Haematological Malignancies Flashcards

1
Q

Haematological Cancers
- Five Groups

A

Groups of Blood Cancers

  1. Leukaemia
  2. Lymphoma
  3. Myeloma
  4. Myelodysplastic syndromes
  5. Myeloproliferative neoplasms
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2
Q

Hemopoiesis
- Myeloid line cells

A

Myeloid line cells

  1. Thrombocytes
    - Megakaryocytes
  2. Erythrocytes
    - Reticulocytes
  3. Mast Cells
  4. Myeloblastic cells
    - Basophils
    - Neutrophils
    - Eosinophils
  • Monocytes
  • Macrophages
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3
Q

Haemopoiesis
- Lymphoid cell line

A

Lymphoid progenitor line

  1. Small lymphocytes
    - B Cells
    - Plasma cells
    - T Cells
  2. Natural Killer
    (Large granular lymphocyte)
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4
Q

Blood pathologies

  • Myeloid
  • Lymphoid
A

Blood pathologies

  • Myeloid
    1. AML
    2. Chronic myeloproliferative neoplasms
  • Lymphoid
    3. ALL
    4. CLL
    5. Lymphoma
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5
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
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6
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
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7
Q

Haematology Malignancy
- Imaging

A

Haem malignancy imaging

  1. CT
  2. Skeletal survey

(3. PET
- Lymphoma/myeloma

  1. MRI spine/pelvis
    - myeloma)
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8
Q

Leukaemia
- Classifications

A

Leukaemias

  1. Myeloid/lymphoid
  2. Acute/chronic
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9
Q

ALL

  • Pres
  • Mx
A

ALL

  • Pres
    0. Young (male)
    1. Pancytopenia symptoms
    2 Bone pain
    3. Lymphadenopathy
  • Mx
    1. Multi-drug chemo
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10
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
  1. APML
    - Tretinoin and rubicin
    - Tretinoin and arsenic trioxide
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10
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
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11
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
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12
Q

Essential thrombocythaemia

  • Presentation
  • Mx
A

ET

  • Presentation
    1. Thrombosis
  • Art/ven
    2. Haemorrhage
    3. Splenomegaly
    4. Transformation
  • Myelofibrosis
  • AML

Mx
1. Cytoreduction
- Hydroxycarbamide

  1. Anti platelet
  2. Anti coagulation
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13
Q

Polycythaemia vera

  • Pathology
  • Presentation
A

PV

  • Pathology

1.JAK2 mutation
2. Low EPO, high RBCs

  • Presentation
  1. Thrombosis
  2. Viscosity
    - Headaches
    - Drowsiness
    - Transient visual
  3. Skin
    - Pruritus
    - Plethoric complexion
  4. Transformation
    - Leukaemia
    - Myelofibrosis (10-20%
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14
Q

Myelofibrosis
- Pathology

A

Myelofibrosis pathology

  1. JAK2 mutation
  2. Clonal disorder
  3. Abnormal cells
  4. Cytokine release, causing fibrosis
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15
Q

Myelofibrosis

  • Presentation
  • Mx
A

Myelofibrosis

  • Presentation
    1. Pancytopenia
    2. Splenomegaly
    3. Transformation (Leukaemia)
  • Mx
    1. Supportive
  1. Cytoreductive
  2. JAK2 inhibitors
  3. Splenectomy
  4. BMT
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16
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
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17
Q

Lymphoma Types
- Four Types

A

Lymphoma types

  1. Hodgkin Lymphoma
  2. Non-Hodgkin B Cell
    - Low grade
  3. Non Hodgkin B Cell
    - High grade
  4. Non-hodgkin T Cell
    - High grade
    - Possibly low grade
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18
Q

Lymphoma
- Classification methods

A

Lymphoma classification

  1. Pathology
    - Histology
    - Morphology
    eg - Follicular
    • Mantle cell
    • Diffuse
  2. Immunophenotypic
    - CD
    - Proteins eg. Cyclin/ALK
  3. Cytogenic
    - eg. t(11;14)
    - Karyotyping
    - FISH
  4. Site
    eg. MALT
    eg. Splenic marginal/Hepatosplenic T cell
    eg. Cutaneous
  5. Causative factors
    - Enteropathy-type T cell
    - Post-transplant
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19
Q

