Lymphoprolifitic Disorders

Question 1

What does the term ‘Leuk’ refer to?

Answer 1

white

Question 2

What does the term ‘aemia’ refer to?

Answer 2

blood

Question 3

In what year was the first diagnosis of leukaemia in a live patient made?

Answer 3

1846

Question 4

What is lymphoma generally used to describe?

Answer 4

a cancer that you can see in the blood

Question 5

What are the common presentations of lymphoma?

Answer 5

enlarged lymph nodes, extranodal involvement, bone marrow involvement

Question 6

What are the systemic (B) symptoms associated with lymphoma?

Answer 6

• Weight loss (> 10% in 6 months)
• Fever
• Night sweats
• Pruritis
• Fatigue

Question 7

How many different disease entities are defined in lymphoma diagnosis?

Answer 7

> 70

Question 8

What is used to determine the type of lymphoma?

Answer 8

Biopsy (e.g. lymph node, bone marrow)

Question 9

What does staging in lymphoma describe?

Answer 9

the location and extent of the disease

Question 10

What are examples of lymphoproliferative disorders?

Answer 10

• Acute lymphoblastic leukaemia (ALL)
• Chronic lymphocytic leukaemia (CLL)
• Hodgkin lymphoma
• Non-Hodgkin lymphoma (NHL)

Question 11

What is the incidence of Acute Lymphoblastic Leukaemia (ALL)?

Answer 11

1-2/100,000 population/year

Question 12

In which age group do 75% of ALL cases occur?

Answer 12

children < 6 years

Question 13

What is the usual lineage of ALL cases?

Answer 13

75-90% are of B-cell lineage

Question 14

What are the characteristics of ALL cells?

Answer 14

• Large cells
• Express CD19
• CD34, TDT – markers of very early, immature cells

Question 15

What is the standard treatment for Acute Lymphoblastic Leukaemia (ALL)?

Answer 15

• Induction chemotherapy to obtain remission
• Consolidation therapy
• CNS directed treatment
• Maintenance treatment for 18 months
• Stem cell transplantation (if high risk)

Question 16

What are the poor risk factors for ALL?

Answer 16

• Increasing age
• Increased white cell count
• Cytogenetics/molecular genetics (t(9;22); t(4;11))
• Slow/poor response to treatment

Question 17

What is the 5-year overall survival rate for children with ALL?

Answer 17

~90%

Question 18

What is Chronic Lymphocytic Leukaemia (CLL) characterized by?

Answer 18

abnormal mature cells that resemble normal lymphocytes

Question 19

What is the lymphocyte count requirement for CLL diagnosis?

Answer 19

> 5 (normal is < 4)

Question 20

What are common findings at presentation for CLL?

Answer 20

• Bone marrow failure (anaemia, thrombocytopenia)
• Lymphadenopathy
• Splenomegaly (30%)
• Fever and sweats (< 25%)

Question 21

What is the staging system used for CLL?

Answer 21

Binet

Question 22

What are the clinical features of Binet Stage A CLL?

Answer 22

<3 lymph node areas

Question 23

What is the median survival for Binet Stage B CLL?

Answer 23

~ 8 years

Question 24

What are indications for treatment in CLL?

Answer 24

• Progressive bone marrow failure
• Massive lymphadenopathy
• Progressive splenomegaly
• Lymphocyte doubling time <6 months or >50% increase over 2 months
• Systemic symptoms
• Autoimmune cytopenias

Question 25

What is the typical treatment approach for CLL?

Answer 25

Often nothing – 'watch and wait'

Question 26

What are the treatment options for CLL?

Answer 26

* Cytotoxic chemotherapy (e.g. fludarabine, bendamustine) * Monoclonal antibodies (e.g. Rituximab, obinatuzumab) * Novel agents (e.g. ibrutinib, idelalisib, venetoclax)

Question 27

What are common presentations of lymphoma?

Answer 27

* Lymphadenopathy * Hepatosplenomegaly * Extranodal disease * Bone marrow involvement

Question 28

How is lymphoma staged?

Answer 28

1-4, with A indicating absence and B indicating presence of B symptoms

Question 29

What percentage of non-Hodgkin lymphoma is of B-cell origin?

Answer 29

90%

Question 30

What characterizes low-grade lymphoma?

Answer 30

Indolent, often asymptomatic, responds to chemotherapy but incurable

Question 31

What characterizes high-grade lymphoma?

Answer 31

Aggressive, fast-growing, require combination chemotherapy, can be cured

Question 32

What is the commonest subtype of lymphoma?

Answer 32

Diffuse large B-cell lymphoma

Question 33

What treatment is typically used for Hodgkin lymphoma?

Answer 33

* Combination chemotherapy (ABVD, eBEACOP-Dac) * +/- radiotherapy * Monoclonal antibodies (anti-CD30) * Immunotherapy (checkpoint inhibitors)

Question 34

What is central to the assessment of response to treatment in Hodgkin lymphoma?

Answer 34

PET scanning