11. Lymphoproliferative disease Flashcards
Woman, middle aged, enlarged LYMPHS, mild aneamia, mildly raised CRP, mild eosinophila, PET CT shows areas of activity around the upper airways. What’s the diagnosis?
Classsical Hodgkin’s lymphoma
What class of classical Hodgkin’s lymphoma does a woman from case 1 have?
2B
How can you treat classical Hodgkin’s lymphoma
Very radiotherapy sensitive
Chemotherapy also very good, not primary treatment
Monoclonal antibodies, on its way
Stem cell transplant ion, alternative to bone marrow transplant
Surgical resection will NOT work a it’s a blood disease
What is a lymphoma?
Cancers of lymphoid origin
Can present with enlarged lymph nodes or with exrranodL involvement or with bone marrow involvement
Also contains classic B symptoms
night sweats, priuitus, fatigue, (weight loss>10%)
How do you diagnose lymphoma/leukaemia?
Biopsy tells you the type every time
What is ALL?
Acute lymphoblastic leukaemia, occurs due to disease in the lymphoid progenitor cells
What is a lymphoma, CLL?
Lymphoma/Chronic lymphoblastic leukaemia
Caused by disease in the germinal centres
Where does acute leukaemia occur?
Bone marrow
What is MM?
Multiple myeloma, occurs due to disease in the plasma cells
What is Burkett lymphoma?
Clinical emergency, cancer that can double in size in 24 hours
What is a common leukaemia in kids?
Acute lymphoblastic leukaemia
What is ALL
Acute lymphoblastic leukaemia, cancerous disease of lymhoid progenitor cells
Normal=immature, rapidly proliferating cells that differentiate into lymphocytes
Leukaemia- no differentiation, instead rapid uncontrolled growth and accumulation
Usually in the bone marrow but can go anywhere
Describe Acute lymphoblastic lymphoma?
75% occur in children under 6
Typically presents over 2-3 weeks, 1 month impaired visions, tiredness, anaemia, increased white and platelet count
What are the pathological characteristics of ALL?
Large cells
Express CD19- all B cells have this
CD34, TDT- markers of very early, immature cells
Not much else, they haven’t matured and develop
How do you treat ALL?
Induction chemotherapy to obtain remission- go into remission v quick Consolidation therapy CNS directed treatment- Maintenance treatment for 18 months Stem cell transplantation (if high risk)
What are the new therapies for ALL?
Harness the immune system to destroy the cancer using monoclonal antibodies
Massively activated T cells to kill lymphomas
CAR t-cells, patients healthy 3rd party T cells harvested.
What are the side effects of the new T cell immunotherapies?
Cytokine release syndrome
Neurotoxicity
What are the poor risk factors for ALL?
Increasing age
Increased white cell count
Cytogenetics/molecular genetics
slow/poor response to treatment
What is CLL?
Chronic lymphocytic leukaemia, resemble normal lymphocytes, grow slowly or not at all, requires a lymphocyte count >5 for diagnosis
What is the presentation of CLL?
Often asymptomatic
Bone marrow failure
Lymphadenopathy
Splenomegaly
Fever and swears
Less common findings: hepatomegaly, infections, weight loss
Haemolytic anaemia
How do you stage CLL?
Stage A- <3 nodes in onvolved
Stage B- 3 or more lymph node areas
Stage Ç- anaemia and thrombocytopenia and 3 or more LYMPHS
What are the indications for treatment of CLL?
Progressive bone marrow failure
Massive lymphadenopathy
progressive splenomegaly
lymphocyte double time
What is the treatment of CLL?
Watch and wait, can’t cure it
Cytotoxic chemotherapy- fludarabine, bendamustine
Monoclonal antibodies- rituximab, obinatuzumab
Novel agents:
Bruton tyrosine kinase inhibitors
PI3K inhibit e.g. idelalisib
BCL-2 inhibitors e.g. venetoclax
What are poor prognostic markers for CLL?
Advanced disease Atypical lymphocyte morphology Rapid lymphocyte doubling time CD38 expression Loss/mutation p53 del11q23 (ATM gene)— good answer for exams! Unmutated IgVh gene status
