocyte-depleted
56
What is the most common symptom of Hodgkin's (HL)?
Painless, rubbery enlarged lymph nodes, often in cervical/supraclavicular region
57
What is a classical textbook symptom of HL but not often seen?
Alcohol-induced pain at nodes
58
What are some symptoms of HL?
-B symptoms (night sweats, weight loss etc)
-Chest discomfort
-Abdo discomfort
-Pruritis-itching
-Fatigue
59
Name 5 differential diagnoses for HL
-Infectious mononucleosis
-Non-H lymphoma
-AIDS
-TB
-Leukaemia
-Myeloma
60
What are the key investigations for HL?
-Lymph node biopsy
-Chest x-ray
-Contrast enhanced CT
-PET-CT
61
What is the gold standard for staging in classical HL?
PET-CT
62
How is HL managed?
-Up to date on vaccinations
-Combination chemo w/ radiotherapy-strength + quantity depends on staging
63
How is relapsed HL normally treated?
-High dose chemo w/
-Autologous stem cell transplant
64
What complications are associated with HL chemo?
-Infection
-Cognitive impairment
-Secondary cancers
-Infertility
65
What complications are associated with HL radiotherapy?
-Tissue fibrosis
-Secondary cancers
-Infertility
66
What is important to remember about blood products + HL patients/patients who have been treated for HL in past?
Can only receive irradiated blood products-for life
67
What is leukaemia?
Cancer of a line of stem cells in the bone marrow causing proliferation of of 'blasts'
68
What is pancytopenia?
-Low RBC, WBC + platelets due to underproduction
69
What are myelodysplastic syndromes?
Syndrome where bone marrow fails to make adequate healthy blood cells + abnormal cells crowd out remaining ones
70
What are the 2 characteristics of AML?
-Immature myeloid cell proliferation >20% blasts
-Bone marrow failure
71
What is AML?
Uncontrolled growth of myeloid cell line in bone marrow
72
What are the 4 types of leukaemia?
-Acute myeloid leukaemia
-Acute lymphoblastic leukaemia
-Chronic myeloid leukaemia
-Chronic lymphocytic leukaemia
73
What age group do most leukaemias affect?
60+
74
Which leukaemia most commonly affects children + is associated with Down's?
ALL
75
Name some differential diagnoses for AML
-B12/folate deficiency
-Infection
-Meds
-Autoimmune
-Liver disease
76
How would you investigate suspected AML?
-History
-FBC
-Blood film
-Haematinics (B12, folate, ferritin etc)
77
What are the risk factors fir AML?
-Most de novo malignancy BUT
-Congenital e.g. Down's, Bloom
-Environmental exposure-prior chemo, radiation, smoking etc
-Pre-existing MDS, MPD, aplastic anaemia
78
How does leukaemia present?
-Non-specific:
-fatigue
-Fever
-Pallor
-Petechiae
-Abnormal bleeding
-Failure to thrive
79
Investigations for leukaemia?
-FBC
-Blood film
-Bone marrow biopsy
-CT/PET
-Genetic testing
-Lactate dehydrogenase (LDH)
80
How is a bone marrow biopsy used in the diagnosis of leukaemia?
Used for staging-% of blasts
81
What analysis is performed on bone marrow samples?
-Immunophenotyping
-Cytogenetics-chromosome study
-FISH-fluorescence in situ hybridisation
-Molecular analyses
82
What is a characteristic finding on blood film with AML?
Auer rods
83
What are the 2 classification systems for AML?
*WHO classification
-ICC classification
84
How is AML treated?
Chemo:
-Induction
-Consolidation
-Maintenance
85
What 3 factors affect prognosis with AML?
-Type of AML
-Age at diagnosis
-Presence of comorbidities
86
What are the most commonly used chemo drugs in AML?
-Cytarabine
-Daunorubicin
-ATRA
87
What is tumour lysis syndrome?
Syndrome caused by chemicals released when cells are destroyed in chemo
88
What are the symptoms of tumour lysis syndrome?
-High uric acid
-High potassium
-High phosphate
-Low calcium
89
What are the side effects of chemo?
-Cytopenias
-Bystander organ damage
-Hair loss
-Reduced fertility
-N+V
-Fatigue
-Loss of appetite
90
Name 4 supportive therapies for AML
-Blood cell transfusions
-Analgesia
-Antimicrobial prophylaxis
-Anti-emetics
91
What is anaemia?
Low conc of haemoglobin in blood
-<115 in women
-<130 in men
92
What is MCV?
Mean cell volume-size of RBC
93
What is a normal MCV?
80-100 femtolitres
94
What are the 3 MCV categories of anaemia?
-Microcytic (low MCV,<80)
-Normocytic (normal MCV, 80-100)
-Macrocytic (large, >100)
95
What is a normal haemoglobin level for women?
110-147g/l
96
What is a normal haemoglobin level for men?
131-166g/l
97
What are the causes of microcytic anaemia + pneumonic?
TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
98
What are the causes of normocytic anaemia?
-Chronic disease
-Renal disease
-Acute bleeding
99
What are the causes of megaloblastic macrocytic anaemia?
-B12 deficiency
-Folate deficiency
-Bone marrow disorders
-Haemolysis
100
What is reticulcytosis?
