Lymphoid Malignancy Flashcards
How are blood/lymphatic cancers defined?
Malignant cell characteristics
How are lymphoma/leukaemia diagnosed?
Biopsy
Clinical examination/imaging for staging
What are the main lymphoproliferative disorders?
Acute lymphoblastic leukaemia (ALL)
Chronic lymphocytic leukaemia (CLL)
Hodgkins and Non-Hodgkins lymphoma
How are non-hodgkins lymphomas differentiated?
High grade (diffuse large B-cell lymphoma) Low grade (Follicular, marginal zone)
What is Acute Lymphoblastic Leukaemia?
Cancerous disorder of Lymphoid progenitor cells
mostly bone marrow, could be anywhere
Acute Lymphoblastic Leukaemia is more common in which groups?
Younger male (<6 likely)
1-2/100,000 per year
Down’s Syndrome++
How does Acute Lymphoblastic Leukaemia present?
Recent History of bone marrow failure or bone/joint pain++ Impaired vision Weight loss Infection Sweats
How does Acute Lymphoblastic Leukaemia present on investigation?
FBC: Anaemia, elevated WCC, low platelets
Bone marrow: Increased B-lymphoblasts
Histology: Large cells, Express CD19 (+CD34, TDT), Loss of fat space
How is Acute Lymphoblastic Leukaemia treated?
Chemotherapy to induce remission, CNS therapy
Stem cell treatment if high risk
Bi-specific T-cell engagers
Chimeric Antigen Receptor (CAR) T-cells
What side effects are associated with T-cell immunotherapy?
Cytokine release syndrome (fever, hypotension, dyspnoea)
Neurotoxicity (seizures, coma)
What are the poor prognostic factors for Acute Lymphoblastic Leukaemia?
Increasing age
Increased WCC
Genetics (philadelphia chromosome)
Poor response to therapy
How is Chronic Lymphocytic leukaemia diagnosed?
Lymphocytes >5
Mature abnormal cells
Low grade markers
How does Chronic Lymphocytic leukaemia present?
Asymptomatic Bone marrow failure (Anaemia, thrombocytopenia) Lymphadenopathy Splenomegaly Fever and sweats Immune paresis Haemolytic anaemia
What findings are often associated with CLL?
Immune paresis (loss of normal immunoglobulin production) Haemolytic anaemia
How is CLL staged?
Binet Staging
A < 3 lymph node areas
B 3 or more
C 3 or more + anaemia or thrombocytopaenia
What median survival is associated with each grade of CLL?
A ~
B - 8 years
C - 6 years
What are the indications for treatment in CLL?
Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Systemic symptoms
What is the treatment for CLL?
“watch and wait”
Cytotoxic chemotherapy
Monoclobal antibodies
TKIs
What are the poor prognostic factors for CLL?
Advanced disease (stage B, C) Atypical lymphocyte morphology Rapid lymphocyte doubling time p53 loss, del 11q23 CD38+ expression
How do lymphomas typically present?
Lymphadenopathy Hepatosplenomegaly Extranodal disease Bone marrow involvement Fever Night sweats Weight loss
How is Lymphoma investigated?
Lymph node biopsy
CT scan
Bone marrow aspirate and trephine
How is Lymphoma staged?
Stage 1: 1 node group on 1 side of diaphragm 2: 2 node groups same side of diaphragm 3: Node groups either sides of diaphragm 4: Extranodal metastases A: absence of B symptoms B: Fever, night sweats, weight loss
Lymphomas typically affect what?
B and T lymphocytes
How are non-hodgkin lymphomas classified?
Lineage (B or T cell) - B cell 90%
High vs Low grade
How do high vs low grade non-Hodgkin lymphomas differ?
Low grade - asymptomatic, responds to chemo but incurable
High grade - aggressive, combination therapy, curable
What are the commonest types of Non-Hodgkin Lymphoma?
Diffuse Large B-cell lymphoma (high grade) Follicular lymphoma (low grade)
How are the common non-hodgkin lymphomas treated?
(follicular watch and wait first)
Combination chemotherapy
Anti-CD20 monoclonal antibody
Hodgkin lymphoma is associated with what?
M:F 2:1
15-35y
Epstein-Barr virus
Familial/geographical
How is Hodgkin lymphoma treated?
Combination therapy Radiotherapy Monoclonal antibodies (anti-CD30) Immunotherapy PET scanning
How is Hodgkin lymphoma monitored?
PET scanning
ALL is specifically a disease of what?
Bone marrow B-lymphocytes (primarily)
Why are the majority of lymphomas B-cell type?
Issue arises in the germinal centre as the B-cell matures