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Pathology by week > W13 - Lymphoma 2 > Flashcards

W13 - Lymphoma 2 Flashcards

1
Q

How could a lymphoma present (6)?

A
  1. Painless progressive lymphadenopathy (palpable node)
  2. Extrinsic compression of any tube (ureter, bile duct, large blood vessel, bowel, trachea oesophagus)
  3. Infiltrate/impair an organ system (skin rash, ocular, CNS, liver failure)
  4. Recurrent infections
  5. Constitutional/B symptoms (weight loss, night sweats, fever)
  6. asymptomatic
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2
Q

15% of lymphomas are _____________ and the other 85% are ______________

A

Hodgkin’s lymphoma (15%)

Non-Hodgkin’s lymphoma (85%)

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3
Q

For lymphoma, you want a histological diagnosis (LN). What investigations (4) do you order next?

A
  1. Imaging - CT/PET scans
  2. BM biopsy - if BM involvement
  3. LP - if meningeal involvement
  4. Bloods - LDH, albumin, kidney/BM function, HIV/HepB serology, HTLV1
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4
Q

NHL can arise from affected precursor and mature B or T cells - T or F

A

True

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5
Q

HL - more common in males or females?

A

Males

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6
Q

Describe the bimodal age incidence of HL

A

Most common age 20-29 = young women with NS (nodular sclerosing) subtype

Second smaller peak affects >60 years

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7
Q

5 common presentations of HL

A
  1. Painless enlarged LN/node
  2. Obstructive symptoms (tracheal obstruction, SVC compression)
  3. B symptoms (fever, WL, night sweats)
  4. Pruritus
  5. Alcohol-induced pain
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8
Q

Describe classification of HL into classical and nodular

A

Classical HL:

  1. Nodular sclerosing (80%) => good prognosis, young women
  2. Mixed cellularity (17%) => good prognosis
  3. Lymphocyte rich (rare) => good prognosis
  4. Lymphocyte depleted (rare) => poor prognosis

Nodular lymphocyte predominant HL (5%) => disorder of the elderly, multiple recurrences

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9
Q

cHL spreads contiguously = what does that mean for staging?

A

spreads to nearby structures first, then to distant areas.

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10
Q

What scans can be used for HL staging?

A

PET or CT scans

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11
Q

Describe the Ann Arbor staging

A

Stage I = one group of nodes
Stage II = >1 group of nodes same side of the diaphragm
Stage III = nodes above AND below the diaphragm (inc. spleen)
Stage IV = extra-nodal spread (liver, bone)

Suffix A IF none of B symptoms
Suffix B if any B symptom present (fever, unexplained WL of >10% in 6m, night sweats)

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12
Q

What is a feature of this nodular sclerosing cHL?

A

large mediastinal mass that may be so massive that it compresses SVC or trachea

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13
Q

What modality of cancer treatment is used for cHL? give name, duration of treatment, effect on fertility, and 2 long-term S/Es.

When is interim response checked?

A
  • chemotherapy
  • ABVD (4 chemo drugs)
  • 4-weekly intervals for 2-6 cycles
  • fertility preserved
  • pulmonary fibrosis, cardiomyopathy
  • Interim response checked after x2 cycles and end of treatment => may need radiotherapy
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14
Q

What is the effect of radiotherapy for cHL? Give 3 issues with its use.

A

Good for eradicating HL but there are 3 problems:

  1. cannot cure HL by itself
  2. Damage to normal tissue (risk of breast cancer, leukaemia, lung or skin cancer)
  3. Combined modality (RT + CT) => greatest risk of 2ndary malignancy
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15
Q

What is the prognosis of HL?

A
  • older patients do less well
  • lymphocyte-depleted cases do less well
  • 80% of those with stage I/II are cured
  • 50% of those with stage IV are cured
  • 10% die from relapse of HL in 1st 10 years
  • 10% die from treatment complication after 10 years
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16
Q

For NHL, you want a histological diagnosis (LN). What investigations (4) do you order next?

A
  1. Stage the disease similarly to HL
    - Imaging - CT or PET
    - BM biopsy
    - LP (if CNS involvement)
  2. Prognostic markers:
    - LDH
    - Performance status
    - HIV/HTLV1 serology
    - Hep B screen
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17
Q

What are the two most common NHL subtypes?

