Lymphomas Flashcards

1
Q

What is a lymphoma?
What are common sites of presentation of lymphomas

A

Lymphoma = neoplasm of lymphoid cells

Usually found in
1. Lymphatic system (lymph nodes, BM or blood)
2. Lymhpoid organy: Spleen or gut associated lymphoid tissue
3. Skin (often T-cell lymphomas)

  1. Rarely: anywher else in the body
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2
Q

What is the incidence of lymphomas in the UK?

Are hodgkin or non-hodgkin’s lymphomas more common?

A

Around 200/1.000.000 new diagnosis per year

Non-Hodgkin’s 80%
Hodgekins 20%

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3
Q

What is usually the first step of classifying Lymphomas?

A

Classification into
1. Hodgkin lymphoma (20%)
2. Non-Hodgkin lymphomas (80%)

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4
Q

What are the characterisics of Hodgekin Lymphomas?

A

Historical + histological difference to NHL

Malignant Lymphoma usually of B-cell origin, with the presence of Reed-Sternbeg cells (picture below) or the presence of Hodgekin cells

Clinical: single/ localised in one group of lymph nodes

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5
Q

What is the epidemiology of Hodgekin Lymphmas?

A

Bimodal age disribution
1st peak: 25-30 years
2nd peak: 50-70 years (EBV association)

In paediatrics: male dominance

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6
Q

How is Hodgekin’s lymphoma sub-classified?

A

Classified into
1. Classical HL (95%) –> focus of future flashcards
2. nodular lymphocyte predominant HL (5%) (similar presentation to B cell -NHL)

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7
Q

A paient presents with the following cell on lymph node biopsy: What is the diagnosis?

A

Cell: Reed- Sternberg cell –> Large binucleaed or polylobaed cells with large nucleoli (malignant B-cells)

–> owel eye appearance

Pathonognomic for Hodgekin lymphomas

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8
Q

What is a Hodgkin cell? What is its significance in the diagnosis of Hodgekin lymphomas?

A

Large mononuclear cells with large nucleoli of B-cell origin

Can be present in HL, but pathonognomic cell for diagnoss and more commonly present is Reed-sernber cell

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9
Q

What are risk factors associaed with the devleopment of Hodgekin Lymphomas?

A
  1. Strong association with Epstein-Barr virus (EBV)
  2. Immunodeficiency: HIV, transplant, immunosuppression
  3. Autoimmune diseases (e.g., rheumatoid arthritis, sarcoidosis)
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10
Q

What is the clinical presentation of Hodgekin lymphoma?

A
  1. B-symptoms
  2. Painless lymphadenopathy (if in mediastinum: might only be cough) –> usually single-group of lymph nodes above the mediastinum
  3. Pruritus
  4. Lymph node pain associated with alcohol (rare but v.specific)
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11
Q

How is Hodgekin lymphoma diagnosed (what investigations + respective findings) ?

A

Mainly history + Lymph node biopsy
1. Bloods
FBC: can be normal, high or low WCC, anaemia, eosinophilia
LDH: increased
calcium: can be increased if paraneoplastic syndrome

  1. Biopsy
    Reed-Sternberg cells (don’t express normal B-cell markers (CD20 and 45 but do expressed CD 15)
    Potential inflammation + granuloma formation
  2. Imaging: disease spread (CT/ PET)
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12
Q

How is Hodgekin Lymphoma staged?

A

Staged by using Ann Arbor system with reference to the diaphragm

+ A for no constitutional symptoms
+ B for constitutional symptoms

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13
Q

What is the prognosis of Hodgekin Lymphoma?

A

Generally good (80-90% 5 year survival), especially in young

–> Curative approach of treatment, regardless of stage

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14
Q

What is the approach to treatment of Hodgekin Lymphoma?

A

Stage 1+2: Combination Chemo + Radiotherapy (very high risk of breast cancer in female)
Stage 3-4: Chemotherapy with radiotherapy in selected cases

If fails or in remission: Stem-cell transplnat (usually autologous)

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15
Q

What is a typical chemotherapy regime used in the treatment of Hodgekin’s lymphoma?

A

ABVD

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

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16
Q

What are the 2 main categories in which non-Hodgekin Lymphomas are sub-classified?

Which one is more common?

A

Non-Hodgekin Lymphomas then classfied into
1. B-cell 85% and
2. T-cell
3. (NK cells very rare)

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17
Q

What is the epidemiology of non-Hodgekin lymphomas?

A

NHL is the most common Haematopoetic neoplasm

Male> Female

  1. Incidence increases with age, peak incidence >50 years
  2. High-grade are more common in children and young adults (20-40)
18
Q

What is the overall clinical presenation of non- Hodgekin lymphomas?

A

Depending on subtype, but overall

High grade:
1. Localised or genralised lymphadenopathy
2. B-symptoms (but more common in HL than NHL)
2. Hepato or Splenomegaly
3. Abdominal discomfort, anaemia, fatigue, change in mental status

Low grade
* Often asymptomatic, insidious onset

19
Q

How are non-Hodgekin lymphomas staged?

A

Same staging as HL: Ann Arbor + A and B

+ A for no constitutional symptoms
+ B for constitutional symptoms

20
Q

What Bloods results might indicate a Lymphoma and which ones should you do?

