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Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

What is myeloma?

A

Cancer of differentiated B lymphocyte plasma cells

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2
Q

Name 4 key features of myeloma

A

-Accumulation of malignant plasma cells in bone marrow
-Characteristic paraprotein
-Kidney failure
-Bone disease + hypercalcaemia

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3
Q

What is the mnemonic for myeloma?

A

C-Ca >0.25mmol/l
R-Renal impairment
A-Anaemia
B-Bone lesions + pain

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4
Q

What is the most common complication of myeloma?

A

Anaemia

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5
Q

What are some of the most common sites for myeloma bone disease?

A

-Skull
-Spine
-Long bones
-Ribs

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6
Q

What causes myeloma bone disease?

A

Increased osteoclast activity + suppressed osteoblast activity-imbalanced bone metabolism, more bone reabsorbed

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7
Q

What causes fractures in patients with myeloma?

A

Myeloma bone disease forms osteolytic lesions-thin patches of bone which fracture easily

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8
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance
-Production of a specific paraprotein without other features of myeloma

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9
Q

What is the chance of progression from MGUS to myeloma?

A

1%

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10
Q

What is multiple myeloma?

A

Myeloma affecting multiple bone marrow areas

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11
Q

What is smouldering myeloma?

A

Abnormal plasma cells + paraproteins but no organ damage/symptoms

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12
Q

What is the chance of progression from smouldering myeloma to myeloma?

A

10%

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13
Q

How do paraproteins damage kidneys?

A

Form protein casts in renal tubules

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14
Q

What are the Ig classes of myeloma?

A

-IgG - 2/3 cases
-IgA - 1/3 cases
-IgD + IgM - rare

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15
Q

What conditions can myeloma cause?

A

-Anaemia
-Thrombocytopenia
-Renal failure
-Infection

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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

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17
Q

How is myeloma managed?

A

*Chemotherapy
*Stem cell transplant (<65yrs)
*Bisphosphonates
-Radiotherapy
-Orthopaedic surgery
-Cement augmentation
-Pain killers
-Antibiotics

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18
Q

What is the prognosis for myeloma?

A

-5-year survival 35%
-High dose chemo + stem cell transplant = 4.5yrs average survival

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19
Q

What are the aims of myeloma Tx?

A

-Reduce myeloma cells
-Reduce symptoms + complications
-Improve QofL + length of life

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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

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21
Q

What is normally done before HSCT?

A

Chemotherapy to control cancer

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22
Q

What are the 2 types of stem cell transplant?

A

Autologous
Allogeneic

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23
Q

What is autologous stem cell transplant?

A

Where stem cells are obtained from the patient

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24
Q

What is allogeneic stem cell transplant?

A

Where stem cells come from a donor

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25
Q

What loci is looked at for transplant matching?

A

HLE-human leukocyte antigen loci
A,B,C,DP,DQ,DR

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26
Q

What is a full match for transplants?

A

10/10

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27
Q

What is a half match for transplants?

A

5/10

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28
Q

What is a mismatch for transplants?

A

8 or 9/10

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29
Q

What does HLA do?

A

Presents peptides to T cells, for elimination of foreign particle + recognition of self

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30
Q

What are the components of a pre-transplant evaluation?

A

-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

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31
Q

What is conditioning chemotherapy + what is its purpose?

A

Chemo given just before stem cell infusion
Aim to suppress host immune system

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32
Q

What is GvHD?

A

Graft versus host disease, can be mild->life-threatening, rejection of donor lymphocytes

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33
Q

What is first line therapy for acute GVHD?

A

Corticosteroids

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34
Q

What is VOD/VOS?

A

Veno-occlusive disease/sinusoidal obstruction syndrome
Reaction to stem cell transplant

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35
Q

What are the key presenting features of VOD/VOS?

A

-Jaundice
-Tender hepatomegaly
-Fluid accumulation-rapid weight gain/ascites

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36
Q

What is prophylaxis for VOD/SOS?

A

-Heparin
-LMWH
-Ursodiol

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37
Q

What is lymphoma?

A

Neoplastic, clonal proliferation of lymphoid cells, typically affects lymph nodes

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38
Q

What are the classifications for lymphomas?

A

-Hodgkin vs non-Hodgkin lymphoma
-Non-H splits into aggressive and indolent

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39
Q

What is the most common type of cell that lymphomas come from?

A

B cells

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40
Q

What are the characteristics of indolent lymphoma?

A

-Slow growing
-Advanced at presentation
-‘Incurable’

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41
Q

What are some risk factors for indolent lymphoma?

