Haematological Malignancies Flashcards
(55 cards)
1
Q
Haematological Cancers
- Five Groups
A
Groups of Blood Cancers
- Leukaemia
- Lymphoma
- Myeloma
- Myelodysplastic syndromes
- Myeloproliferative neoplasms
2
Q
Blood pathologies
- Myeloid
- Lymphoid
A
Blood pathologies
- Myeloid
1. AML
2. Chronic myeloproliferative neoplasms - Lymphoid
3. ALL
4. CLL
5. Lymphoma
3
Q
Haematological symptoms
- Marrow failure
- Disease
- Constitutional
A
Haematological symptoms
- Marrow failure
1. Anaemia
2. Thrombocytopenia
3. Neutropaenia - Disease
1. Lumps
2. Organomegaly - Constitutional
1. Weight loss
2. Night sweats, fevers, pruritus
4
Q
Haematological malignancy
- Routine Bloods
- Special Bloods
A
Haematological malignancy
- Routine Bloods
1. FBC, UE, LFT, CRP, Ca
2. Haematinics, retics, film - Special Bloods
1. LDH, urate, B2M (Beta 2 microglob) PV (plasma visco)
2. Ig +/- SFLC (serum free light chain)
3. Peri. bl. immunophenotyping
5
Q
Haematology Malignancy
- Imaging
A
Haem malignancy imaging
- CT
- Skeletal survey
(3. PET
- Lymphoma/myeloma
- MRI spine/pelvis
- myeloma)
6
Q
Leukaemia
- Classifications
A
Leukaemias
- Myeloid/lymphoid
- Acute/chronic
7
Q
ALL
- Pres
- Mx
A
ALL
- Pres
0. Young (male)
1. Pancytopenia symptoms
2 Bone pain
3. Lymphadenopathy - Mx
1. Multi-drug chemo
8
Q
AML
- Pres
- Mx
(APML)
A
AML
- Pres
0. Older (males)
1. Pancytopenia - Mx
1. Intensive vs non-intensive - Cytarabine + -rubicin
- Cytarabine or monotherapy
2. Allogeneic stem cell transplant
- APML
- Tretinoin and rubicin
- Tretinoin and arsenic trioxide
8
Q
CML
- Pres
- Mx
A
CML
- Pres
0. Philadelphia 9:22
1. High WCC
2. Splenomegaly
3. Can progress to AML - Mx
1. Tyrosine kinase inhibitors - Imatinib
2. Remission off-medication
9
Q
CLL
- Pres
- Mx
A
CLL
- Pres
1. Mature B cells
2. Nodal and splenic
3. Dysregulation - AIHA (AI Haemolytic)
- ITP
- Mx
1. Chemo
2. Targeted immune treatment - Ibrutinib
10
Q
Essential thrombocythaemia
- Presentation
- Mx
A
ET
- Presentation
1. Thrombosis - Art/ven
2. Haemorrhage
3. Splenomegaly
4. Transformation - Myelofibrosis
- AML
Mx
1. Cytoreduction
- Hydroxycarbamide
- Anti platelet
- Anti coagulation
11
Q
Polycythaemia vera
- Pathology
- Presentation
A
PV
- Pathology
1.JAK2 mutation
2. Low EPO, high RBCs
- Presentation
- Thrombosis
- Viscosity
- Headaches
- Drowsiness
- Transient visual - Skin
- Pruritus
- Plethoric complexion - Transformation
- Leukaemia
- Myelofibrosis (10-20%
12
Q
Myelofibrosis
- Pathology
A
Myelofibrosis pathology
- JAK2 mutation
- Clonal disorder
- Abnormal cells
- Cytokine release, causing fibrosis
13
Q
Myelofibrosis
- Presentation
- Mx
A
Myelofibrosis
- Presentation
1. Pancytopenia
2. Splenomegaly
3. Transformation (Leukaemia) - Mx
1. Supportive
- Cytoreductive
- JAK2 inhibitors
- Splenectomy
- BMT
14
Q
Myelodysplasia
- Pathology
- Diagnosis
A
Myelodysplasia
- Pathology
1. Clones of dysplastic cells
2. Spectrum with AML
3. Multiple cytopenias - Diagnosis
1. Diagnosis of exclusion
2. Bone marrow classification
15
Q
Lymphoma Types
- Four Types
A
Lymphoma types
- Hodgkin Lymphoma
