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RADT317 - Brain > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

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1
Q

What does myeloproliferative mean?

A

Rare disorders of the bone marrow that causes an increase in the number of blood cells

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2
Q

What does myelo mean?

A

Bone marrow

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3
Q

What does proliferative mean?

A

Grow or reproduce quickly

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4
Q

Why are there different types of myeloproliferative disorders?

A

Due to different types of blood cells

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5
Q

What is polycythemia vera-?

A

Increased concentration of red blood cells in blood

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6
Q

What is apparent polycythemia vera-?

A

Normal red cell count but lowered plasma, making blood “thicker”. Mainly caused by lifestyle- overweight, smoking, drinking alcohol and certain medicines including diuretics – more concentrated

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7
Q

What is relative polycythemia vera-?

A

Similar to apparent but caused by dehydration

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8
Q

What is absolute polycythemia vera-?

A

over production of red cells-

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9
Q

What percentage of polycythemia vera- cases have a genetic link to the JAK2 gene?

A

Approx 95% of cases have a genetic link to JAK2 gene- leading to uncontrolled reproduction of RBC. JAK2 fault develops during life- not passed on to offspring > producing more = not as good quality of cell

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10
Q

Symptoms of polycythemia vera-?

A

Fatigue
Trouble breathing
Itchy skin/ reddening of skin
Head ache and vision problems
High blood pressure
Blockage of blood vessels/ blood clots- heart attack/strokes/ DVT
Bleeding gums, peptic ulcers
Splenomegaly (75% off patients at presentation) – enlarged spleen

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11
Q

Risk factors of polycythemia vera-?

A

More common in males
Smoking
Hypoxia
CO exposure)
Approx. 2/100,00 diagnoses each year- rare
Ave age at diagnosis 60+, uncommon in under 40
Exposure to radiation

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12
Q

Diagnosis of polycythemia vera-?

A

FBC
Bone marrow biopsy
Major criteria- Hb of more than 18.5g/dl men or 16.5g/dl women

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13
Q

Treatment of polycythemia vera-?

A

Currently not curable

Management is based on symptom control:
Watch and wait if asymptomatic
Lifestyle changes
Lowering of blood count- venesection, usually ½ litre of blood taken once a week initially, then on individual need
Low dose aspirin
Control of cardio vascular risk factors
Chemotherapy- Hydroxycarbamine, mild chemo drug used to prevent RBC production ( possible increased risk of AML with long term use)
Interferon- used if associated high platelet count.

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14
Q

What is essential thrombocythaemia?

A

Overproduction of platelets

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15
Q

Essential thrombocythaemia genetic cause associated with?

A

JAK2 gene (60% of cases)
CALR gene (30% of cases)
MPL gene (5% of cases)

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16
Q

JAK2 and MPL gene fault affect what?

A

Response of bone marrow to growth factors causing over production

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17
Q

CALR gene is responsible for?

A

Producing calreticulin protein, the function of which is thought to involve folding of new proteins and calcium regulation, although exact function is not known.

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18
Q

Signs and symptoms of essential thrombocythaemia?

A

Heart attack/stroke
Headaches, dizziness, migraines, seizures
Burning/ throbbing pain in hands and feet
Bruising/ bleeding
Pain and redness in hands/feet/face, Erythromelalgia
GI bleeding/ blood in urine
Thrombosis- Stroke/heart attack/blurred vision or blindness
Splenomegaly (40-50% at presentation)

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19
Q

Risk factors of essential thrombocythaemia?

A

60 years +, although 20% of those diagnosed under 40 years, rare in children.

Possible environmental link- exposure to chemicals or electrical wiring

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20
Q

What is primary myelofibrosis (myeloscelerosis)?

