Haematology Flashcards
What are the 4 types of lymphoma?
Acute myeloid leukamia
Chronic myeloid leukamia
Acute lymphoblastic leukamia
Chronic lymphocytic leukamia
Talk about multiple myeloma.
Cancer of differentiated B-lymphocytes, known as plasma cells, featuring:
> The accumulation of malignant plasma cells in the bone marrow leading to progressive bone marrow failure
> The production of a characteristic paraprotein
Kidney failure
> Destructive bone disease and hypercalcaemia
Talk about spikey “CRAB” in multiple myeloma.
hyperCalcemia
Renal disease
Anaemia
lytic Bone disease
C - ad Ca>0.25mmol/l above upper limit of normal or >2.75mmol/l
R – renal impairment, creatinine>173mmol/l (40% pts at Δ have some degree of RF; 10% req dialysis Dimopolous et al, 2008, Leukaemia, 22, 1485-1493 Pathogenesis and treatment of renal failiure in multiple myeloma)
A – anaemia 2g <NR or <10g/dl (75% at diagnosis; normochromic, normocytic, Kyle et al 2003)
B – lytic lesions, osteoporosis c compression #, spinal cord compression
nb also hyperviscosity; recurrent infection ie >2 episodes in 12/12 , amyloid; raised ESR and globulin frequent signals
Why is myeloma always incurable?
The problem - myeloma remains almost always incurable; it is a remitting, relapsing condition which inexorably progresses to refractory disease and death
Talk about systemic AL amyloidosis.
Systemic AL amyloidosis, is a protein conformation disorder associated with a subtle clonal plasma cell dyscrasia
Multiple organ disease resulting from the extracellular deposition of monoclonal immunoglobulin light chain fragments
Amyloid fibrils associate in vivo with the normal plasma protein serum amyloid P component (SAP), basis for the use of SAP scanning
Presents with nephrotic syndrome +/- renal impairment (33%), congestive cardiomyopathy (20%), sensorimotor and/or autonomic neuropathy (20%) often with GI disturbance and hepatomegaly; rarely, bleeding nb Raccoon eyes and macroglossia
Natural history – progressive and fatal within 2yrs if untreated
Treat plasma cell dyscrasia similar regimens to myeloma
What biopsy test can be done for myeloma?
Bone marrow biopsy
Talk about Immunoglobulin product aka paraprotein, m-spike.
Myeloma cells usually produce both an intact immunoglobulin product - IgG (2/3), IgA (1/3) or rarely IgD (1.8%), IgM (0.4%) or IgE and monoclonal free light chains (90% of cases); in a minority of cases, myeloma cells produce FLC only (10%) termed ‘free light chain myeloma’
What are the other test that can be done to test myeloma?
Serum electrophoresis (SEP)
If theres polyclonal antibody - we will see smear
if theres single AB- we will see band
What are the skeletal related events in patients with multiple myeloma.
kyphoisis, walking impairment, permanent deformity, paralysis, bone pain, fatigue
What are the treatment aims in multiple myeloma?
- Reduce number of myeloma cells
- Reduce symptoms and complications (protects body organs and tissues)
- improve quality and length of life
What are the supportive treatment in patients with multiple myeloma?
Bisphosphonate to protect bones
Blood transfusions/EPO injections
Antibiotics as needed
Pain-killers as required
Radiotherapy
Kyphoplasty occasionally indicated
Psychological support
What are the types of anti-myeloma chemotherapies?
Monoclonal antibodies – daratumumab (anti CD38)
Proteasome inhibitors – bortezomib (sc), carfilzomib (iv), ixazomib (po)
Immunomodulatory drugs – thalidomide and analogues lenalidomide (Revlimid) and pomalidomide
Corticosteroids – dexamethasone, prednisolone
Alkylating agents – cyclophosphamide, melphalan
Quadruplet, triplet and doublet combinations commonly used including