A

-HIV
-Trabsplant recipients
-Infection e.g. EBV, Helicobacter pylori
-Autoimmune disorders
-Family history

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42
Q

What is the main presentation for indolent lymphomas?

A

Painless lymphadenopathy (node enlargement)

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43
Q

What are B-symptoms?

A

Systemic symptoms of lymphoma e.g.
Fever
Weight loss
Night sweats

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44
Q

What is the main diagnostic investigation for lymphoma?

A

Lymph node biopsy

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45
Q

What is the key biopsy finding for Hodgkin’s lymphomas?

A

Reed-Sternberg cells-cancerous B lymphocytes

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46
Q

What are additional investigations for lymphoma?

A

-CT
-MRI
-PET scan

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47
Q

What is the staging system for lymphomas?

A

Lugano-stages 1-4

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48
Q

What are the main Txs for non-H lymphoma?

A

-Watchful waiting
-Chemo
-Radiotherapy
-Monoclonal antibodies
-Stem cell transplant
Tx depends on grading

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48
Q

What are the Lugano stages?

A

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

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49
Q

Describe the bimodal age distribution with Hodgkin’s lymphoma

A

Most common in:
-20-25
-80

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50
Q

What are the risk factors for Hodgkin’s?

A

-HIV
-EBV
-Autoimmune conditions e.g. rheumatoid arthritis
-FH

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51
Q

What are Reed-Sternberg cells?

A

Large cancerous B lymphocytes with 2 nuclei + prominent nucleoli-owl w/ large eyes

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52
Q

What is Hodgkin’s lymphoma?

A

When B lymphocytes mutate + create Reed-Sternberg cells + Hodgkin cells

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53
Q

What are the 2 types of Hodgkin’s?

A

-Classical (95% cases)
-Nodular

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54
Q

What are the 4 subclasses of classical Hodgkin’s?

A

-Nodular sclerosis
-Mixed cellularity
-Lymphocyte-rich
-Lymphocyte-depleted

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55
Q

What is the most common symptom of Hodgkin’s (HL)?

A

Painless, rubbery enlarged lymph nodes, often in cervical/supraclavicular region

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56
Q

What is a classical textbook symptom of HL but not often seen?

A

Alcohol-induced pain at nodes

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57
Q

What are some symptoms of HL?

A

-B symptoms (night sweats, weight loss etc)
-Chest discomfort
-Abdo discomfort
-Pruritis-itching
-Fatigue

58
Q

Name 5 differential diagnoses for HL

A

-Infectious mononucleosis
-Non-H lymphoma
-AIDS
-TB
-Leukaemia
-Myeloma

59
Q

What are the key investigations for HL?

A

-Lymph node biopsy
-Chest x-ray
-Contrast enhanced CT
-PET-CT

60
Q

What is the gold standard for staging in classical HL?

A

PET-CT

61
Q

How is HL managed?

A

-Up to date on vaccinations
-Combination chemo w/ radiotherapy-strength + quantity depends on staging

62
Q

How is relapsed HL normally treated?

A

-High dose chemo w/
-Autologous stem cell transplant

63
Q

What complications are associated with HL chemo?

A

-Infection
-Cognitive impairment
-Secondary cancers
-Infertility

64
Q

What complications are associated with HL radiotherapy?

A

-Tissue fibrosis
-Secondary cancers
-Infertility

65
Q

What is important to remember about blood products + HL patients/patients who have been treated for HL in past?

A

Can only receive irradiated blood products-for life

66
Q

What is leukaemia?

A

Cancer of a line of stem cells in the bone marrow causing proliferation of of ‘blasts’

67
Q

What is pancytopenia?

A

-Low RBC, WBC + platelets due to underproduction

68
Q

What are myelodysplastic syndromes?

A

Syndrome where bone marrow fails to make adequate healthy blood cells + abnormal cells crowd out remaining ones

69
Q

What are the 2 characteristics of AML?

A

-Immature myeloid cell proliferation >20% blasts
-Bone marrow failure

70
Q

What is AML?

A

Uncontrolled growth of myeloid cell line in bone marrow

71
Q

What are the 4 types of leukaemia?

A

-Acute myeloid leukaemia
-Acute lymphoblastic leukaemia
-Chronic myeloid leukaemia
-Chronic lymphocytic leukaemia

72
Q

What age group do most leukaemias affect?

A

60+

73
Q

Which leukaemia most commonly affects children + is associated with Down’s?

