Lymphoma Flashcards

1
Q

What are the types of lymphoma?

A

Hodgkin - characterised by presence of Hodgkin/Reed Sternberg cells
Non-Hodgkin - more than 60 subtypes, B cell lymphomas most common

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2
Q

What are the types of Hodgkin lymphoma?

A

Classical - nodular sclerosis, mixed cellularity, lymphocyte rich and lymphocyte depleted

Nodular lymphocyte predominant

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3
Q

What are Reed-Sternberg cells?

A

HRS cells
Large multinucleate malignant cells
Described as having an owl like appearance
Hodgkin cells - mononuclear variant of these

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4
Q

What are the clinical features of HL?

A

Lymphadenopathy - painless, firm, enlarged nodes
Most commonly around neck
B symptoms - fever, night sweats, weight loss
Mediastinal mass - may be incidental finding on CXR, or present with SOB, cough, pain, SVCO
Pruritus
Hepatosplenomegaly
Malaise
Fatigue

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5
Q

What investigations are required for a diagnosis of HL?

A

Excision biopsy - FNA or core biopsy, of affected lymph nodes

Bloods - FBC, UE, LFT
Bone profile, LDH, uric acid
ESR, BBV, HTLV-1

Imaging
PET/CT for staging
CXR, CT, MRI if suspected CNS involvement
LP, CSF analysis
ECHO
PFTs
Bone marrow biopsy
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6
Q

What is Lugano staging?

A

System for classifying HL

Limited - Stage I and II
I - one node or group of adjacent nodes
II - two or more nodal groups on same side of diaphragm

Stage II BULKY
II with bulky disease

Advanced - III and IV
III - on both side of diaphragm, nodes above diaphragm with spleen involvement
IV - additional non-contiguous extra lymphatic involvement

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7
Q

What is ‘bulky disease’?

A

Typically refers to disease >10cm

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8
Q

What is the management of cHL?

A

Chemo and radio

ABVD common chemo regimen:
Doxorubicin (A)
Bleomycin (B)
Vinblastine (V)
Dacarbazine (D)
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9
Q

What is the function of doxorubicin?

A

Inhibits topoisomerase II

leads to inhibition of DNA and RNA synthesis

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10
Q

What are some of the side effects of the ABVD regimen?

A

A - cardiomyopathy, myelosuppression, skin reactions
B - pulmonary fibrosis, idiosyncratic reaction - hypotension, confusion, fever, wheeze
V - peripheral neuropathy, bladder atony
D - bone marrow suppression, hepatic necrosis

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11
Q

What treatment is given in early stage of HL?

A

Stage I/II - ABVD chemo
3-4 cycles

Advanced stage III/IV
ABVD
BEACOPP
Stanford V regimen

Radiotherapy may be used as an adjunct

For relapse -
Salvage chemotherapy
Radiotherapy
Autologous haematopoietic cell transplantation

Must receive irradiated blood for life for transfusion - reduce risk of graft versus host disease

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12
Q

What is the presentation of NHL?

A

Lymphadenopathy
Fever, WL, night sweats
Pruritus
Splenomegaly, hepatomegaly

High grade lots of features
Low grade more indolent, gradual lymphadenopathy

Each subtype has own pattern of clinical features
Dermatological, SVCO etc

Primary CNS lymphoma - neurological features, headache, confusion etc

Primary cutaneous lymphoma - rashes, plaques, ulcers

Primary GI tract lymphoma - abdo pain, nausea, obstruction

ONCOLOGICAL EMERGENCIES

SVCO
Cord compression
Hypercalcaemia
TLS
Neutropenic sepsis
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13
Q

What are the investigations for NHL?

A

Biopsy - excision preferred, FNA not adequate

Bloods
FBC, UE, LFT, bone profile
Uric acid, LDH, ESR, BBV

Imaging
CXR, CT chest abdo pelvis
PETCT, MRI, testicular USS
Bone scan

Bone marrow aspirate
Lumbar puncture
FISH

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14
Q

What is the staging of NHL?

A

Lugano staging

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15
Q

What is R-CHOP?

A

Regimen of chemo for NHL

Rituximab 
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
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16
Q

What are the side effects of rituximab?

A
Monoclonal antibody
Activity against CD20
Infusion reactions
Hep B reactivation
Mucocutaneous reactions
Leucoencephalopathy
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17
Q

What are the side effects of cyclophosphamide?

A
Alkylating agent
Carcinogenic tendencies
Linked to development of TCC of the bladder
Bone marrow suppression
Infertility
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18
Q

What is diffuse large B cell lymphoma?

A

Most common form of NHL
Slightly more common in women

Presents with rapidly enlarging mass
Commonly in the neck, abdomen or mediastinum
B symptoms, extranodal

Treatment is complex
Assessment for CNS
CNS prophylaxis

Limited stage - chemoimmunotherapy with R-CHOP
Advanced stage - R-CHOP
Autologous cell transplants

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19
Q

What is follicular lymphoma and its presentation?

