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MBBS II HIS > L20- Lymphomas – principles of diagnosis and management > Flashcards

L20- Lymphomas – principles of diagnosis and management Flashcards

1
Q

Define lymphoma

A

neoplasm/ Clonal malignancy of the lymphoid system

Typically mature** lymphoid neoplasm

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2
Q

Classify broad types of lymphomas by cell of origin?

A
  1. B cells: B cell lymphoma:
    Rearranged IgH gene in stem cell&raquo_space; clonal rearrangement of IgH
  2. T cells: T cell lymphoma:
    Rearranged TCR gene in stem cell&raquo_space; clonal rearrangement of TCR
  3. Natural killer (NK) cells&raquo_space; NK cell lymphoma
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3
Q

List some precursor B cell neoplasms?

A

B lymphoblastic leukemia/ lymphoma

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4
Q

List some Pre-germinal center B cell neoplasms

A

Mantle cell lymphoma

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5
Q

List some germinal center B cell neoplasms?

A

Follicular lymphoma

Burkitt lymphoma

Diffuse large B cell lymphoma

Hodgkin lymphomas

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6
Q

List some post- germinal center B cell neoplasms?

A 
Marginal zone (MALT) lymphoma 
Lymphoplasmacytic lymphoma 
CLL/SLL
Diffuse large B-cell lymphoma 
Plasmacytoma
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7
Q

Classify lymphoma by neoplastic behavior.

A

1) B cell lymphomas + B - lymphoproliferative diseases
a) High grade or Low-grade B cell lymphomas/ BLPD

2) T-cell lymphomas
3) NK/T cell lymphomas

4) Hodgkin lymphoma
a) Classical HL
b) Nodular lymphocyte predominant HL

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8
Q

List examples of high grade and low grade B cell lymphomas?

A

High grade = DLBCL, Burkitt lymphoma

Low grade = Follicular lymphoma, chronic B-LPD i.e. CLL

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9
Q

Difference between high grade and low grade B cell lymphoma in presentation?

A

High = Rapid proliferation, acute onset with severe symptoms

Low = Indolent, late-stage presentation with moderate symptoms

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10
Q

Which viral infections are asso. with T cell lymphomas?

A

EBV*** = asso. with NK cell lymphomas

HTLV-1
HIV

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11
Q

Age group affected by lymphomas?

A

Typical = adults after 4th decade

Extremely rare in children/ young adults

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12
Q

List some general symptoms of lymphoma?

A

 Unexplained fever > 38oC (because inflammatory)

 Night sweats

 Unintentional loss of > 10% body weight in 6 months

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13
Q

Fine-needle aspiration of affected lymph nodes is adequate for lymphoma investigation. T or F?

A

False

Simple cytological exam not acceptable

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14
Q

Describe the sequence of investigations for lymphoma? (from dx to treatment)

A
  1. Accurate Dx by clinical, morphological, immunophenotypic and genetic features
  2. Staging for prognosis, complications + choose treatment
  3. Determine suitability or fitness for treatment
  4. Detect response and complications to treatment
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15
Q

List the blood metrics that are investigated in lymphoma?

A

General:

  • CBC
  • Serum electrolyte levels (Na, K, Ca, PO4)
  • LDH and urate levels (tumor lysis syndrome)

Specific:

  • ESR (marker for early classical HL)
  • β2-microglobulin (marker for follicular lymphoma)
  • Serum protein electrophoresis used for finding para-proteins
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16
Q

List expected abnormal blood findings in tumour lysis syndrome?

A
  • Very high K+, PO4, Urate, LDH

- Low Ca

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17
Q

Define tumour lysis syndrome?

A

when a large number of cancer cells die within a short period (i.e. by chemotherapy), releasing their contents in to the blood

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18
Q

List some radiological exams for lymphoma investigation?

A
  • CXR (to look for disease-related complications, active or prior infections)
  • PET-CT for staging
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19
Q

List some investigation for staging of lymphoma?

A

PET-CT

Bone marrow aspiration and trephine biopsy

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20
Q

Name the system used to stage lymphomas?

A

Ann Arbor Staging

4 stages

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21
Q

Describe stage 1 lymphoma in Ann Arbor Staging?

A

Involvement of Single lymph node region or single extralymphatic site

22
Q

Describe stage 2 lymphoma in Ann Arbor Staging?

A

Involvement of TWO OR MORE lymph node regions on the SAME SIDE of the diaphragm

May include localized extra-lymphatic region

23
Q

Describe stage 3 lymphoma in Ann Arbor Staging?

A

Involvement of lymph node regions on BOTH SIDES of the diaphragm

May include spleen or localized extralymphatic regions

24
Q

Describe stage 4 lymphoma in Ann Arbor Staging?