A

Over production of collagen or fibrous tissue in the bone marrow reducing its ability to produce blood cells

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21
Q

Primary myelofibrosis (myeloscelerosis) risk factors

A

Similar male: female ratio
Ages 60-70+
Environment- exposure to petrochemicals, benzene and toluene; radiation exposure
Previous diagnosis of a myeloproliferative neoplasm

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22
Q

Hodgkins histology

A

Classical, nodular

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23
Q

Non-hodgkins histology

A

Most common are follicular and Diffuse large B-cell lymphoma (Both types of B-Cell lymphoma) - DLBCL
Low or high grade

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24
Q

Hodgkins characterised by?

A

Contiguous spread
Adjacent nodal groups

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25
Q

Non-Hodgkins characterised by?

A

Non-contiguous spread
Random lymph node involvement

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26
Q

Who demonstrated multinucleate giant cells?

A

Sternberg (1898) and Reed (1902)

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27
Q

What is CD15?

A

75-85% of CHD cases
A carbohydrate used in diagnosis of HL
Present on; neutrophils, eosinophils and some monocytes
Not present on basophils or lymphocytes
Present on Reed-Sternberg cells
Mediates phagocytosis and chemotaxis

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28
Q

What is CD30?

A

seen in almost all CHD cases
lymphocyte activation antigen

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29
Q

What are the 5 Hodgkins categories?

A

Nodular sclerosing, mixed cellularity, lymphocyte depleted, lymphocyte rich and nodular lymphocyte predominant

30
Q

Hodgkins aetiology links?

A

EBV (Epstein Barr virus), HIV, rheumatoid arthritis, genetics, smoking

31
Q

Hodgkins signs and symptoms

A

Persisten fever, night sweats, weight loss, cough, itching,

32
Q

Diagnosis Hodgkins

A

First line treatment antibiotics to rule out infection
Physical examination
Medical and family history
FBC
Lymph node biopsy
Bone marrow biopsy

33
Q

Hodgkins stage 1

A

Only a single lymph node site or extra nodal site is involved

34
Q

Hodgkins stage 2

A

Two or more lymph node sites on one side of the diaphragm are involved or limited contiguous extra nodal site involvement

35
Q

Hodgkins stage 3

A

Lymph node sites of both sides of the diaphragm are involved with splenic or limited contiguous extranodal site involvement

36
Q

Hodgkins stage 4

A

Extensive involvement of extra nodal sites with or without lymph node involvement

37
Q

Staging Hodgkins

A

A = asymptomatic
B = symptoms other than lymphadenopathy present
E = extra nodal disease
S = spleen involvement
X = bulky disease >10cm or mediastinal mass of 1/3 of chest diameter

38
Q

Very favourable prognosis

A

Clinical stage 1
Age < 40yrs
Stage A
ESR <50 (blood chemistry)
Female
Mediastinal: thoracic diameter ratio <0.35

39
Q

Favourable prognosis

A

All other patients not in very favourable or unfavourable

40
Q

Unfavourable prognosis

A

Age >50 yrs
Stage A and ESR >50
Stage B and ESR > 30
Clinical stage 2 with 4 sites of disease
Mediastinal: thoracic diameter ratio > 0.3

41
Q

Non-hodgkins aetiology

A

Weakened immune system, autoimmune condition, EBV, Hepatitis C, Human T-cell lymphotropic virus, previous chemo or RT, psoriasis

42
Q

NHL Signs and symptoms

A

Very similar to Hodgkins

43
Q

What 2 classification types is non-hodgkins divided into?

A

B-cell and T-cell

44
Q

B-cell lymphoma

A

90%
Diffuse
Follicular
Mantle
Mucosa
Burkitts

45
Q

T-cell lymphoma

A

10%
Anaplastic
Peripheral
Mycosis fungoides

46
Q

Non-hodgkins prognosis

A

Overall UK NHL 5year survival is approx. 68%. 10 year survival rate is 63%.

47
Q

Early stage - stage 1 (one lymph node group) or 2 (2 or more lymph node groups but not past diaphragm) , no B-symptoms) HL management

A

Short course chemo (ABVD or ChiVPP) and RT

48
Q

What was the German H10 study?

