Lymphoprolifitic Disorders Flashcards
What does the term ‘Leuk’ refer to?
white
What does the term ‘aemia’ refer to?
blood
In what year was the first diagnosis of leukaemia in a live patient made?
1846
What is lymphoma generally used to describe?
a cancer that you can see in the blood
What are the common presentations of lymphoma?
enlarged lymph nodes, extranodal involvement, bone marrow involvement
What are the systemic (B) symptoms associated with lymphoma?
- Weight loss (> 10% in 6 months)
- Fever
- Night sweats
- Pruritis
- Fatigue
How many different disease entities are defined in lymphoma diagnosis?
> 70
What is used to determine the type of lymphoma?
Biopsy (e.g. lymph node, bone marrow)
What does staging in lymphoma describe?
the location and extent of the disease
What are examples of lymphoproliferative disorders?
- Acute lymphoblastic leukaemia (ALL)
- Chronic lymphocytic leukaemia (CLL)
- Hodgkin lymphoma
- Non-Hodgkin lymphoma (NHL)
What is the incidence of Acute Lymphoblastic Leukaemia (ALL)?
1-2/100,000 population/year
In which age group do 75% of ALL cases occur?
children < 6 years
What is the usual lineage of ALL cases?
75-90% are of B-cell lineage
What are the characteristics of ALL cells?
- Large cells
- Express CD19
- CD34, TDT – markers of very early, immature cells
What is the standard treatment for Acute Lymphoblastic Leukaemia (ALL)?
- Induction chemotherapy to obtain remission
- Consolidation therapy
- CNS directed treatment
- Maintenance treatment for 18 months
- Stem cell transplantation (if high risk)
What are the poor risk factors for ALL?
- Increasing age
- Increased white cell count
- Cytogenetics/molecular genetics (t(9;22); t(4;11))
- Slow/poor response to treatment
What is the 5-year overall survival rate for children with ALL?
~90%
What is Chronic Lymphocytic Leukaemia (CLL) characterized by?
abnormal mature cells that resemble normal lymphocytes
What is the lymphocyte count requirement for CLL diagnosis?
> 5 (normal is < 4)
What are common findings at presentation for CLL?
- Bone marrow failure (anaemia, thrombocytopenia)
- Lymphadenopathy
- Splenomegaly (30%)
- Fever and sweats (< 25%)
What is the staging system used for CLL?
Binet
What are the clinical features of Binet Stage A CLL?
<3 lymph node areas
What is the median survival for Binet Stage B CLL?
~ 8 years
What are indications for treatment in CLL?
- Progressive bone marrow failure
- Massive lymphadenopathy
- Progressive splenomegaly
- Lymphocyte doubling time <6 months or >50% increase over 2 months
- Systemic symptoms
- Autoimmune cytopenias
What is the typical treatment approach for CLL?
Often nothing – ‘watch and wait’
What are the treatment options for CLL?
- Cytotoxic chemotherapy (e.g. fludarabine, bendamustine)
- Monoclonal antibodies (e.g. Rituximab, obinatuzumab)
- Novel agents (e.g. ibrutinib, idelalisib, venetoclax)
What are common presentations of lymphoma?
- Lymphadenopathy
- Hepatosplenomegaly
- Extranodal disease
- Bone marrow involvement
How is lymphoma staged?
1-4, with A indicating absence and B indicating presence of B symptoms
What percentage of non-Hodgkin lymphoma is of B-cell origin?
90%
What characterizes low-grade lymphoma?
Indolent, often asymptomatic, responds to chemotherapy but incurable
What characterizes high-grade lymphoma?
Aggressive, fast-growing, require combination chemotherapy, can be cured
What is the commonest subtype of lymphoma?
Diffuse large B-cell lymphoma
What treatment is typically used for Hodgkin lymphoma?
- Combination chemotherapy (ABVD, eBEACOP-Dac)
- +/- radiotherapy
- Monoclonal antibodies (anti-CD30)
- Immunotherapy (checkpoint inhibitors)
What is central to the assessment of response to treatment in Hodgkin lymphoma?
PET scanning
