Lymphoid Malignancy Flashcards
What is the general presentation of lymphoid malignancies?
Enlarged lymph nodes (lymphadenopathy) Extranodal involvement Bone marrow involvement Weight loss Fever Night sweats Pruritis Fatigue
What is acute lymphoid leukaemia?
Neoplastic disorder of lymphoblasts
Diagnosed by >20% lymphoblasts present in bone marrow
How does ALL typically present?
2-3 week history of bone marrow failure or bone/joint pain
Anaemia
Infection
Possible raised WCC
What is the treatment for ALL?
Induction chemotherapy to obtain remission Consolidation therapy CNS directed treatment Maintenance treatment for 18 months Stem cell transplantation
What are the risk factors for ALL poor outcome?
Over 60 White cell count >300x10^9/L Immunophenotype Cytogenetics/ molecular genetics Slow/poor response to treatment
How is a diagnosis of chronic lymphoid leukaemia (CLL) made?
Blood>5x10^9/L lymphocytes
Bone marrow >30% lymphocytes
Characteristic immunophenotyping (B cell markers and CD5 positive)
What is the presentation of CLL?
Either asymptomatic or gradual onset Lymphadenopathy Bone marrow failure (anaemia, thrombocytopenia) Splenomegaly Fever and sweats Hepatomegaly Infections Weight loss
What are the associated findings of CLL?
Immune paresis (loss of normal immunoglobulin production)
Paraprotein band
Haemolytic anaemia
What is the staging for CLL?
Stage A - <3 lymph node areas
Stage B - 3 or more lymph node areas
Stage C - Stage B + anaemia or thrombocytopenia
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 cytopenia
What is the treatment for CLL?
None/supportive care/intravenous immunoglobulins
Cytotoxic chemotherapy e.g. fludarabine, bendamustine
Monoclonal antibodies e.g. Rituximab, obinutuzamab
Corticosteroids
Radiotherapy
Novel agents
What are the poor prognostic markers for CLL?
Advanced disease (Binet stage B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time (<12 mth) CD 38+ expression Loss/mutation p53; del 11q23 (ATM gene) Unmutated IgVH gene status
What is the general presentation of a lymphoma?
Lymphadenopathy/ hepatosplenomegaly
Extranodal disease
B symptoms
Bone marrow involvement
How are lymphomas assessed/staged?
Lymph node biopsy
CT scan
Bone marrow aspirate and trephine
What is Hodgkin lymphoma associated with?
Epstein barr virus
Familial and geographical clustering
What is the treatment for Hodgkin lymphoma?
Combination chemotherapy (ABVD)
Radiotherapy
Use of PET scan to assess response to treatment and to limit use of radiotherapy
How are non-Hodgkin lymphomas classified?
Lineage (B cell or T cell)
Grade of disease
Histological features of disease
What are the features of a low grade non-Hodgkin lymphoma?
Indolent, often asymptomatic
Responds to chemotherapy but incurable
Median survival varies by subtype
What are the features of a high-grade non-Hodgkin lymphoma?
Aggressive, fast-growing
Require combination chemotherapy
Can be cured, but varies widely
What are the most common subtypes of Non-Hodgkin lymphoma?
Diffuse large B-cell lymphoma (High grade) Follicular lymphoma (low grade)
What are monoclonal bands (paraproteins)?
Represent immunoglobulin production by a single clone of cells
Detectable in either serum or urine
What is a myeloma?
Neoplastic disorder of plasma cells, resulting in excessive production of a single type of immunoglobulin
What are the features of myeloma?
Bone disease (lytic bone lesions, cord compression, hypercalcaemia)
Renal failure
Bone marrow failure esp anaemia
Infections
What is the treatment for myeloma?
Chemotherapy (cyclophosphamide, thalidomide and dexamethasone, proteasome ihibitors, IMiDs, monoclonal antibodies) Bisphosphonate therapy (zoledronic acid) Radiotherapy Surgery Autologous stem cell transplant
What are the stages of myeloma?
Stage 1 - serum beta 2 microglobulin <3.5, albumin >35
Stage 2 - not stage 1 or 3
Stage 3 - serum beta 2 microglobulin >5.5
What is the staging for lymphoma?
Stage 1 one region
Stage 2 two regions on the same side of diaphragm
Stage 3 above and below diaphragm
Stage 4 extranodal disease
What is the treatment fro non-Hodgkin lymphoma?
Combination chemotherapy (Anti CD20 monoclonal antibody and chemotherapy)