A
  1. Diffuse large B cell lymphoma (30-40%)
  2. Follicular lymphoma (25-30%)
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18
Q

Classify subtypes (7) of NHL into very aggressive, aggressive, and indolent types

A

Very aggressive:

  1. Burkitt Lymphoma
  2. T or B cell lymphoblastic leukaemia/lymphoma

Aggressive:

  1. Diffuse large B cell lymphoma
  2. Mantle cell

Indolent:

  1. Follicular lymphoma
  2. Small lymphocytic/CLL
  3. Mucosa associated (MALT)
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19
Q

Describe median survival of very aggressive, aggressive, and indolent NHL

and describe their response to chemo

A

Very aggressive = 2-5 weeks without Rx

Aggressive = 3-12 months without Rx

Indolent = 10-15 years

paradoxically, very aggressive is curable with chemo whereas indolent is incurable (long remissions)

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20
Q

Describe treatment for NHL

A

Very aggressive = treated like acute leukaemia

Aggressive NHL & indolent NHL = depends on type

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21
Q

Treatment for DLBCL

A

Chemo (Rituximab-CHOP) - 6-8 cycles

  • 50% curative
  • if relapsed => autologous SCT salvage 25% of patients
22
Q

Treatment for follicular NHL

A
  1. W&W - only treat if clinically indicated (i.e. nodal extrinsic compression, massive painful node)
  2. Treatment - immunotherapy R-CHOP (not curative)
23
Q

What is the translocation associated with follicular NHL

A

t(14;18) => antibody H chain enhancer + Bcl2 => overexpress Bcl2 which is an anti-apoptosis protein

24
Q

Prognosis for follicular NHL

A

incurable with median survival 12-15 years
- may require 2-3 different chemotherapy schedules over the 15 year period

25
Q

Name 2 types of Marginal Zone Lymphoma (MZL) NHL?

A

Extra-nodal lymphoid tissue:
1 - Gastric mucosa-associated lymphoid tissue MALT/H pylori

2 - Parotid MZL/Sjogren’s syndrome)

26
Q

What % of all NHL is extra-nodal MZL?
What is the median age at presentation?

A

10% of all NHL
55-60 years

27
Q

Symptoms of Gastric MALT? Treatment?

A

epigastric pain, ulceration, or bleeding

H pylori eradication may cure 75% of patients

28
Q

What lymphoma is associated with coeliac disease? Is it aggressive or indolent?

A

EATL = Enteropathy associated T cell lymphoma = a T cell NHL that is aggressive

29
Q

What type of cells are involved in EATL? which regions of GI tract are involved?

A

T cell NHL (mature T cells), small intestine jejunum and ileum

30
Q

What is the aetiology of EATL and Coeliac disease?

A

Chronic antigen stimulation (gluten) in a gluten sensitive person (Coeliac disease +)

31
Q

What is the clinical presentation (5) of EATL?

A
  1. Abdominal pain
  2. Obstruction/perforation
  3. GI bleeding
  4. Malabsorption
  5. Systemic symptoms
32
Q

What is the treatment for EATL?

A
  • responds poorly to chemo
  • generally fatal
  • best option is to aim to PREVENT (strict adherence to gluten free diet)
33
Q

22 year old female with cHL, mediastinal mass - most likely subtype is:

A

nodular sclerosis

34
Q

cHL PET CT shows disease involving mediastinum, spleen, and liver. What Ann Arbor Stage?

A

Stage IV

35
Q

NHL: Monitoring only is appropriate for asymptomatic small volume disease in this lymphoma subtype:

  • Burkitt lymphoma
  • Gastric MALT (extra-nodal MZL)
  • Follicular lymphoma
  • Diffuse large B-cell lymphoma
A
  • Follicular lymphoma!! (indolent but incurable - just W&W)

burkitt = aggressive
diffuse large = aggressive
MALT = indolent but should give abx

36
Q

CLL is proliferation of mature _________ and most commonly presents at median age ____

A

B lymphocytes, 72

37
Q

What are typical lymphocyte counts on FBC for CLL?

A

between 5 - 300 x 10^9 cells/L

if above 200, you are certain it’s CLL
if between 10-20 => do peripheral blood smear

38
Q

What FBC laboratory findings are found for CLL?

A
  1. Lymphocytosis (5-300x10^9/L)
  2. Anaemia (reduced RBC count)
  3. Low platelets
39
Q

What features are seen in peripheral blood smear and on BM biopsy for CLL?