A

2 cycles ABVD + 20gy 10#
Indicates chemo + RT gives best chance of cure (chemo then IFRT - involved field RT)
Long term follow up, UK RAPID study

49
Q

IFRT

A

Involved field - mantle/inverted Y with extended FSD

50
Q

ISRT

A

Involved site - smaller CTV based on pre-chemo image

51
Q

INRT

A

Involved nodes - even smaller CTV based on PET

52
Q

EFRT

A

Extended field

53
Q

RT + Chemo eval (Early HL management)

A

Know where going to develop, not massive fields
Plan RT using pre-chemo images - PET - not ones that show response response (may be microscopic disease remaining, localised spread, is systemic disease)

54
Q

Importance of PET/CT (Early HL management)

A

Deep nodes unpalatable

55
Q

Deauville criteria

A

FDG uptake, used for PET imaging, shows the degree of malignancy of an area or mass in the body
The degree of uptake can be altered due to various factors including treatment related inflammation
FDG uptake can show a positive result but when compared with original, pre-treatment uptake, the actual findings can be very different
Due to these and other factors a criteria to be used to evaluate disease response was developed for lymphomas
The Deauville criteria and Deauville scale, take in to account initial findings at diagnosis and possible treatment reaction

56
Q

When is CTV increased? (Early HL management)

A

If used as primary treatment with no systemic treatment given, the CTV is increased due to increased likelihood of microscopic disease.

57
Q

RT dose and fractionation early stage HL?

A

Early stage disease and favourable characteristics- recommendation of 20Gy 10#
Early stage disease with unfavourable characteristics- 30Gy 15#

58
Q

Intermediate stage - Stage 1 or 2 (still same side of diaphragm) but with risk factors management

A

Chemo (4 cycles ABVD) and RT (IFRT - 30.6Gy 17#)

59
Q

What do National Comprehensive Cancer Network (NCCN) recommend?

A

if a Complete remission (CR) achieved after 4 cycles of ABVD then IF-RT alone, or 2 additional cycles of ABVD followed by IF-RT are commenced (patient-led consideration)

if a Partial Remission (PR) or stable disease occurs then 2 additional cycles (no choice – will have additional 2 cycles) are commenced followed by IF-RT and restaging:
no further treatment is necessary if they are responding to IFRT
patients with residual disease on PET scan and biopsy are managed as progressive disease

60
Q

Advanced stage HL (3-4) management

A

Usually chemotherapy alone- not localised disease anymore – invaded both sides of diaphragm > usually no RT given due to the large area

However, Combined with RT if large residual mass (only case)

Chemo (4 cycles ABVD or BEACOPP)

Consolidative RT

61
Q

Progressive and relapsed HL management

A

Chemo+ Brentuximab (monoclonal antibody), High dose therapy and stem cell transplant (TBI), RT alone

62
Q

HL Late effects

A

Secondary cancers - solid tumours are most common, most develop more than 10 years after treatment, annual chest imaging

63
Q

NHL prognosis - indolent forms

A

Good prognosis - median survival up to 20 years but largely incurable in advanced stages (weighing up treatment vs no treatment depending of individual patient characteristics).

64
Q

NHL prognosis - aggressive forms

A

Shorter natural history - curative in 50% of cases

65
Q

What is a MAB?

A

Monoclonal antibody

66
Q

Why are MABs used?

A

They take advantage of tumour-related targets, using; cell surface molecules, soluble effectors and cell signalling machinery. The ‘bare’ antibody is covalently linked to a cytotoxic compound. These home in on target antigens to deliver the cytotoxic compound directly.

67
Q

What is CD20?

A

Transmembrane protein that serves as a calcium channel

Overexpressed on 90% of b-cell NHL

68
Q

MAB therapy

A

Rituximab

69
Q

Other monoclonal antibodies

A

Brentuximab vedotin (CD30 +ve HL, relapse)
Nivolumab (Relapse classical HL, following Auto SCT and Brentiximab)
Ibritumomab

70
Q
A

RADT317 - Brain (10 decks)

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