- Non-Hodgkin B Cell
- Low grade - Non Hodgkin B Cell
- High grade - Non-hodgkin T Cell
- High grade
- Possibly low grade
16
Q
Lymphoma
- Classification methods
A
Lymphoma classification
- Pathology
- Histology
- Morphology
eg - Follicular- Mantle cell
- Diffuse
- Immunophenotypic
- CD
- Proteins eg. Cyclin/ALK - Cytogenic
- eg. t(11;14)
- Karyotyping
- FISH - Site
eg. MALT
eg. Splenic marginal/Hepatosplenic T cell
eg. Cutaneous - Causative factors
- Enteropathy-type T cell
- Post-transplant
17
Q
Myeloma
- Pathology
A
Myeloma
- Bone marrow malignancy
- Clonal Plasma Cells
- Monoclonal Igs (nonfunctioning) - Bone marrow infiltration
- Anaemia
- Thrombocytopenia - Bone disease + renal disease
- Solid tumours
- Plasmacytomas - Immunopathy
18
Q
Myeloma
- Presentation
A
Myeloma
- Presentation
CRAB
C - Hypercalcaemia
R - Renal impairment
A - Marrow infiltration
- Anaemia/thrombocytopaenia/neutropaenia
B - Bones
- Osteopenia
- Path fractures
- Infections
- Hyperviscosity
- Headache/transient visual - Neuropathy
- Bleeding diathes
- Associations
- Amyloid/cryoglobulinaemia
19
Q
Myeloma
- SLiM CRAB
1. Defining Events
2. End organ failure
A
Myeloma
S - ixty % BM Plasmacytosis
Li - ght chain ratio >100
M -RI >1 focal lesion of 5mm
C -alcium elevation (>2.75)
R - enal impairment (CR>177)
A - naemia (Hb<100)
B- one lesion (1+ on XR/CT/PET)
20
Q
Myeloma Ix
- Bloods
A
Myeloma Bloods
- Ig
- Poly or Mono clonal - Paraprotein
- Monoclonal “M-protein” - SFLC
- Light chains
- Kappa vs lambda
20
Q
Myeloma Ix
- Bloods
A
Myeloma Bloods
- Ig
- Poly or Mono clonal - Paraprotein
- Monoclonal “M-protein” - SFLC
- Light chains
- Kappa vs lambda
- Ratio if mono, quantity if polyclonal
21
Q
Myeloma
- Asymptomatic
A
Smouldering Myeloma
- Absence of myeloma defining events
- Paraprotein/M-Protein >30g/L
- Clonal BM plasma cells 10-60%
22
Q
MGUS
- Definition
A
Monoclonal Gammopathy of Undetermined Significance
- Serum paraprotein <30g/L
- Clonal bone marrow plasma cells <10%
- Absence of CRAB or amyloidosis
23
Amyloidosis
- Definition
Amyloidosis definition
1. Generic term
2. Fibril deposition
- Beta-pleated sheet
- In extracellular tissue
24
Amyloidosis (AL)
- Associations
Amyloidosis associations
-Alone
+Plasma cell dyscrasias
1. Multiple myeloma
2. Waldenstrom macroglobulinaemia
25
Blood transfusion
- Red Cells efficacy
Packed Red Cells
1. 1 unit
2. Raise Hb by 10
3. In a 70kg man
26
Blood transfusion
- Platelet dosing
Platelet dosing
1. 1 adult dose
2. 10^11 platelets
27
Irradiated blood components
1. Indications
2. Process
Irradiated Blood
- Indications
1. BMT
2. Hodgkin's
3. Alemtuzumab
4. T Cell immunodeficiency
5. HLA matched
6. Intra-uterine
- Method
1. Gamma or X-rays
2. Prevents donor white cells replicating
3. Avoids TA-GvH-D
- Transfusion associated graft vs host
28
CMV Neg blood
- Indications
CMV Negative blood
- Indications
1. Intrauterine
2. Neonates <28 days post EDD
3. Elective pregnancy transfusion
29
Tunnelled Central Line
- Practitioners
- Incisions
Tunnelled central line
- Practitioners
1. Radiologist/surgeon
