Haem - Myeloma, Lymphoma, DIC Flashcards
Myeloma - what is it?
Haematological malignancy that arises due to plasma cell proliferation
Cancer of the plasma cells - type of B lymphocyte that produce antibodies
Myeloma - what is multiple myeloma?
Is when the myeloma affects multiple areas of the body
Myeloma - pathophysiology?
- Plasma cells are B cells (B lymphocytes) of the immune system that have become activated to produce a certain antibody
- Called B cells because they are found in the bone marrow
- Myeloma is a cancer of a specific type of plasma cell where there is a genetic mutation causing it to rapidly and uncontrollably multiply
- These plasma cells produce one type of antibody, and antibodies are also called immunoglobulins
- They are complex molecules made up of two heavy chains and two light chains arranged in a Y shape
- Help the immune system recognise and fight infections by targeting specific proteins on the pathogen
- They come in 5 main types: A, G, M, D and E
- When you measure immunoglobulins in a patient with myeloma, one of those types will be significantly abundant
- More than 50% of the time this is immunoglobulin type G (IgG)
- This single type of antibody that is produced by all the identical cancerous plasma cells can be called a monoclonal paraprotein
- This means a single type of abnormal protein
The “Bence Jones protein” that can be found in the urine of many patients with myeloma is actually a part (subunit) of the antibody called the light chains
Myeloma - what is the median age at presentation?
70 years old
Myeloma - what are the clinical features?
CRABBI
Calcium (elevated) - due to increased osteoclast activity
Renal failure - due to light chain deposition within renal tubules
Anaemia (normocytic, normochromic) - bone marrow crowding suppresses erythropoiesis
Bone pain/lesions - bone marrow infiltration by plasma cells
Bleeding - bone marrow crowding also results in thrombocytopenia, so increased risk of bleeding/bruising
Infection - reduction in the production of normal immunoglobulins results in increased susceptibility to infection
Myeloma - what area does bone pain commonly present at?
Presents as back pain commonly
Myeloma - what are the blood results on investigation?
Bloods:
FBC - show anaemia and thrombocytopenia (because of A and B in CRABBI)
U&E - raised urea and creatinine (because of renal failure)
Raised calcium
Myeloma - what are the other investigations you can do, and what are their results?
- Serum or urine protein electrophoresis: raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
- In the urine, they are known as Bence Jones proteins
- Bone marrow aspiration and trephine biopsy: confirms the diagnosis if the number of plasma cells is significantly raised
- Whole-body MRI (or CT if MRI is not suitable) is used to survey the skeleton for bone lesions
Myeloma - what is a common XRAY finding?
‘Rain-drop’ skull
Numerous randomly placed dark spots seen on X-ray which occur due to bone lysis
‘Raindrops keep falling on MYeloma head’ - way to remember finding
Myeloma - what three factors is symptomatic multiple myeloma defined at diagnosis by?
Symptomatic multiple myeloma is defined at diagnosis by the presence of the following three factors:
- Monoclonal plasma cells in the bone marrow >10%
- Monoclonal protein within the serum or the urine (as determined by electrophoresis)
- Evidence of end-organ damage e.g. hypercalcaemia, elevated creatinine, anaemia or lytic bone lesions/fractures
Myeloma - management?
No Cure
Initial treatment:
- If suitable for autologous stem cell transplant (ASCT) - Bortezomib + Dexamethasone
- If UNsuitable for ASCT - Thalidomide + an Alkylating agent + Dexamethasone
Myeloma - what do you give if the patient relapses after initial treatment?
Bortezomib monotherapy
Myeloma - what are the complications and how do you manage them?
- Pain: treat with analgesia (using the WHO analgesic ladder)
- Pathological fracture: Zoledronic acid is given to prevent and manage osteoporosis and fragility fractures
- Infection: patients receive annual influenza vaccinations, may also receive Immunoglobulin replacement therapy
Lymphoma - what is it?
- Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system
- These cancerous cells proliferate within the lymph nodes
- Cause lymph nodes to become abnormally large (lymphadenopathy)
Lymphoma - what are the definitions of Hodgkin’s lymphoma and non-Hodgkin’s lymphoma?
Hodgkin’s lymphoma is a specific disease
Non-Hodgkins lymphoma encompasses all the other lymphomas
Hodgkin’s Lymphoma - what is it caused by?
Caused by the proliferation of lymphocytes
Hodgkin’s Lymphoma - what is the age distribution?
Bimodal age distribution
20 years old
75 years old