A

ALL

74
Q

Name some differential diagnoses for AML

A

-B12/folate deficiency
-Infection
-Meds
-Autoimmune
-Liver disease

75
Q

How would you investigate suspected AML?

A

-History
-FBC
-Blood film
-Haematinics (B12, folate, ferritin etc)

76
Q

What are the risk factors fir AML?

A

-Most de novo malignancy BUT
-Congenital e.g. Down’s, Bloom
-Environmental exposure-prior chemo, radiation, smoking etc
-Pre-existing MDS, MPD, aplastic anaemia

77
Q

How does leukaemia present?

A

-Non-specific:
-fatigue
-Fever
-Pallor
-Petechiae
-Abnormal bleeding
-Failure to thrive

78
Q

Investigations for leukaemia?

A

-FBC
-Blood film
-Bone marrow biopsy
-CT/PET
-Genetic testing
-Lactate dehydrogenase (LDH)

79
Q

How is a bone marrow biopsy used in the diagnosis of leukaemia?

A

Used for staging-% of blasts

80
Q

What analysis is performed on bone marrow samples?

A

-Immunophenotyping
-Cytogenetics-chromosome study
-FISH-fluorescence in situ hybridisation
-Molecular analyses

81
Q

What is a characteristic finding on blood film with AML?

A

Auer rods

82
Q

What are the 2 classification systems for AML?

A

*WHO classification
-ICC classification

83
Q

How is AML treated?

A

Chemo:
-Induction
-Consolidation
-Maintenance

84
Q

What 3 factors affect prognosis with AML?

A

-Type of AML
-Age at diagnosis
-Presence of comorbidities

85
Q

What are the most commonly used chemo drugs in AML?

A

-Cytarabine
-Daunorubicin
-ATRA

86
Q

What is tumour lysis syndrome?

A

Syndrome caused by chemicals released when cells are destroyed in chemo

87
Q

What are the symptoms of tumour lysis syndrome?

A

-High uric acid
-High potassium
-High phosphate
-Low calcium

88
Q

What are the side effects of chemo?

A

-Cytopenias
-Bystander organ damage
-Hair loss
-Reduced fertility
-N+V
-Fatigue
-Loss of appetite

89
Q

Name 4 supportive therapies for AML

A

-Blood cell transfusions
-Analgesia
-Antimicrobial prophylaxis
-Anti-emetics

90
Q

What is anaemia?

A

Low conc of haemoglobin in blood
-<115 in women
-<130 in men

91
Q

What is MCV?

A

Mean cell volume-size of RBC

92
Q

What is a normal MCV?

A

80-100 femtolitres

93
Q

What are the 3 MCV categories of anaemia?

A

-Microcytic (low MCV,<80)
-Normocytic (normal MCV, 80-100)
-Macrocytic (large, >100)

94
Q

What is a normal haemoglobin level for women?

A

110-147g/l

95
Q

What is a normal haemoglobin level for men?

A

131-166g/l

96
Q

What are the causes of microcytic anaemia + pneumonic?

A

TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

97
Q

What are the causes of normocytic anaemia?

A

-Chronic disease
-Renal disease
-Acute bleeding

98
Q

What are the causes of megaloblastic macrocytic anaemia?

A

-B12 deficiency
-Folate deficiency
-Bone marrow disorders
-Haemolysis

99
Q

What is reticulcytosis?

A

Increased conc of reticulocytes-immature RBC-rapid turnover of RBC due to e.g. blood loss

100
Q

What are the causes of normoblastic macrocytic anaemia?

A

-Alcohol
-Reticulocytosis
-Hypothyroidism
-Liver disease
-Drugs

101
Q

Name 2 specific symptoms of iron deficiency anaemia

A

-Pica-abnormal food cravings
-Hair loss

102
Q

Name 6 generic anaemia symptoms

A

-Fatigue
-SOB
-Dizziness
-Palpitations
-Headaches

103
Q

How much iron do you need in a normal diet?

A

15mg/day

104
Q

What should you assume about the cause of iron deficiency until proven otherwise?

A

Assume blood loss e.g. p/r, heavy periods, GI

105
Q

What happens to iron lvls in pregnancy?

A

Foetus takes iron, levels drop-lots need supplements

106
Q

What conditions impair iron absorption?

A

-Coeliac disease
-Gastrectomy

107
Q

What are some specific signs of iron deficiency anaemia?

A

-Koilonychia-spoon-shaped nails
-Angular cheilitis-red, swollen corners of mouth
-Atrophic glossitis-smooth tongue
-Brittle hair + nails

108
Q

What kind of anaemia can jaundice indicate?