Increased conc of reticulocytes-immature RBC-rapid turnover of RBC due to e.g. blood loss
101
What are the causes of normoblastic macrocytic anaemia?
-Alcohol
-Reticulocytosis
-Hypothyroidism
-Liver disease
-Drugs
102
Name 2 specific symptoms of iron deficiency anaemia
-Pica-abnormal food cravings
-Hair loss
103
Name 6 generic anaemia symptoms
-Fatigue
-SOB
-Dizziness
-Palpitations
-Headaches
104
How much iron do you need in a normal diet?
15mg/day
105
What should you assume about the cause of iron deficiency until proven otherwise?
Assume blood loss e.g. p/r, heavy periods, GI
106
What happens to iron lvls in pregnancy?
Foetus takes iron, levels drop-lots need supplements
107
What conditions impair iron absorption?
-Coeliac disease
-Gastrectomy
108
What are some specific signs of iron deficiency anaemia?
-Koilonychia-spoon-shaped nails
-Angular cheilitis-red, swollen corners of mouth
-Atrophic glossitis-smooth tongue
-Brittle hair + nails
109
What kind of anaemia can jaundice indicate?
Haemolytic
110
How is anaemia managed?
-Treat any source of blood loss
-Replace iron-ferrous sulphate 20mg 1-3 daily
111
What blood tests could you do for anaemia?
-FBC
-Reticulocyte
-Blood film
-Ferritin
-B12 + folate
-LFTs
112
What is a normal level of folate?
>3.9ug/l
113
Where is folate absorbed?
Proximal jejunum
114
What are the main causes of folate deficiency?
-Poor nutrition
-Coeliac
-Crohns
-Pregnancy
-Haemolysis
115
Name some sources of B12
-Meat
-Fish
-Eggs
-Dairy
116
Describe the absorption of B12
Absorbed in terminal ileum by binding to intrinsic factor
117
What is B12 needed for?
-DNA synthesis
-Fatty acid synthesis
118
What are some causes of B12 deficiency?
-Pernicious anaemia
-Gastrectomy
-Vegan diet
-Oral contraceptives
-Nitric oxide
119
What is pernicious anaemia?
Autoimmune gastric atrophy + loss of intrinsic factor production
120
Name 4 types of anaemia
-Iron deficiency
-Pernicious
-Haemolytic
-Sickle cell
121
What is haemolysis?
Increased RBC destruction
122
What investigations would you do for suspected haemolysis?
-Blood film
-Reticulocyte count
-Bilirubin
-Lactate dehydrogenase
-Direct antiglobulin test-Coombs
123
What are some causes of haemolysis?
-RBC disorders
-Sickle cell
-Haemolytic anaemias
-Prosthetic heart valves
-Autoimmune haemolytic anaemias
124
Which should be check + replaced first, B12 or folate + why?
B12, high folate risks neuro damage
125
What are the main 3 causes of B12 deficiency?
-Pernicious anaemia
-Low dietary B12
-Meds e.g. PPI, metformin
126
What is the inheritance pattern for sickle cell?
Autosomal recessive
127
What is HbS?
Sickle cell
128
How does sickle cell cause ischaemia?
-HbS polymerises when deoxygenated
-Blocks blood vessels=ischaemia, sequestration
129
How is sickle cell diagnosed?
-Sickle solubility test
-Hb separation
130
What are some of the complications associated with sickle cell?
-Acute painful crisis
-Stroke
-Sequestration (blood pools in liver/spleen)
-Acute anaemia
131
What does someone with only one abnormal copy of haemoglobin gene have?
Sickle-cell trait-carrier, normally asymptomatic
132
What things can trigger sickle cell crises?
-Dehydration
-Infection
-Stress
-Cold weather
133
How is sickle cell disease treated (general)?
-Supportively:
-Treat infections
-Keep warm
-Vaccinations
-Good hydration
-Antibiotic prophylaxis
134
How is sickle cell disease treated (medically/surgically)?
-Hydroxycarbamide to increase HbF
-Crizanlizumab-monoclonal antibody
-Blood transfusions
-Cure=bone marrow transplant
135
What is sickle cell disease?
Abnormal mutation in Beta globin gene-causing abnormal haemoglobin production
136
What is thalassaemia?
Autosomal recessive condition caused by genetic defect in protein chains making up haemoglobin-causes anaemia
137
What are the different types of thalassaemia?
-Alpha thalassaemia
-Beta thalassaemia-splits into minor + intermedia
138
How does thalassaemia cause haemolytic anaemia?
-RBC more fragile, break down easily
139
How does thalassaemia cause splenomegaly?
More old, destroyed RBC in thalassaemia-they are collected by the spleen
140
What are the typical presenting features of thalassaemia?
-Microcytic anaemia
-Fatigue
-Pallor
-Jaundice
-Gallstones
-Poor growth/development
-Splenomegaly
141
What investigations would you do for thalassaemia?
-Ferritin
-Mean cell volume
-Haemoglobin electrophoresis
142
Describing screening for thalassaemia
-Antenatal routinely offered
-New UK arrivals
-Pre-surgery
-Guthrie test-day 5 heel prick, neonates
143