2. GA/Sedation
- Incisions
1. Jugular vein
- Advance line into SVC
2. Chest wall
- access point
30
PICC Line
- Route
Peripherally inserted central catheter
1. Arm vein
- Right basilic
- Cephalic
- Brachial/medial cubital
2. Axillary
3. Subclavian
4. SVC/RA
31
Implantable port
- Use
Implantable port
"Portacath/SC Port"
- Use
1. Huber needle inserts into port
- Central or peripheral
2. Chemo/venepuncture/transfusion
3. Fluids/ABx
32
Tumour lysis syndrome
- Pathophysiology
- Associations
TLS
- Pathophysiology
1. Rapid breakdown of cells
2. Intracellular content in blood
3. AKI, Arrhythmias, seizures, NM dysfunction
- Associations
1. Proliferative, bulky haematological malignancies
- High-grade B-cell lymphoid
- eg. ALL, Burkitt's lymphoma
2. Chemosensitive malignancies
- Including solid tumours
33
TLS
- Classification
TLS Classification
- Laboratory
1. 2+ metabolic abnormalities
- Urate +25%
- Phosphate/potassium +25%
- Calcium -25%
- Clinical
2. Lab TLS +
+ AKI
+ Arrhythmia
+ Seizure
+ Sudden death
34
TLS
- Management
TLS Mx
1. Risk-adapted approach
2. Allopurinol
- Blocks UA production
- (xanthine oxidase i)
3. Rasburicase
(urate oxidase)
35
Haematology
- Six preventions
Haematology preventions
1. Mouth care
2. Prophylactic antimicrobials
3. Contraception against pregnancy
4. Against infertility
5. Neutropenic sepsis vigilance
6. Coagulopathy
36
Haematology
- Infection Prevention
Haematology
1. Mouth care
- Chlorhexidine mouthwash qds
2. Prophylaxis
- Fluconazole/isavuconazole/posaconazole
- Co-trimoxazole (PCP)
- Aciclovir
- ABx
3. Neutropenic Sepsis
- Neutropenic sepsis protocol
1. Tazobactam (mero if allergic)
2. Gentamicin if shocked
3. Vancomycin for gram positive cover
37
Haematology management
- Pregnancy
- Fertility
Haematology: pregnancy/fertility mx
1. Contraception (on chemo)
- Norethisterone
- Avoid COCP if thrombotic
- Barrier protection
2. Fertility
- Minimise chemo toxicity eg. ABVD in Hodgkins
- Risk informed
- Fertility preservation (egg/sperm storage)
38
Neutropenic sepsis
- Criteria
Neutropenic sepsis criteria
1. Fever
- 38.0
- 37.5 < 1 hr
2. Neutropenia
- <0.5
- 1.0 and falling
3. A febrile infection
39
Neutropenic sepsis abx
1. First line UHL dose
2. Pen allergic
3. Hypotension/shock
4. Severe mucositis/line infection
Neutropenic sepsis abx
- First line UHL dose
1. Tazocin, 4.5g tds
- Pen allergic
2. Meropenem
- Hypotension/shock
3. Gentamicin, 7mg/kg
- Severe mucositis/line infection
4. Vancomycin (teicoplanin/linezolid)
40
Neutropenic sepsis
- Non ABx mx
Neutropenic sepsis non-abx mx
1. G-CSF
- Granulocyte-colony stimulating factor
2. SC injection
3. Reduce severity and duration
4. ADRs
- Bone pain
- Headache
- Fatigue and nausea
41
Haematology
- Coagulopathy mx
Haematological coagulopathy mx
- DOACs
1 Apixaban
2 Dabigatran (heparin at initiation + reversible)
- Not used in low eGFR
- Longer pre-op suspension
3 Edoxaban (heparin at initiation)
4 Rivaroxaban
42
DOAC Reversal
1. Dabigatran
2. Rivaroxaban, apixaban, edoxaban
DOAC Reversal
- Dabigatran
1. Idarucizumab (Praxbind)