A

B cell NHL
Second most common NHL

Insidious presentation
Gradual lymphadenopathy
Painless

85% have translocation on chromosomes 14-18

20
Q

What is the treatment for follicular lymphoma?

A

Depends on Stage
Early can have radiotherapy

Cure not common in those with stage II, III and IV
Relapses frequently
Immunotherapy, chemo

21
Q

What is Burkitt’s lymphoma?

A

High grade
Rapidly proliferating
B cell NHL
Commonly affects kids

Endemic - EBV, follows distribution of malaria

Sporadic - Europe, USA

Immunodeficiency - associated with AIDS and other conditions

22
Q

What causes Burkitt’s lymphoma?

A

c-myc proto oncogene mutation

Due to a translocation between 8 and 14

23
Q

What is the presentation of Burkitt’s?

A

Endemic - rapidly enlarging tumour in the jaw of a child
Enlarged nodes in neck, abdominal masses

Sporadic - abdominal symptoms, ileocaecal valve affected, bowel obstruction

Fever, weight loss, night sweats present in all

24
Q

What is the management of Burkitt’s lymphoma?

A

Immunochemotherapy options

TLS prophylaxis is key

25
Q

When does incidence of Hodgkins Lymphoma peak?

A

20-30

Smaller peak in old age

26
Q

Describe the features of a Reed Sternberg Cell

A
Multi-nucleated
Giant
Malignant
B-Cell Derivatives
On a background of a variety of cells
27
Q

What is a contiguous disease?

A

Only spreads to adjacent nodes and structures due to the flow of lymph

28
Q

Describe Nodular Sclerosing Hodgkins Lymphoma

A

lacunar cells

young women

29
Q

Describe mixed cellularity Hodgkins Lymphoma

A

+++Reed-Sternberg
lymphocytes, eosinophils, neutrophils, and histiocytes
no fibrotic bands
elderly

30
Q

Describe lymphocyte rich Hodgkins Lymphoma

A

small lymphocytes

good prognosis

31
Q

Describe lymphocyte depleted Hodgkins Lymphoma

A

associated with HIV
popcorn cells
worst prognosis

32
Q

What are popcorn cells?

A

a variant of Reed Sternberg cells also seen in Nodular Lymphocyte Predominant HL

Of B cell lineage and express CD20

Also known as L&H hodgkin cells

33
Q

What is Nodular Lymphocyte Predominant Hodgkins Lymphoma?

A

A non classical HL which has popcorn cells rather than Reed Sternberg cells

Has a different management

34
Q

What is average 5 year survival for Hodgkins Lymphoma?

A

81%

35
Q

What are poor prognostic indicators for Hodgkins Lymphoma?

A
Male
>45
leucocytosis
lymphocytopaenia
low Hb
low albumin
36
Q

What are the sub-divisions of Non-Hodgkin’s lymphoma?

A

B Cell
T Cell
NK Cell

37
Q

What are the subdivisions of B Cell Non-Hodgkin’s lymphomas?

A

High Grade

Low Grade

38
Q

Give examples of high grade B Cell Non-Hodgkin’s lymphomas

A

Diffuse Large B Cell Lymphoma (DLBL)

Burkitt’s Lymphoma

39
Q

Give examples of low grade B Cell Non-Hodgkin’s lymphoma

A

Follicular lymphoma

Chronic Lymphocytic Leukaemia

Mantle Cell Lymphoma

40
Q

Identify risk factors for Non-Hodgkin’s lymphoma

A
Age - generally over 50
Prolonged immunosuppression: diabetes, steroids, HIV
EBV
H. Pylori
Chlamydia
autoimmune diseases: SLE, sjrogrens, RA
41
Q

Apart from by cell type, how else can Non-Hodgkin’s lymphoma be classified?

A

By clinical behaviour:

Indolent - low grade
Aggressive - high grade

42
Q

What is the prognosis of low grade non-hodgkins lymphoma?

A

incurable but compatible with a number of years survival

43
Q

What is the prognosis of high grade non-hodgkins lymphoma?

A

rapidly fatal if untreated but modern treatment can cure in majority of cases

44
Q

How do low grade non-hodgkins lymphomas present?

A

1 or more areas of painless lymphadenopathy
Hepatosplenomegaly
Bone marrow involvement (cytopaenia)

45
Q

How are high grade non-hodgkins lymphomas treated?

What else is needed in the management of them?

A

R-CHOP

CNS prophylaxis - intrathecal methotrexate

Prevent tumour lysis syndrome: Rasburicase
Prevent herpes: aciclovir
Prevent pneumocystis: trimoxazole

46
Q

Why must you be wary of low grade non-hodgkins lymphomas?

A

They can transform to high grade