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frcr2 > haematology > Flashcards

haematology Flashcards

1
Q

What is the pathology of myeloma, vs plasmacytoma

What are the complications

A

Myeloma - Plasma cell neoplasm, secreting monoclonal protein, typically IgG or IgA, and rarely IgD
Plasmacytoma - localised proliferation of plasma cells, either arising from bone or extra medullary

Pathological fractures
Myelosuppression
Hyper-Ca, renal failure, amyloid deposition

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2
Q

What is the diagnostic criteria for MGUS vs axis myeloma vs sx myeloma

A

MGUS - serum M protein <30g/L, plasma cells in bone marrow <10%, asx

Asx myeloma - serum M protein >30g/L, plasma cells in bone marrow ≥10%, asx

Sx myeloma - serum M protein >30g/L, plasma cells in bone marrow ≥10%, sx of CRAB
C - Ca > 2.75
R - renal impairment Cr >173
A - anaemia Hb <10
B - bone lesions or compression fracture

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3
Q

How is myeloma staged

A

I - III

I - b2-microglobulin <3.5 and albumin >35
II - neither I nor III
III - b2-microglobulin >5.5

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4
Q

What is the first line treatment for myeloma

A

If <70 & fit - Velcade (bortezomid)-based ChT (with thalmidomide/dex or cyclophosphamide/dex) & stem cell transplant

If >70 or less fit - weekly chemotherapy (CTDa / MPT (melphalen-pred-thal) / VCD (weekly)

RT for bone disease - pain, impending fracture or post-fracture, MSCC - 8Gy/1# or 20Gy/5

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5
Q

What are the side effects of thalidomide

A

Somnolence, neuropathy, constipation, teratogenic risk

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6
Q

What is the diagnostic criteria for a plasmacytoma

What is the progression rate from plasmacytoma to myeloma

A

histologically confirmed single lesion, commonly vertebrae, ribs, sternum
and with normal bone marrow biopsy (<10% plasma cells)
and serum paraprotein <20g/L
Normal bloods - Ca, Hb, renal function
Normal skeletal survey

50% of bone will progress in 2-4yrs, 30% of soft tissue plasmacytoma

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7
Q

What are the investigations for myeloma / plasmacytoma

A

MRI whole body or PET
MRI whole spine

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8
Q

What is the management of a bone plasmacytoma

A

Radical RT - 45Gy/25#, but high rate of progression to MM
surgery - usually if structural instability of bone

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9
Q

What is the RT regimen for a bone plasmacytoma

A

CTV = whole vertebra, and any soft tissue extension with a 1.5cm margin
CTV-PTV margin - further 0.5-1cm

If adjuvant RT - CTV is whole surgical field

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10
Q

Where is the commonest site for an extra medullary plasmacytoma

How are they treated

A

90% in head and neck

Radical RT - 45Gy/25#
Or surgery, with adjuvant RT if incomplete resection

CTV = GTV +1.5-2cm
CTV-PTV margin 0.5-1cm

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11
Q

What are the two types of Hodgkin lymphoma
What are the markers for these
What cell marker is indicative of HL

A

Classical - 95% - CD15 & CD30+, CD20 & CD45-
Nodular lymphocyte predominant - 5% - CD15 & CD30-, CD20 & CD45+

Reed-Sternberg cells

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12
Q

How does HL present
How is it investigated

A

Painless LN enlargement
B symptoms

Excision biopsy of lymph node
ESR - prognostic
Bloods incl viral hepatitis and HIV, b2-microglobin, LDH, albumin
Staging imaging - CTCAP and PET

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13
Q

How is Hodgkin lymphoma staged

A

Stage 1 - Single LN region or Single Extranodal site (I-E)

Stage 2 - ≥ 2 LN regions on same side of diaphragm
Or Single Extranodal site + regional LNs +/- contiguous LN regions on same side of diaphragm (II-E)

Stage 3 - Nodes both sides of diaphragm (includes spleen as LN)

Stage 4 - Non-contiguous extranodal involvement

E (stage 1&2 only) - extranodal contiguous extension
A - no B symptoms
B - B symptoms

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14
Q

What is the management of an early stage nodular lymphocyte predominant HL?

A

Management according to stage:
1A or 2A with ≤2 sites - RT only, 30Gy/15#

Stage 2A otherwise - ABVD chemotherapy then RT 20Gy/10#

If bulky disease (>10cm) - 4x ABVD followed by RT 30Gy/15#

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15
Q

What is the management of an advanced stage nodular lymphocyte predominant HL?