A

Depening on stage, aggressiveness etc and can be normal

  1. FBC (anaemia, cytopenia (infiltration or chronic disease)
  2. High ESR
  3. If B-cell –> rouleaux formation (if paraprotein present)
  4. Cold agglutinin

Others needed
1. U&E, LFTs, LDH
2. Serum protein electrophoresis
3. BBV screen (HIV, Hep B and C)

21
Q

You have esablished that a patient has a
1. Non- Hodgekin Lymphoma of
2. B-cell origin

How can it be further sub-classified?

A

By maturity of cells
1. Mature
2. Immature (much closer to ALL)

If mature: by aggressiveness of disease
1. Aggressiveness
2. Indolent
3. Other

22
Q

What is the most common B-cell NHL?

What is the malignant cells that are present in it?

A

Diffuse large B-cell lymphoma
(40% of all NHL)

–> Mature Large B-cells

23
Q

What is the epidemiology and prognosis of Diffuse Large B-cell lymphomas?

A

Can occur any age, most common middle aged and elderly

Usually agressive, but 60% can be cured after 6 months of aggressive chemo

Can occur from malignant transformation of low-grade lymphomas

24
Q

What are the histological findings of Large B-cell lymphomas?

A

Sheets of large lymphoid cells

25
Q

What is Rituximab? When is it used?

A

Ritixumab = monocolonal antibody against CD20 (B-cell marker)

Cn be used in conjuction for B-cell malignancies ( and auto-immune diseases)

26
Q

What is the tratment regimen for Diffuse Large B-cell lymphomas?

A

Usually R-CHOP for 6 months

  1. Ritiximab
    • Cyclophosphamide, doxorubicin, vincristinem prednisolole

If that does not work: transplant or other treatments

27
Q

What is the 2nd most common sub-type of NHL after diffuse Large B-cell lymphoma?

A

Follicular lymphoma (25% of NHL lymphomas)

28
Q

What is the characteristic cell+ typical histological findings of Follicular lymphoma on Lympho node biopsy?

A

Mature B-cells related to follicle centre and show follicular growth pattern + nodular appearance on histology
with small cells with cleft nuclei (someimes can also be seen on film)

29
Q

What is the progression and prognosis of patients with follicular lymphoma?

A

Usualy diagnosed at stage III and IV

Indolent but not amazing treatment option –> prognosis 12-15 yeas survival after diagnosis

Might undergo tranformation to Diffuse large B-cell (1-3% risk every year)

30
Q

What genetic mutation is associated with Follicular Lymphoma?

A

Follicular lymphoma associated with
t(14:18) translocation –> formation of BCL-2 gene (can be tesed for diagnosis)

31
Q

What is the managemen of Follicular Lymphoma?

A

Usually wait and watch, until treatment is needed

Then treatment in
1. Chemo-immunotherapy, overall less aggressive if diffuse large B -cell lymphoma
2. Might need to be repeated, usually with shorter intervals between until becomes refractory to treatment

32
Q

What type of Lymphoma is Burkittt’s lymphoma?

What is the epidemiology ?

A

Burkitt’s = Very Agressive, mature B-cell NHL

Most commonly occurs in children (except for HIV associated Burkitt’s lymphoma)

33
Q

What common genetic mutation is associated with Burkitt’s lymphomas?

A

t(8;14) with involvement of myc gene (present in 75%)

34
Q

What are the 3 sub-types of Burkitt’s lymphoma?

What are risk factors associated with each?

A
  1. Sporadic: typically located in the abdomen or pelvis (no jaw)
  2. Endemic: associated with EBV (most prevalent in equatorial Africa and South America - EBV and malaria ineracion) and is typically located in the maxillary and mandibular bones (CHILDREN - EBV- JAW)
  3. Immunodeficiency-associated: e.g. HIV infection
35
Q

How is Burkitt’s lymphoma diagnosed?

A
  1. Haematology (lymhoma cells with strongly basophilic vacuolated cytoplasm)
  2. Lymph node biopts: Starry sky appearance (lymph nodes infilrate tissue but macrophages stand out –> starry sky)
36
Q

How is Burkitt’s lymphoma managed and what is the prognosis?

A

Intensive chemo incl. protection against tumour lysis syndrome

37
Q

What are common clinical presentaions of T-cell non-Hodgekins lyphomas?

A

Variable as verry differnet disease

Generally incureable

Site:
if skin involvement, usually T-cell
Others: lymph nodes, Splee, GI tract (e.g. in Coeliac )

Otherwise typical lymphoma symptoms

38
Q

What are risk facors for the developmet of T-cell lymphomas?

A

Depending on sub-type of t-cell lymphoma

  1. HTLV1 : (viral risk factor for Adult T-cell lymphoma)
  2. EBV infection for several sub-types
39
Q

What is HTLV1?
What is an infecion with HTLV-1 associated with?

A

Virus: e human T-cell lymphotropic virus, common in japan and carribean

Associated with development of Adult T-cell lymphoma (2.5% by age 70), a type of NHL

40
Q

What sub-type of lymphomas are caused by chonic antigen stimmulation with H.pylii, autoimmune conditions, esp. Sjögrens etc. associated with?

A

Extra nodal Marginal Zone lymphomas (MZL)/ MALT

Mature B-cell lymphoma

H.pylori: Gastric
Sjögrens: parotic lymphoma