Indolent Non-Hodgkin Lymphoma Flashcards
what is the definition of indolent NHL?
Cancer of white blood cells (majority B cells)
Type of non-hodgkin lymphoma
Low grade
Slow growing and advanced at presentation
”Incurable”
Many different sub-types (follicular, CLL, marginal zone)
folicular lymphoma
what is the epidemiology of indolent NHL?
Incidence increases with age
Accounts for 15-20% of adult NHL cases
Median age = 60
70% transform into DLBCL
what is the aetiology of indolent NHL?
Most cases unknown
immunodeficiency
what are the risk factors for indolent NHL?
Primary Immunodeficiency e.g. Wiskott-Aldrich Syndrome; Common Variable Immunodeficiency
Secondary Immunodeficiency e.g. HIV; Recipients of Transplant
Infection e.g. EBV; HTLV-1; Helicobacter Pylori
Autoimmune Disorders
what is the pathophysiology of indolent NHL?
Constitutive overexpression of the BCL2 protein, allowing B cells to abrogate the default germinal centre apoptotic program.
what are the key presentations of indolent NHL?
Majority of Indolent Lymphomas present with painless
Lymphadenopathy
Association with B-Symptoms – Fevers, Night Sweats and weight Loss
Bone Marrow involvement – leukaemia component
Autoimmune Phenomena
Compression Syndromes
Organ Involvement e.g. Skin
what are the signs of indolent NHL?
lymphadenopathy
what are the symptoms of indolent NHL?
Fevers, night sweats, weight lossUsually painless
Rashes
what are the first line investigations for indolent NHL?
Lymph node biopsy - core needle, excision node
what are the gold standard investigations for indolent NHL?
Staging:
Lugarno (o Ann Arbor) Staging classification typical for most Indolent lymphomas, Requires Imaging – CT Neck/Thorax/Abdomen/Pelvis or PET-CT
Bloods
Patient assessment to obtain grade
what other investigations could be done for indolent NHL|?
Bone marrow biopsy Genetic tests (FISH, PCR)
what are the differential diagnoses for indolent NHL?
Chronic Lymphocytic Leukaemia/Small Lymphocytic Lymphoma. Diffuse Large Cell Lymphoma. Diffuse Mixed Lymphoma
how is indolent NHL managed?
Varies between subtypes - Watch and wait: Asymptomatic in advanced stage Regular follow up No compromise - Radiotherapy: Local control (palliative) Not curable - Chemotherapy: Alkylating agents Anthracyclines Vinca alkaloids Corticosteroids MCAs
how is indolent NHL monitored?
Some may be treated with the watch and wait method including active monitoring. Regular blood tests and scans to assess tumour
what are the complications of indolent NHL?
Complications due to low levels of blood cells
Around 70% will develop into high grade NHL