A

Haemolytic

109
Q

How is anaemia managed?

A

-Treat any source of blood loss
-Replace iron-ferrous sulphate 20mg 1-3 daily

110
Q

What blood tests could you do for anaemia?

A

-FBC
-Reticulocyte
-Blood film
-Ferritin
-B12 + folate
-LFTs

111
Q

What is a normal level of folate?

A

> 3.9ug/l

112
Q

Where is folate absorbed?

A

Proximal jejunum

113
Q

What are the main causes of folate deficiency?

A

-Poor nutrition
-Coeliac
-Crohns
-Pregnancy
-Haemolysis

114
Q

Name some sources of B12

A

-Meat
-Fish
-Eggs
-Dairy

115
Q

Describe the absorption of B12

A

Absorbed in terminal ileum by binding to intrinsic factor

116
Q

What is B12 needed for?

A

-DNA synthesis
-Fatty acid synthesis

117
Q

What are some causes of B12 deficiency?

A

-Pernicious anaemia
-Gastrectomy
-Vegan diet
-Oral contraceptives
-Nitric oxide

118
Q

What is pernicious anaemia?

A

Autoimmune gastric atrophy + loss of intrinsic factor production

119
Q

Name 4 types of anaemia

A

-Iron deficiency
-Pernicious
-Haemolytic
-Sickle cell

120
Q

What is haemolysis?

A

Increased RBC destruction

121
Q

What investigations would you do for suspected haemolysis?

A

-Blood film
-Reticulocyte count
-Bilirubin
-Lactate dehydrogenase
-Direct antiglobulin test-Coombs

122
Q

What are some causes of haemolysis?

A

-RBC disorders
-Sickle cell
-Haemolytic anaemias
-Prosthetic heart valves
-Autoimmune haemolytic anaemias

123
Q

Which should be check + replaced first, B12 or folate + why?

A

B12, high folate risks neuro damage

124
Q

What are the main 3 causes of B12 deficiency?

A

-Pernicious anaemia
-Low dietary B12
-Meds e.g. PPI, metformin

125
Q

What is the inheritance pattern for sickle cell?

A

Autosomal recessive

126
Q

What is HbS?

A

Sickle cell

127
Q

How does sickle cell cause ischaemia?

A

-HbS polymerises when deoxygenated
-Blocks blood vessels=ischaemia, sequestration

128
Q

How is sickle cell diagnosed?

A

-Sickle solubility test
-Hb separation

129
Q

What are some of the complications associated with sickle cell?

A

-Acute painful crisis
-Stroke
-Sequestration (blood pools in liver/spleen)
-Acute anaemia

130
Q

What does someone with only one abnormal copy of haemoglobin gene have?

A

Sickle-cell trait-carrier, normally asymptomatic

131
Q

What things can trigger sickle cell crises?

A

-Dehydration
-Infection
-Stress
-Cold weather

132
Q

How is sickle cell disease treated (general)?

A

-Supportively:
-Treat infections
-Keep warm
-Vaccinations
-Good hydration
-Antibiotic prophylaxis

133
Q

How is sickle cell disease treated (medically/surgically)?

A

-Hydroxycarbamide to increase HbF
-Crizanlizumab-monoclonal antibody
-Blood transfusions
-Cure=bone marrow transplant

134
Q

What is sickle cell disease?

A

Abnormal mutation in Beta globin gene-causing abnormal haemoglobin production

135
Q

What is thalassaemia?

A

Autosomal recessive condition caused by genetic defect in protein chains making up haemoglobin-causes anaemia

136
Q

What are the different types of thalassaemia?

A

-Alpha thalassaemia
-Beta thalassaemia-splits into minor + intermedia

137
Q

How does thalassaemia cause haemolytic anaemia?

A

-RBC more fragile, break down easily

138
Q

How does thalassaemia cause splenomegaly?

A

More old, destroyed RBC in thalassaemia-they are collected by the spleen

139
Q

What are the typical presenting features of thalassaemia?

A

-Microcytic anaemia
-Fatigue
-Pallor
-Jaundice
-Gallstones
-Poor growth/development
-Splenomegaly

140
Q

What investigations would you do for thalassaemia?

A

-Ferritin
-Mean cell volume
-Haemoglobin electrophoresis

141
Q

Describing screening for thalassaemia

A

-Antenatal routinely offered
-New UK arrivals
-Pre-surgery
-Guthrie test-day 5 heel prick, neonates

142
Q
A

Phase 2a (8 decks)

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