A

Stage 3 - 4: 6x R-ABVD, or R-CHOP

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16
Q

How is the management of classical HL divided

A

According to stage:
1-2A, split also according to favourable or unfavourable features
2B-4

Where 2B = ≥2LNs sites on same side of diaphragm but with B-symptoms

Unfavourable features for an early HL - BEEN:
- Bulky
- Extranodal areas
- ESR >50
- ≥3 LN areas

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17
Q

What is the management of an early HL (stage 1-2A) with favourable criteria

A

2x ABVD + ISRT 20Gy/10#
or 3x ADVD can be considered if PET negative at this point
Consider 4x ABVD chemo only and no RT, if female and axillary/mediastinal mass, due to breast irradiation risk

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18
Q

What is the management of an early HL (stage 1-2A) with unfavourable criteria (BEEN)

A

4x ADVD followed by 30Gy/10# ISRT
or 6x ABVD only

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19
Q

What are the components of ABVD chemotherapy

A

Doxorubicin, bleomycin, vinblastine, dacarbazine

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20
Q

What is the management of an advanced HL (stage 2B and above)

A

3x ABVD chemotherapy, then PET
If negative, give further 4x AVD only

If positive, 4x escalated BEACOPP +/- RT

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21
Q

What are the volumes for involved site radiotherapy

A

CTV - define superior and inferior extent of original disease on pre-chemo imaging
Expanded cranio-caudally by 1.5cm and axially to include involved nodal space
Edit off natural barriers to spread: air, muscle, bone

PTV
H&N 0.3 – 0.5cm
Mediastinum - 1cm transversely and 1.5cm cranio-caudally
All other sites 1cm

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22
Q

What breast screening is done after RT for Hodgkins lymphoma

A

Screening offered to all women given mediastinal RT <35 years
Start screening 8 years post RT or by age 30, whichever comes later

Annual MRI if <30 years/dense breasts

Annual mammogram age 30-50
Back to 3 yearly mammogram >50 years

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23
Q

What translocation is associated with an anaplastic large-cell lymphoma

A

t (2;5) - Anaplastic large-cell lymphoma

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24
Q

What translocation is associated with Burkitts lymphoma & what oncogene

A

t (8;14) or t (2:8) - Burkitt’s lymphoma - C-myc

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25
Q

What translocation is associated withDLBCL & what oncogene

A

t (3,14) - DLBCL - BCL6

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26
Q

What translocation is associated with Mantle cell lymphoma & what oncogene

A

t (11;14) - Mantle cell lymphoma - cyclin D1

27
Q

What translocation is associated with MALT lymphoma

A

t (11;18) - MALT lymphoma

28
Q

What translocation is associated with follicular lymphoma & what oncogene

A

t (14;18) - Follicular lymphoma - BCL2 (in 80-90%)

29
Q

What translocation is associated with CML

A

t (9:22) - CML- Abl - massive splenomegaly

30
Q

What proportion of DLBCL have bone marrow involvement
And what genetic change should be tested for, and how

A

10%

MYC rearrangement via FISH

If detected -> further testing for BCL2 and BCL6 rearrangements (prognostic markers)

If all three positive - poor prognosis

31
Q

When is PET indicated in lymphoma staging

A

DLBCL, follicular and Burkitts

32
Q

What factors influence the prognosis of DLBCL?

A

‘APLES’
Age > 60 years
PS >2
LDH
>1 Extra-nodal site
Stage III or IV

33
Q

How is the management of DLBCL categorised

A

Stage 1A
Stage 1b-4, with max 1 extra-nodal site
Stage 4 with ≥2 extranodal sites
Bulky disease

34
Q

What is the management of a stage 1A DLBCL

A

R-CHOP x3 + ISRT 30Gy/15#
or R-CHOP x6

35
Q

What is the management of a stage 1B-4 DLBCL, with max 1 extranodal site

A

R-CHOP x6

36
Q

What is the management of a stage 1B-4 DLBCL, with ≥2 extranodal sites

A

R-CHOP x6 + CNS prophylaxis with intrathecal or high dose methotrexate

37
Q

What is the management of bulky DLBCL

A

R-CHOP x6 + ISRT 30Gy/15#

38
Q

When is CNS prophylaxis with IT/high dose methotrexate indicated

A

Testis, Breast, Adrenal gland or Kidney
Raised LDH and 2+ Extra nodal sites

Or 4 / 5 IPI risk factors:
Age>60, PS ≥2, LDH raised, >1 Extranodal site, Stage 3/4

39
Q

When is consolidation RT indicated for DLBCL

A

Stage 1A disease (if 3x R-CHOP only given)
Bulky disease
In elderly pts unable to have chemo
High risk cases (e.g. testes, breast)

40
Q

How is involved site RT done for DLBCL

A

GTV planned according to pre-tx volume
CTV - GTV grown sup/inf by 1.5cm and axillary to include nodal level
*Parotid, Breast, Spleen, Stomach & Liver - CTV is whole organ

PTV
H&N: + 3-5mm
Mediastinum: +1cm transversely & +1.5cm Sup/Inf
All other sites: +1cm

41
Q

What forms the majority of primary CNS lymphoma

What imaging and investigation is required

A

90% DLBCL
20% also have intra-ocular component - need ophthalmic assessment

Imaging:
USS testes, CTCAP - exclude primary
MRI head

Ix:
Bloods incl viral hepatitis & HIV
Biopsy
LP

42
Q

What is the treatment for primary CNS lymphoma

A

<60 &fit - 4x matrix chemotherapy and stem cell transplant or WBRT (equivalent outcomes)

WBRT dose:
Partial response to chemo - 36Gy/20# +/- 9Gy/5# boost to 45Gy/25#
Complete response - 23.4Gy/13#

