Lymphoproliferative disorders Flashcards
What can lymphoid cancers present with?
Enlarged lymph nodes OR extra nodal disease OR bone marrow involvement
May have systemic symptoms
What are the types of lymphoproliferative malignancies?
Hodgkin lymphoma
Non Hodgkin lymphoma
Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia
What is the diagnostic feature of ALL?
> 20% lymphoblasts present in bone marrow
Who are the majority of ALL cases seen in?
75% under 6
What is the presentation of ALL?
2-3 weeks history bone marrow failure or bone/joint pain
Infection, sweats
What investigations are done for ALL?
FBC
Bone marrow aspiration
LP if CNS involvement
What is the standard treatment of ALL?
Chemo- indiction, consolidate and maintenance
CNS directed treatment
What is the treatment for high risk ALL?
Standrad therapy + Stem cell transplant
What newer therapies are available for ALL?
Bispecific T cell engagers
CAR T cells
What are CAR T cells?
Genetically engineered T cells that have certain receptors to attack cancer
What are the possible side effects of CAR T cells?
Cytokine release syndrome
Neurotoxicity
What are the symptoms of cytokine release syndrome?
Fever
Hypotension
Dyspnoea
What symptoms can neurotoxicity in CAR T cell therapy cause?
Confusion Seizure HEadahe Focal neurology Coma
What are the indicators for a bad prognosis in ALL?
Increasing age Increased WCC Immunophenotype Molecular genetics Slow/poor response to treatment
What are the diagnostic features of CLL?
Bone marrow >30% lymphocytes
Blood >5x10^9 lymphocytes
Who is CLL mainly seen in?
Males
What are the common presentations of CLL?
Asymptomatic Bone marrow failure Lymphadenopathy Spelnomegaly Fever and sweats
What are the less common presentation of CLL?
Hepatomegaly
Infections
Weight loss
What is the staging of CLL?
A= <3 lymph node areas B= 3 or more lymph node areas C= Stage B and anaemia or thrombocytopenia
What is the prognosis for CLL?
A= good B= ~8 years C= ~6 years
What are the indications for treatment of 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 treatment of CLL?
Watch and wait
Cytotoxic chemo
Monoclonal antibodies
Novel agents
What gives CLL a bad prognosis?
Advanced disease Atypical lymphocyte morphology Rapid lymphocyte doubling time CD38+ expression Loss/mutation of p53 Unmutated IgVH gene status
What is the presentation of lymphoma?
Lymphadenopathy Hepatosplenomegaly Extra nodal disease Systemic disease Bone marrow involvement
How is lymphoma assessed?
Lymph node biopsy
CT
Bone marrow aspirate
What is the numerical staging of lymphoma?
1- localised
disease
2- 2 or ore lymph node regions on same side of diaphragm
3- 2 or more lymph node regions on opposite side of diaphragm
4- Extranodeal disease
What is the letter staging of lymphoma?
A- no systemic features
B- fever, night sweats, weight loss
How can Non Hodgkin lymphoma be classed?
Lineage
Grade of disease
Histological features of disease
How is non Hodgkin classed by lineage?
B cell or T cell- mainly B
How is Non hodgkin classed by grade?
Low grade- indolent, often asymptomatic, responds to chemo but incurable
High grade- aggressive, require combination chemo, can be cured
What are 2 specific disease entities within Non Hodgkin?
Diffuse large B cell lymphoma
Follicular lymphoma
What is diffuse large B cell lymphoma?
Most common type of non Hodgkin
High grade
Treat with combination chemo
What is follicular lymphoma?
Most common low grade non Hodgkin
Treat with combination chemo
What is the epidemiology of Hodgkin lymphoma?
Males > females
What is the treatment of Hodgkin lymphoma?
Combination chemo +/- radiotherapy
Monoclonal antibodies
Immunotherapy
Monitor with PET scans