Myeloma

  • Pathology
A

Myeloma

  1. Bone marrow malignancy
  2. Clonal Plasma Cells
    - Monoclonal Igs (nonfunctioning)
  3. Bone marrow infiltration
    - Anaemia
    - Thrombocytopenia
  4. Bone disease + renal disease
  5. Solid tumours
    - Plasmacytomas
  6. Immunopathy
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20
Q

Myeloma
- Presentation

A

Myeloma
- Presentation

CRAB

C - Hypercalcaemia

R - Renal impairment

A - Marrow infiltration
- Anaemia/thrombocytopaenia/neutropaenia

B - Bones
- Osteopenia
- Path fractures

  1. Infections
  2. Hyperviscosity
    - Headache/transient visual
  3. Neuropathy
  4. Bleeding diathes
  5. Associations
    - Amyloid/cryoglobulinaemia
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21
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)

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22
Q

Myeloma Ix
- Bloods

A

Myeloma Bloods

  1. Ig
    - Poly or Mono clonal
  2. Paraprotein
    - Monoclonal “M-protein”
  3. SFLC
    - Light chains
    - Kappa vs lambda
22
Q

Myeloma Ix
- Bloods

A

Myeloma Bloods

  1. Ig
    - Poly or Mono clonal
  2. Paraprotein
    - Monoclonal “M-protein”
  3. SFLC
    - Light chains
    - Kappa vs lambda
    - Ratio if mono, quantity if polyclonal
23
Q

Myeloma
- Asymptomatic

A

Smouldering Myeloma

  1. Absence of myeloma defining events
  2. Paraprotein/M-Protein >30g/L
  3. Clonal BM plasma cells 10-60%
24
Q

MGUS
- Definition

A

Monoclonal Gammopathy of Undetermined Significance

  1. Serum paraprotein <30g/L
  2. Clonal bone marrow plasma cells <10%
  3. Absence of CRAB or amyloidosis
25
Q

Amyloidosis
- Definition

A

Amyloidosis definition

  1. Generic term
  2. Fibril deposition
    - Beta-pleated sheet
    - In extracellular tissue
26
Q

Amyloidosis (AL)

  • Associations
A

Amyloidosis associations

-Alone

+Plasma cell dyscrasias
1. Multiple myeloma
2. Waldenstrom macroglobulinaemia

27
Q

Blood transfusion
- Red Cells efficacy

A

Packed Red Cells

  1. 1 unit
  2. Raise Hb by 10
  3. In a 70kg man
28
Q

Blood transfusion
- Platelet dosing

A

Platelet dosing

  1. 1 adult dose
  2. 10^11 platelets
29
Q

Irradiated blood components

  1. Indications
  2. Process
A

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
30
Q

CMV Neg blood
- Indications

A

CMV Negative blood
- Indications

  1. Intrauterine
  2. Neonates <28 days post EDD
  3. Elective pregnancy transfusion
31
Q

Tunnelled Central Line

  • Practitioners
  • Incisions
A

Tunnelled central line

  • Practitioners
    1. Radiologist/surgeon
    2. GA/Sedation
  • Incisions
    1. Jugular vein
  • Advance line into SVC
    2. Chest wall
  • access point
32
Q

PICC Line

  • Route
A

Peripherally inserted central catheter

  1. Arm vein
    - Right basilic
    - Cephalic
    - Brachial/medial cubital
  2. Axillary
  3. Subclavian
  4. SVC/RA
33
Q

Implantable port
- Use

A

Implantable port
“Portacath/SC Port”

  • Use
    1. Huber needle inserts into port
  • Central or peripheral
    2. Chemo/venepuncture/transfusion
    3. Fluids/ABx
34
Q

Tumour lysis syndrome
- Pathophysiology
- Associations

A

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
35
Q

TLS
- Classification

A

TLS Classification

  • Laboratory
    1. 2+ metabolic abnormalities
  • Urate +25%
  • Phosphate/potassium +25%
  • Calcium -25%
  • Clinical
    2. Lab TLS +
    + AKI
    + Arrhythmia
    + Seizure
    + Sudden death
36
Q

TLS
- Management

A

TLS Mx

  1. Risk-adapted approach
  2. Allopurinol
    - Blocks UA production
    - (xanthine oxidase i)
  3. Rasburicase
    (urate oxidase)
37
Q

