Haematology - Lymphoma Flashcards

1
Q

What is a lymphoma?

A

A neoplastic tumour of lymphoid tissue
- Often LNs (+BM +/- spill out to blood)
- Sometimes other lymphoid tissue (spleen, MALT)

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2
Q

What is the prevalence and epidemiology of Hodgkin’s lymphoma?

A

20%
- M>F
- 20-29yrs + >60yrs
- EBV-associated

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3
Q

What is the spread of Hogkin’s lymphoma?

A
  • Aggressive
  • Spreads continuously to adjacent lymph nodes, often involves single LN group
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4
Q

What is the prognosis of Hodgkin’s lymphoma?

A

Good - mostly curable

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5
Q

What is the clinical presentation of Hodgkin’s lymphoma?

A
  • Asymmetrical painless lymphadenopathy +/- obstructive/mass effect Sx
  • PAIN in AFFECTED NOTES AFTER ALCOHOL
  • Nodes tend to be mediastinal/cervical
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6
Q

What are the B Symptoms?

A
  • Fever >38C
  • Drenching sweats at night
  • Weight loss >10% in 6mths (unintentional)
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7
Q

What are some investigations and findings for Hodgkin’s lymphoma?

A
  • Dx: Tissue = LN/BM biopsy (cells stain with CD15 + CD30)
  • CT/PET
  • REED-STERNBERG CELLS (bi-nucleate/multinucleate cell on background of lymphocytes + reactive cells
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8
Q

What are seom subtypes of Hodgkin’s lymphoma and which is most common?

A
  • Nodular sclerosing (most common)
  • Mixed cellularity
  • Lymphocyte rich
  • Lymphocyte depleted
  • Nodular lymphocyte predominant
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9
Q

How is Hodgkin’s lymphoma staged?

A

Ann-Arbor

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10
Q

What is the Ann-Arbor Staging?

A
  • Stage 1: 1 LN region (inc. spleen)
  • Stage 2: 2+ LNs on SAME SIDE OF DIAPHRAGM
  • Stage 3: 2+ LNs on OPPOSITE SIDES OF DIAPHRAGM
  • Stage 4: Extranodal sites (Liver, BM)
  • A: No constitutional Sx
  • B: Constitutional Sx (B symptoms)
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11
Q

What is the treatment for Hodgkin’s lymphoma?

A
  1. Combination chemotherapy (ABVD - Adraimycin, Bleomycin, Vinblastine + Decarbazine)
  2. Radiotherapy (V. high risk of breast cancer in women - often used alongside chemo)
  3. Relapsed pts: second line chemotherapy (Brentuximab/pembrolizumab/nivolumab) - may need auto/allo-SCT
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12
Q

What is the process of a stem cell transplant?

A
  • Stem cells harvested from: peripheral blood, M or umbilical cord blood
  • Used in leukaemia, lymphoma, MM, aplastic anaemia, MDS, sickle cell anaemia + β thalassaemia
  • Works best if pt in remission as consolidation Tx to reduce relapse risk
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13
Q

What is an autologous stem cell transplant?

A
  • PTs own SCs are harvested + frozen
  • Enables high-dose chemo +/- radiotherapy to eradicate malignant cells
  • Frozen SCs then reintroduced into pt
  • No graft vs leukaemia effect
  • NO GVHD (graft versus host disease)
  • LOWER RISK OF INFECTION

Most commonly used in:
- MM
- Lymphoma (particularly relapse)

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14
Q

What is Graft Vs leukaemia effect?

A

Where the graft is contaminated with malignant cells
- Higher relapse rate

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15
Q

What is an allogenic stem cell transplant?

A
  • HLA-matched donor SCs are harvested
  • Pts own BM is completely eradicated (high-dose chemo +/- radiotherapy)
  • Donor SCs introduced + colonise empty BM
  • GVHD risk
  • Risk of opportunistic infections
  • Risk of infertility
  • Risk of secondary malignancy

Most commonly used in:
- Leukaemia (Graft vs leukaemia effect)
- Myelodysplastic syndrome

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16
Q

What is Non-Hodgkin’s Lymphoma?

A

All lymphomas other than Hodgkin’s

17
Q

How are Hodgkin’s lymphomas classed?

