Haematology Flashcards
What proportion of lymphomas are Hodgkin lymphomas?
15%
What proportion of lymphomas are non-Hodgkin lymphomas?
85%
What percentage of non-hodgkin lymphomas B cell?
> 90%
What is the peak incidence of Hodgkin’s lymphoma?
20-24
How do patients with lymphoma generally present?
Painless lymphadenopathy
Splenomegaly
Anaemia
B symptoms (pyrexia of unknown origin, night sweats, weight loss)
What are the investigations for a patient with suspected lymphoma?
History - symptoms, duration, B symptoms
Examination - lymph nodes, splenomegaly
Bloods - FBCs, U&Es, LFT, Ca
ESR
Imaging - CT scan, PET-CT
Bone marrow biopsy - aspirate and trephine
Additional tests: pulmonary function, ECHO
What staging system is used in Lymphoma?
Ann-Arbour Classification System
Define the Ann-Arbour Classification System for Lymphoma
Stage 1 = single lymph node group
Stage 2 = >1 lymph node group same side of diaphragm
Stage 3 = Lymph node groups both sides of diaphragm (includes spleen)
Stage 4 = Extranodal involvement
A/B to signify absence/presence of B symptoms
At what stage are most HL diagnosed?
Early stage
At what stage are most NHL diagnosed?
Advanced stage
Describe Follicular Lymphoma
Low grade lymphoma Median age of diagnosis 65 yrs Often present at Stage IV B symptoms are less common Indolent clinical cause Usually incurable Slow growth but reduced apoptosis
What cells do follicular lymphoma resemble?
Normal germinal centre cells
What translocation is associated with follicular lymphomas?
BCL2 gene (t14:18)
Describe the RRx for follicular lymphomas
Aim: relieving symptoms
If early stage - localised radiotherapy (may be curable)
Advanced stage:
Asymptomatic + no end organ compromise - watch and wait
Symptomatic/end organ compromise –> immunochemotherapy
Ritixumab (anti-CD20) + chemotherapy
Maintenance Ritixumab every 2/12 for 2 years
What is the survival of follicular lymphoma like?
Survival >15 yrs
Describe Diffuse Large B-cell Lymphoma
High grade lymphoma
Associated with various translocations and genetic abnormalities
High proliferation fraction, variable rate of cell death
What do the neoplastic cells in Diffuse Large B Cell Lymphoma resemble?
Activated B cells
Describe the presentation of Diffuse Large B cell Lymphoma
Lymphadenopathy - rapidly enlarging LN mass
Extra-nodal presentation is common (Waldeyer’s ring, GIT, Skin, Bones, CNS)
B symptoms
Describe the treatment in Diffuse Large B Cell Lymphoma
Aggressive chemotherapy with intention to cure
1A: R-CHOP x 3 + radiotherapy
Other stages: R-CHOP x 6
Describe R-CHOP chemotherapy
Rituximab Cyclophosphamide Adriamycin Vincristine Prednisolone
Describe Burkitt Lymphoma
High grade lymphoma
Very high rate of proliferation and high rate of apoptosis (tumour lysis syndrome –> release of K –> cardiac arrest)
What gene is involved in Burkitt Lymphomas?
MYC gene
Describe the presentation of Burkitt lymphoma
Short history
Marked B symptoms
Rapidly growing tumour with massive tumour bulk
Presents with extra nodal disease Jaw and facial bones Illeocaecal region of GIT Ovaries Kidneys Breasts
Describe the treatment for Burkitt Lymphoma
Requires intensive chemotherapy managed in ITU/HDU
Describe Classic Hodgkin Lymphoma
High grade lymphoma with prominent component of reactive cells
Approximately 40% of cases are associated with EBV infections
What do the neoplastic cells in Hodgkin lymphoma resemble?
Atypical activated B cells as seen in some viral infections
What is the characteristic pathology cell type seen in Hodgkin lymphoma?
Reed-Steinburg cells
What is strongly expressed in Hodgkin Lymphoma?
CD30
Describe the presentation of Hodgkin Lymphoma
Peak in 20s and elderly patients
Painless lymphadenopathy (may be sore with alcohol consumption)
Cough, SOB - mediastinal disease
Itch - may precede diagnosis by many months
How does Hodgkin lymphoma spread
Initially to immediately adjacent lymph nodes
Later, haematogenous spread to liver, lungs, bone marrow
How is early stage Hodgkin lymphoma treated?
Chemo + Radio
How is late stage Hodgkin Lymphoma treated?
Chemo
What Chemo regimen in used to treat Hodgkin lymphoma?
ABVD
Adriamycin
Bleomycin
Vanblastine
Dacarbazine
When should a person being treated for Hodgkin lymphoma have a PET-CT scan?
After 2nd cycle of chemo
Negative = scar tissue not active disease
Positive = change regimen
Describe plasma cell myeloma
Neoplasm of mature plasma cells with varied clinical cause
What do neoplastic cells in plasma cell myeloma resemble?
Normal plasma cells
Describe the presentation of plasma cell myeloma
Non-specific symptoms
Backache/rib pain Fatigue Symptoms associated with hypercalcaemia Recurrent infections Renal impairment
Describe paraprotein
In myeloma, abnormal plasma cells produce an abnormal monoclonal protein called paraprotein or M protein
5 different types (IgA etc)
What is IgM myeloma associated with?
Waldenstroms macroglobulinaemia
What is light chain myeloma?
Only part of the immunoglobulin is produced
What is non-secretory myeloma
No Ig is produced
What is the classical myeloma triad?
- Increased plasma cells in bone marrow
- Clonal immunoglobulin or paraprotein
- Lytic bone lesions
What blood tests should be arranged in suspected myeloma?
FBCs
ESR (>100)
U&E
Ca
Serum protein electrophoresis - rouleaux
SFLC quantity (serum free light chain)
What proteins may be present in the urine of a patient with myeloma?
Bence-Jones proteins (immunoglobulin light chains)
What is the diagnostic requirement for myeloma?
Neoplastic plasma cells in bone marrow >10% of total cells
AND >1 of:
HyperCalcaemia
Renal insufficiency
Anaemia
Bone lesions
What biomarkers are associated with myeloma?
Clonal plasm cell % >40%
Serum light free chain ratio >100
>1 focal lesion on MRI
Describe the treatment of myeloma
Asymptomatic = watch and wait
Symptomatic (CRAB criteria)
Chemotherapy + steroid + thalidomide
Radiotherapy - severe bone pain
Bisphosphonates
Blood transfusions/EPO
Why are bisphosphonates useful in patients with myeloma?
Reduces pain
Reduces pathological fractures
Reduces hypercalcaemia
Reduces need for radiotherapy