Haematology
- Six preventions

A

Haematology preventions

  1. Mouth care
  2. Prophylactic antimicrobials
  3. Contraception against pregnancy
  4. Against infertility
  5. Neutropenic sepsis vigilance
  6. Coagulopathy
38
Q

Haematology
- Infection Prevention

A

Haematology

  1. Mouth care
    - Chlorhexidine mouthwash qds
  2. Prophylaxis
    - Fluconazole/isavuconazole/posaconazole
    - Co-trimoxazole (PCP)
    - Aciclovir
    - ABx
  3. Neutropenic Sepsis
    - Neutropenic sepsis protocol
  4. Tazobactam (mero if allergic)
  5. Gentamicin if shocked
  6. Vancomycin for gram positive cover
39
Q

Haematology management

  • Pregnancy
  • Fertility
A

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)
40
Q

Neutropenic sepsis
- Criteria

A

Neutropenic sepsis criteria

  1. Fever
    - 38.0
    - 37.5 < 1 hr
  2. Neutropenia
    - <0.5
    - 1.0 and falling
  3. A febrile infection
41
Q

Neutropenic sepsis abx

  1. First line UHL dose
  2. Pen allergic
  3. Hypotension/shock
  4. Severe mucositis/line infection
A

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)
42
Q

Neutropenic sepsis
- Non ABx mx

A

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
43
Q

Haematology
- Coagulopathy mx

A

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

44
Q

DOAC Reversal

  1. Dabigatran
  2. Rivaroxaban, apixaban, edoxaban
A

DOAC Reversal

  • Dabigatran
    1. Idarucizumab (Praxbind)
  • Dedicated, flushed IV line
  • Monitor in fructose intolerance
  • Rivaroxaban, apixaban, edoxaban
    1. Prothrombin complex concentrate
  • (Octaplex, beriplex)
45
Q

Monoclonal gammopathies
- Common factor

A

Monoclonal gammopathies

  1. Monoclonal protein
    - Serum
    - Urine
  2. Monoclonal plasma cells
    - Bone marrow
    - Soft tissue (plasmacytomas)
    - Peripheral circulation (advanced)
46
Q

Plasma cells

  1. Division
  2. Role
  3. Abnormalities
A

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
47
Q

Immunoglobulins
- Heavy chains vs light chains

  1. Domains
  2. Classes
  3. Bonding
A

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)
48
Q

Monoclonal proteins

  1. Detection
  2. Quantitation
  3. Immunohistochemistry
  4. Bone marrow aspirate
A

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
49
Q

MGUS

-Epidemiology
-Pathology

A

MGUS

  • Epidemiology
    1. 1-2% of adults
    2. Increased age
    3. Male sex

Monoclonal gammopathy of undetermined significance

50
Q

Monoclonal proteins
- Diseases
- Predisposing conditions

A

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
51
Q

Monoclonal gammopathies

  • 8 emergencies
A

Monoclonal gammopathies
- Emergencies

  1. AKI
  2. Hypercalcaemia
  3. Bone fracture
  4. Compressive myelopathy
  5. Hyperviscosity
  6. Bleeding
  7. VTE
  8. Infection
52
Q

Monoclonal gammopathies
- Emergencies

  1. AKI
  2. Hypercalcaemia
  3. Fractures
A

Monoclonal gammopathies
- Emergencies

  1. AKI
    - 20% of multiple myeloma
    - Light chain cast nephropathy
  2. Hypercalcaemia
    - Osteoclast activity
  3. Fractures
    - Lytic lesions
    - Myeloma cells
53
Q

Monoclonal gammopathies
- Emergencies

  1. Hyperviscosity
    - Sx & Tx
  2. Bleeding
    - DDx
  3. VTE
    - Prevalence
A

Monoclonal gammopathies
- Emergencies

  1. Hyperviscosity
    - Headache/visual symptoms/ mental status change
    - Tx: Plasmapheresis
  2. Bleeding
    - Thrombocytopenia from myeloma
    - Acquired von Willebrand’s disease
  3. VTE
    - 4-20% of myeloma patients
54
Q

Monoclonal gammopathies
- Infection susceptibility
- Microbes

A

Monoclonal gammopathies
- Infections

  1. Suppression of normal immunoglobulin
  2. Encapsulated bacteria
55
Q

Monoclonal gammopathies
- Dx

A

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