A

Blood film:

  1. Lymphocytosis
  2. Smear cells
  3. Normocytic normochromic anaemia
  4. Thrombocytopaenia

BM:
- BM lymphocytic replacement of normal marrow elements

40
Q

If you perform immunophenotyping by flow cytometry of peripheral blood of someone with CLL, what do you find?

A

CD5- CD19+ cells = normal B cells

CD5+ CD19- cells = normal T cells

CD5+ CD19+ cells = double positive cells are CLL!! CD5 is expressed by intermediate B cells and should normally be LOST prior to B cell maturation and exit into peripheral blood.

41
Q

What is the prognosis of CLL?

A

The rule of 1/3s:

  • In a disorder of elderly
    1) 1/3 never progress

2) 1/3 Progress, respond to CLL Rx (death from unrelated disorder)
3) 1/3 Progress, require multiple lines of Rx, refractory disease, death from CLL

42
Q

What are 3 cell based prognostic factors in CLL?

A
  1. IgHV mutation status (mutated = BETTER prognosis)
  2. CLL FISH cytogenetic panel
  3. TP53 mutation status (chr 17p deletion and/or TP53 point mutation) = MOST IMP**** know this!
43
Q

What are some of the clinical issues (5) of CLL?

A
  1. Population of non-functional malignant cells + hypo-gammaglobulinaemia = INCREASED RISK OF INFECTION
  2. Proliferation within BM = BM FAILURE
  3. Circulate to LNs, spleen, blood = lLYMPHADENOPATHY/ SPLENOMEGALY/ LYMPHOCYTOSIS
  4. Acquire further mutations = TRANSFORM TO HIGH-GRADE LYMPHOMA
  5. Disease of immune cells = AUTO-IMMUNE COMPLICATIONS i.e. IHA
44
Q

Management of CLL - 3 steps

A
  1. Supportive care (vaccination, abx, etc)
  2. W&W
  3. immuno-chemotherapy
  4. Cellular therapy
45
Q

Describe supportive care and W&W for CLL

A
  1. Supportive care:
    - Sino-pulmonary infections:
    => early abx
    => pneumocystis prophylaxis
    => recurrent infection + igG low > give IVIG replacement
    - vaccinations:
    => penumococcal
    => covid19
    => seasonal flu
    => avoid live vaccines
  2. W&W if no:
    => rapidly progressive lymphocytosis
    => BM failure, or
    => massive splenomegaly
46
Q

Describe immuno-chemotherapy and cellular therapy for CLL

A
  1. Immuno-chemotherapy:
    - BTK inhibitors (ibrutinib)
    - BCL2 inhibitors (Venetoclax)
  2. Cellular therapy (only for relapsed high risk cases):
    - allogeneic SCT
    - CAR-T therapy
47
Q

Name of BCR kinase inhibitor and BCL2 inhibitor used in CLL treatment

A

BCR kinase inhibitor = IBRUTINIB

BCL2 inhibitor = VENETOCLAX

48
Q

How do Ibrutinib and venetoclax work? which is associated with tumour lysis syndrome?

A

Ibrutinib = bind BTK = blocks this kinase = cells dont divide as quickly (BTK highly expressed on B cells)

Venetoclax = bind BLC2 = blocks it = permits apoptosis of CLL cells

Venetoclax associated with risk of Tumour lysis syndrome

49
Q

Immunophenotype of a normal peripheral blood lymphocyte is:
1. CD3+ CD5+ CD19-

  1. CD3- CD5- CD19+
  2. CD3+ CD5- CD19+
A
  1. CD3- CD5- CD19+
50
Q

CLL: Who has the worst prognosis?
1. IgHV mutated, TP53 WT

  1. IgHV mutated, TP53 mut+
  2. IgHV unmutated, TP53 WT
  3. IgHV unmutated, TP53 mut+
A

IgHV unmutated has worse prognosis,
TP53 mutated has worse prognosis
so

  1. IgHV unmutated, TP53 mut+
51
Q

Venetoclax in CLL treatment:

  1. Blocks BCL2 protein
  2. Targets CD20 antigen on B cells
  3. Inhibits TP53 protein
  4. Irreversibly binds to BTK
  5. PD1 ligand inhibitor
A
  1. Blocks BCL2 protein

Pathology by week (70 decks)

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