A
  • Mature or Immature
  • Lineage: T/B Cell

High grade histology:
- Very aggressive = Burkitt’s
- Aggressive = Diffuce Large B cell + Mantle Cell

Low grade histollogy:
- Indolent: Follicular, Marginal zone, Small lymphocytic

18
Q

What are some similarities in the presentation of different Non-Hodgkin lymphomas?

A
  • Painless lymphadenopathy (often multiple sites)
  • Constitutional Sx
  • NO PAIN AFTER ALCOHOL
19
Q

What staging system is used for Non-Hodgkin’s lymphoma?

A

Ann- Arbor Staging

20
Q

What are the 3 types of Burkitt’s lymphoma?

A
  • Endemic
  • Sporadic
  • Immunodeficiency
21
Q

What are some general features of Burkitt’s lymphoma?

A
  • Very aggressive
  • Fast growing
  • t(8;14) translocation
  • c-myc oncogene overexpression
  • Rapidly responsive to Tx
22
Q

What is seen on the histology of Burkitt’s lymphoma?

A

STARRY SKY APPEARANCE

23
Q

What is the general management of Burkitt’s lymphoma?

A

Chemotherapy:
- Rituximab (anti-CD20 found on B cells)

+ Secondary CNS prophylaxis

24
Q

What are some features of endemic Burkitt’s lymphoma?

A
  • Most common malignancy in equatorial Africa
  • EBV-ASSOCIATED
  • JAW INVOLVEMENT + abdominal masses
25
Q

What are some features of Sporadic Burkitt’s lymphoma?

A
  • Foun doutside Africa
  • EBV-ASSOCIATED
  • Jaw less commonly involved
26
Q

What are some features of immunodeficiency Burkitt’s lymphoma?

A
  • Non-EBV associated
  • HIV/post-transplant pts
27
Q

What are some features of Diffuse Large B Cell Lymphoma?

A
  • Middle aged + elderly
  • Aggressive
  • Can be transformed from low grade lymphoma
28
Q

What is seen on histology of Diffuse Large B cell lymphoma?

A

Sheets of large lymphoid cells

29
Q

What is the treatment of Diffuse Large B Cell lymphoma?

A
  • Rituximab-CHOP
  • Auto-SCT/CAR-T for relapse
30
Q

What are some features of Mantle Cell lymphoma?

A
  • Middle-aged
  • M>F
  • AGGRESSIVE
  • Disseminated at presentation
  • Median survival = 3-5yrs
  • t(11;14) translocation = over-expression of cyclin D1 (causes deregulation)
31
Q

What is seen on histology of Mantle Cell lymphoma?

A

Angular/CLEFTED nuclei

32
Q

What is the treatment of Mantle Cell Lymphoma?

A
  • Rituximab-CHOP + High-dose Cytarabine
  • Auto-SCT for relapse
33
Q

What are some features of Follicular lymphoma?

A
  • Indolent
  • Mostly incurable
  • Median survival = 12-15yrs
  • t(14;18) translocation = fusion of BCL2 gene
34
Q

What is seen on histology of follicular lymphoma?

A
  • Follicular pattern
  • Nodular appearance
35
Q

What is the treatment of follicular lymphoma?

A
  • Watch + wait
  • Rituximab or obinutuzumab + chemo
36
Q

What are some features of MALT (Mucosal assocaited lymphoid tissue)?

A
  • Marginal zone NHL
  • Middle-aged
  • Chronic antigen stimulation: H. pylori causing gastric MALT OR Sjogren’s causing parotid lymphoma
37
Q

What is the treatment of MALT?

A
  • REMOVE ANTIGENIC STIMULUS
    (e.g. H. pylori)
  • Triple therapy
  • Chemotherapy
38
Q

What are some different T-cell lymphomas and some comments?

A

Anaplastic Large Cell Lymphoma:
- Children + young adults
- Aggressive
- Large “epithelioid” lymphocytes
- t(2;5)
- Alk-1 protein expression

Peripheral T-cell Lymphoma:
- Middle-aged + elderly
- Aggressive
- Large T cells

Adult T cell leukaemia/lymphoma:
- Caribbean + Japanese
- HTLV-1 infection
- Aggressive
- Flower Cells on blood film

Enteropathy-associated T cell lymphoma (EATL):
- A/w: longstanding coeliac disease

Cutaenous T cell lymphoma:
- A/w: mycosis fungoides (weird rashes)

39
Q

What is the general treatment for T-cell lymphomas?

A

Alemtuzumab (anti-CD52)