- Dedicated, flushed IV line
- Monitor in fructose intolerance
- Rivaroxaban, apixaban, edoxaban
1. Prothrombin complex concentrate
- (Octaplex, beriplex)
43
Monoclonal gammopathies
- Common factor
Monoclonal gammopathies
1. Monoclonal protein
- Serum
- Urine
2. Monoclonal plasma cells
- Bone marrow
- Soft tissue (plasmacytomas)
- Peripheral circulation (advanced)
44
Plasma cells
1. Division
2. Role
3. Abnormalities
Plasma cells
- Division
1. Terminal B-cell
- Role
1. Humoral immunity
- Primary mediator
2. Immunglobulin secretion
- antigen-specific
- Abnormalities
1. Autoimmune disease
2. Plasma cell neoplasms
45
Immunoglobulins
- Heavy chains vs light chains
1. Domains
2. Classes
3. Bonding
Immunoglobulins
- Domains
1. HC - 4-5 domains
2. LC - 2 domains
- Classes
1. HC - alpha, gamma, delta, epsilon, mu
2. LC - kappa, lambda
- Bonding
1. Covalently bonded (disulphide)
46
Monoclonal proteins
1. Detection
2. Quantitation
3. Immunohistochemistry
4. Bone marrow aspirate
Monoclonal proteins
- Detection
1. Urine protein electrophoresis
- Quantitation
1. Nephelometry
- Immunohistochemistry
1. CD138 (Syndecan)
2. Kappa-lambda ratio
- Bone marrow aspirate
1. Sensitive flow cytometry
2. Immunophenotypic characterisation
47
MGUS
-Epidemiology
-Pathology
MGUS
- Epidemiology
1. 1-2% of adults
2. Increased age
3. Male sex
Monoclonal gammopathy of undetermined significance
48
Monoclonal proteins
- Diseases
- Predisposing conditions
Monoclonal proteins
- Diseases
1. MGUS
- Monoclonal gammopathy of undetermined significance
2. Lymphoproliferative disease
- B Cell Lineages
- CLL, Non-Hodgkins
- Post-transplant MGs
- Multiple myeloma
3. Predisposing conditions
- Hep C infection
- HIV infection
4. Infections/inflammatory developments
- SLE/RA/PA
- Sjogren, Schnitzler
49
Monoclonal gammopathies
- 8 emergencies
Monoclonal gammopathies
- Emergencies
1. AKI
2. Hypercalcaemia
3. Bone fracture
—
4. Compressive myelopathy
—
5. Hyperviscosity
6. Bleeding
7. VTE
—
8. Infection
50
Monoclonal gammopathies
- Emergencies
1. AKI
2. Hypercalcaemia
3. Fractures
Monoclonal gammopathies
- Emergencies
1. AKI
- 20% of multiple myeloma
- Light chain cast nephropathy
2. Hypercalcaemia
- Osteoclast activity
3. Fractures
- Lytic lesions
- Myeloma cells
51
Monoclonal gammopathies
- Emergencies
5. Hyperviscosity
- Sx & Tx
6. Bleeding
- DDx
7. VTE
- Prevalence
Monoclonal gammopathies
- Emergencies
5. Hyperviscosity
- Headache/visual symptoms/ mental status change
- Tx: Plasmapheresis
6. Bleeding
- Thrombocytopenia from myeloma
- Acquired von Willebrand's disease
7. VTE
- 4-20% of myeloma patients
52
Monoclonal gammopathies
- Infection susceptibility
- Microbes
Monoclonal gammopathies
- Infections
1. Suppression of normal immunoglobulin
2. Encapsulated bacteria
53
Monoclonal gammopathies
- Dx
Monoclonal gammopathies
- Ix
1. SPEP
- Serum protein electrophoresis
2. UPEP
- Urine protein electrophoresis
- 24hr urine exam
3. sFLC
- Serum free light chain assay
4. M protein level
5. Bloods
- FBC
- Calcium
- Creatinine
- LDH
- Bone marrow FISH