> 60 or not fit
R-MP chemotherapy (ritual, metho, procarbazine)

43
Q

What is the commonest histology for primary testicular lymphoma

What is the treatment

A

80-90% are DLBCL, with propensity for CNS spread
30% rate of relapse within 2yrs

Tx:
Orchidectomy
6x R-CHOP + CNS prophylaxis with high dose / IT methotrexate
RT to contralateral testis - 30Gy/15# - typically treated with 9MeV electrons

44
Q

How is a primary mediastinal B cell lymphoma treated

A

6x R-CHOP + 30Gy/15# to primary

45
Q

How is a Burkett’s lymphoma treated

A

Rituximab-based chemotherapy with CNS prophylaxis
High relapse rate

46
Q

What is the classification of peripheral T-cell lymphoma

How are they generally treated

A

Peripheral
Primary cutaneous
Leukaemia

Tx: R-CHOP followed by stem cell transplant

47
Q

How is an EBV NK/T-cell associated lymphoma treated

A

If early stage, I-II - treat with early RT
Otherwise, neo-adjuvant GELOX chemo, followed by RT and further GELOX chemo
RT: 50Gy/25# to GTV +1-2cm

48
Q

What is Sezary syndrome

A

Sezary syndrome = bone marrow involvement: presence of erythroderma, LN and Sezary cells in peripheral blood

Mycosis fungicides with sezary syndrome is the commonest form of cutaneous T-cell lymphoma

49
Q

How is MF/sezary syndrome treated

A

Observe until symptomatic
Skin-directed treatment - topical steroids, psoralen or UV therapy
Palliative RT to symptomatic lesions - 8Gy/2# with 0.5-1cm margin, treating to a depth of 7-9mm

Total skin electron beam therapy
30Gy/15#, treating 4-5x/week
6MeV with angled fields with shielding of eyes and nails

50
Q

Where may be under-dosed with total skin electron therapy

A

Areas that may be under-dosed: top of scalp, soles, palms, perineum, infra-mammary areas. These regions may require additional treatment

51
Q

what are the side effects with total skin electron therapy

A

Acute: erythema, dry desquamation, hair and nail loss, reduced sweating
Late: secondary skin cancers, long term skin changes: hyperpigmentation & telangiectasia

52
Q

How is a follicular lymphoma treated

A

If asx and low tumour burden - observe

Stage 1A-2A with contiguous nodes - radical curative ISRT 24Gy/12#, but not many present at this stage

> stage 2A - R-CVP or R-CHOP, followed by maintenance rituximab

Must re-biopsy recurrence as risk of transformation to high grade DLBCL

53
Q

How is a gastric marginal B cell lymphoma (MALT) treated, after failure of H. pylori eradication or extra-nodal elsewhere

A

RT - 24Gy/12#

54
Q

What is the treatment for a mantle cell lymphoma

A

Tend to present disseminated

Fit pts - NORDIC regime - R-CHOP + cytarabine, followed by stem cell transplant

Less fit pts - R-CHOP followed by maintenance rituximab

2nd line ibrutinib

Mantle cell lymphoma is very radiosensitive so RT can be used for local disease or palliation

55
Q

How is an orbital lymphoma treated

A

Stage 1 - RT only, 30Gy/15# (24Gy/12# if follicular lymphoma)
Stage >1 - chemotherapy followed by RT

If posterior orbit involved, give intrathecal methotrexate

RT given to whole orbit, as wedge pair with corneal shielding - angled back to avoid contralateral lens, or as half beam block

56
Q

How is a conjunctival lymphoma treated

A

RT - 24Gy in 12#
CTV - Whole conjunctiva
Typically with low energy electrons and bolus

57
Q

How can RT be used to treat Waldenstroms macroglobulinaemia

A

TBI before stem cell transplant
14.4Gy/8#, treating twice a day

58
Q

What are the side effects of TBI, and how are they classified

A

Acute
Skin erythema
Dry sore throat
Fatigue
Nausea & decreased appetite
Diarrhoea
Mucositis and swelling of parotids
Acute bone marrow suppression

Late
Second malignancy
Infertility
Hypothyroid
Pulmonary fibrosis
Cataracts

59
Q

What are the adverse prognostic risk factors for NHL (IPI prognostic score)

A

A - age >60
P - PS >2
L - elevated LDH
E - >1 extra nodal site
S - stage III or IV

60
Q

What CD markers are positive for a primary mediastinal B cell lymphoma
What is the standard of care

A

CD30 & CD 79A
6x R-CHOP & ISRT 30Gy/15#

61
Q

When is R-CHOP-14 used

A

Primary mediastinal B cell lymphoma

62
Q

When is R-CHOP-21 used

A

CD20+ve Diffuse Large B-Cell Non-Hodgkin’s Lymphoma
Patients with symptomatic Stage III or IV follicular lymphoma

63
Q

What prognostic score is used for follicular lymphoma
What does it consist of

A

FLIPI score - HALLS
H - Hb<12
A - Age >60
L - LDH >ULN
L - ≥5 LN sites
S - stage 3-4

64
Q
A

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