A

DIFFUSE EXTRALYMPHATIC disease

e.g. in liver, BM, lung, skin…

25
List the investigations for suitability or fitness for treatment for lymphoma? Explain why these tests are done?
Due to chemo drug ADR: - ECG, transthoracic echocardiogram (doxorubicin cardiotoxicity) - Lung function study (MTX lung, pulmonary fibrosis from bleomycin) - Hep. B and C serology (reactivation after immunosuppressant) - HIV serology (concurrent feature) - G6PD assay (Oxidative hemolysis from Co-trimoxazole)
26
Main treatment modality of lymphoma? ***
- Multi-agent cytotoxic chemotherapy +/- monoclonal antibodies +prevention of tumour lysis syndrome and complications - Multi-cycle with interim and end assessments i. e. R- CHOP - Rituximab (mAb) - Cyclophosphamide (alkylating) - Doxorubicin (anthracycline) - Vincristine (plant derivative) - Prednisolone (steroid)
27
Which patients with lymphoma is indicated for HSCT?
autologous HSCT | selected relapsed patients who are chemo-sensitive
28
List 2 types of drugs used against less typical types of lymphoma?
Immuno-conjugates Checkpoint inhibitor
29
Diffuse Large B Cell Lymphoma: clinical presentation?
- Unintentional weight loss - Rapidly enlarging, firm, non-tender lymph nodes typically in neck, abdomen, mediastinum - Extranodal involvement is common in GIT - Fever, night sweat possible
30
Histological features of DLBCL lymph nodes?
1. Effaced nodal architecture, loss of follicular structure 2. Sheets of large abnormal, hypermetabolic lymphoid cells
31
What is the expected immunohistochemistry findings in DLBCL?
CD19+, CD20+, CD79a+, PAX5+. T-cell and NK cell markers negative
32
Treatment modality of DLBCL?
Entecavir prophylaxis + R-CHOP Rituxumab + Cyclophosphamide + Doxorubicine + Vincristine + Prednisolone
33
Typical presentation of Classical Hodgkins lymphoma?
symptomatic lymphadenopathy +/- mediastinal mass*****on chest radiography fever, night sweats, unintended weight loss, loss of appetite
34
Possible causes of mediastinal mass on CXR?
- Lymphoma/ metastatic LN - Thymoma - Germ cell tumour, Neurofibroma - Retrosternal Goitre - Dilated aortic arch
35
Histological features of LN in classical Hodgkin lymphoma?
1. Distorted nodal architecture 2. Typical multinucleated Reed-Sternberg cells**** were present 3. Prominent infiltration by inflammatory cells (reactive lymphocytes, eosinophils and plasma cells)
36
Typical immuno-histochemical study for classical Hodgkin lymphoma?
1. CD30 immunostain***** highlight the malignant Hodgkin and ReedSternberg cells 2. negative for other B- and T cell markers
37
Typical blood investigation results for classical Hodgkin lymphoma?
- elevated LDH and urate - Eosinophilia** - Normal serum electrolytes
38
Typical blood investigation results for DLBCL?
- Grossly elevated LDH, Urate | - Normal CBC, serum electrolytes
39
Typical treatment modality for classical Hodgkin lymphoma?
ABVD +/- Rituximab (+ Febuxostat) Adriamycin, Bleomycin, Vinblastine, Dacarbazine
40
Why is Febuxostat added to chemotherapy regimens for diff. lymphomas?
To manage tumour lysis syndrome
41
Which chemotherapy drug can accumulate if taken with allopurinol or febuxostat?
6-Mercaptopurine
42
Treatment modality for relapsed/ unresponsive Classical Hodgkin Lymphoma?
1) immuno-conjugate Brentuximab vedotin: anti-CD30 monoclonal ab linked to MMAE (MMAE is a microtubule disrupting agent) 2) Check-point inhibitor: Pembrolizumab (anti-PD1)
43
MoA of Anti-PDl or Anti-PDL-1 chemo drugs?
PD-L1 binds to PD-1 and inhibits T cell killing of the tumour cell Chemo drug blocks PD-L1 or PD-1 >> allow T cell killing
44
Typical clinical presentation of NK cell lymphoma?
- Nasal swelling, perforation of hard palate, deviated nasal septum - Intermittent epistaxis for 4 weeks - Tumour in nasal cavity and nasopharynx with local destruction - RARE primary nodal presentation
45
Typical histological finding in biopsy of nasal tumour in NK cell lymphoma?
- neoplastic lymphoid infiltrate exhibiting angiocentricity and angiodestruction
46
Typical immunohistochemical test finding for NK cell lymphoma?
- CD16 and CD56 positive **marker for NK** | - cytoplasmic CD3ɛ, cytotoxic markers (perforin, granzyme) and Ebstein-Barr virus encoded RNA (EBER) positive
47
List some clinical presentations of aggressive NK cell lymphoma?
fever, rash, hepatosplenomegaly, pancytopenia and hemophagocytosis
48
List some non-nasal sites that may be involved in NK cell lymphoma?
skin, GI tract and testes
49
Finding primary nodal involvement is very common for NK cell lymphomas. T or F?
False Primary nodal presentation is extremely rare
50
Typical treatment modality for NK cell lymphoma?
``` Multi-drug chemo: LEMIL + L-asparaginase + Etoposide (topoisomerase-I inhibitor) + Methotrexate (anti-metabolite) + Ifosfamide (alkylating) + Local irradiation ``` Steroids

MBBS II HIS (30 decks)

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