Haematology 5 - lymphoma 2

Question 1

Radiotherapy dilemma in HL

Answer 1

Rdiotherapy can increase cure rate but more patients die of secondary malignancies

Question 2

CD5+ and CD19+ cells

Answer 2

B-CLL, these cells should have switched off CD5

Question 3

hODGKIN’S LYMPHOMA epidemiology

Answer 3

M>F (but more common in F in 20-30s)

Bi-modal: 20-29 most common then >60

Question 4

Which subtype of HL is more common in females?

Answer 4

nodular sclerosing type

Question 5

Where does HL arise f rom?

Answer 5

GC or post GC

Question 6

Which virus is HL associated with?

Answer 6

EBV

Question 7

Diagnostic markers for HL

Answer 7

CD30, CD15, CD20-ve

Question 8

Most common type of HL

Answer 8

Nodular sclerosing

Question 9

Classical HL with poor prognosis

Answer 9

Lymphocyte depleted

Question 10

disorder of the elderly multiple recurrences (HL)

Answer 10

Nodular Lymphcoyte Predominant HL

Question 11

Difference between NLPHL and Classical HL

Answer 11

Not associated with EBV
No eosinophils or macrophages
Can transform in to high grade B cell lymphoma (NHL)
Negative for CD30 + CD15, positive for CD20

Question 12

Staging methods for HL

Answer 12

FDG-PET, CT, biopsy

Question 13

In Ann Arbor staging, what is the spleen considered as?

Answer 13

One giant lymph node

Question 14

Main complications of nodular sclerosing HL

Answer 14

SVCO, tracheal compression

Question 15

1st line mx of HL (chemo)

Answer 15

1st: ABVD
Adriamycin
Bleomycin
Vincristine
Dacarbazine

Given at 4-weekly intervals, preserves fertility

2nd: PET-CT –> Radiotherapy

Question 16

Long term consequences of ABVD

Answer 16

Pulmonary fibrosis

Cardiomyopathy

Question 17

Disadvantages of radiotherpay in HL

Answer 17

Collateral damage + increased risk of breast/lung/skin cancer, leukaemia, myelodysplasia

Question 18

Mx of patients who relapse?

Answer 18

High dose chemotherapy + autologous SCT

3rd line: anti-CD30 +ANTI-PD1

Question 19

wHAT % of HL patients will be long term survivors?

Answer 19

80%

Question 20

After 5 years post cure, patients more likely to die from…

Answer 20

secondary malignancy or CVS complications

Question 21

What is the fastest growing human cancer?

Answer 21

Burkitt’s

Question 22

Prognostic markers and important tests in NHL

Answer 22

Prognostic marker: LDH (marker of cell turnover), performance status
Important tests: HIV, HTLV-1 serology, hepB serology (treatment of NHL depletes B cells and if infected with HepB they can –> fulminant liver failure)

Question 23

Marginal zone lymphoma of the stomach = ?

Answer 23

MALToma

Question 24

Difficulty in treating indolent lymphomas

Answer 24

Go in to remission but tend to reoccur and when they do, less responsive to treatment

Question 25

System used to assess prognosis in DLBCL

Answer 25

IPI = international prognostic index

Question 26

Treatment of DLBCL

Answer 26

``` 6-8 cycles of R-CHOP Rituximab Cyclophosphamide Adriamycin Vincristine Prednisolone ```

Question 27

If relapse after R-CHOP for DLBCL

Answer 27

Autologous SCT

Question 28

Translocaiton in follicular lymphoma and what does it lead to the overexpression of?

Answer 28

t(14;18), BCL-2

Question 29

Treatment of follicular lymphoma

Answer 29

It is incurable, at presentation you watch adn wait | If symptomatic e.g. obstruction --> R-CVP

Question 30

Casue of marginal zone lymphomas

Answer 30

Chronic antigen stimulation (they tend to be extra nodal and NOT associated with B symptoms)

Question 31

Characteristic of EATL

Answer 31

Aggressive but NOT that responsive to treatment

Question 32

Tdt CD19

Answer 32

CLL

Question 33

Mature b cells specifically express

Answer 33

CD19, sIg

Question 34

CD19 and CD5 status in normal mature B cells

Answer 34

CD19+ve, CD5-ve

Question 35

Normal mature T cells immunophenotyping

Answer 35

CD3, CD4, CD8, CD5+ve, CD19 -ve

Question 36

Mature B cells in cLL

Answer 36

CD19 and CD5 +Ve

Question 37

Staging used in CLL

Answer 37

Rai or Binnet

Question 38

3 stages in Binet staging

Answer 38

``` A = <3 Lymphoid areas B = >3 lymphoid areas C = Hb <100g/L, plts <100x10^9/L ```

Question 39

Ig gene mutation status and prognosis

Answer 39

IgH mutated = good, unmutated = bad!!!

Question 40

If IgH mutated and unmated, where inthe lymphoid follicle do they arise from?

Answer 40

``` Mutated = post-GC CLL Unmutated = pre-GC CLL ```

Question 41

Most important deletion in CLL which is associated with bad prognosis

Answer 41

chromosome 17p deletion (TP53) --> LOSS OF P53 TSG

Question 42

Transformation in CLL

Answer 42

Richter's transformation --> DLBCL

Question 43

Autoimmune disease associated with CLL

Answer 43

Warm AIHA

Question 44

Treatment of CLL

Answer 44

1) Supportive - vaccination (pneumococcus flu), anti-infective prophylaxis e.g. aciclovir, PCP prophylaxis, IVIG for those with hypogammaglobulinaemia and recurrent infections 2) mainly watch and wait 3) Richter's transofrmation --> R-CHOP 4) Young pts allogenic SCT

Question 45

Indications for treatment in CLL

Answer 45

``` Progressive lymphocytosis, count doubling <6 months Progressive BMF (hB<100, Plts <100, neutrophils <1) , massive splenomegaly/lymphadenopathy, autoimmune cytopaenias (treat with steroids) ```

Question 46

Management of TP53/17p deleted CLL

Answer 46

1) ibrutinib (Bruton TKI) - refractory CLL p53mutation 2) venetoclax (bcl2 inhibitor) 3) CAR-T therapy