Plasma Cell Disorders Flashcards
Multiple myeloma is a haematological malignancy characterised by proliferation of what?
Plasma cells
Which type of immunoglobulin is a dimer?
IgA
Which type of immunoglobulin is a pentamer?
IgM
What is the name for a monoclonal immunoglobulin, which can be used as a marker of underlying clonal B cell disorders?
Paraprotein
Excess free light chains leaking into the urine are known as what?
Bence-Jones proteins
What investigation is used to detect abnormal paraproteins in the serum or urine?
Electrophoresis
What is the median age at presentation of myeloma?
70 years
What electrolyte abnormality is most associated with myeloma?
Hypercalcaemia
Predominantly, why does renal impairment occur in myeloma?
Deposition of light chains in the renal tubules
Bone marrow crowding with plasma cells leads to what complications of myeloma?
Anaemia, thrombocytopenia, neutropenia
What bony features are often present in patients with myeloma?
Back pain, lytic bone lesions, pathological fractures
What type of anaemia occurs in individuals with myeloma?
Normocytic, normochromic
What will be seen on an FBC of someone with myeloma?
Anaemia, thrombocytopenia, possibly neutropenia
What will be seen on U&Es of someone with myeloma?
Raised urea and creatinine
What will a blood film show in myeloma?
Rouleaux formation
What are the most important screening investigations for myeloma?
Serum and urine electrophoresis
In myeloma, raised concentrations of which monoclonal proteins will be present in the serum?
IgA and IgG
What is the classic x-ray finding in individuals with myeloma?
Lytic ‘punched out’ lesions (pepper pot skull)
How is myeloma definitively diagnosed?
Bone marrow biopsy showing > 10% plasma cells
What investigation is used to survey the whole skeleton for bone lesions in patients with myeloma?
Whole body MRI
The ability to undergo what treatment is used to decide on what induction therapy is used for patients with myeloma?
Autologous stem cell transplant
What induction therapy is used for patients with myeloma who are suitable for an autologous stem cell transplant?
Bortezomib and dexamethasone
What induction therapy is used for patients with myeloma who are not suitable for an autologous stem cell transplant?
An alkylating agents (e.g. cyclophosphamide), thalidomide and dexamethasone
How are myeloma patients monitored after completion of induction treatment?
Blood tests and electrophoresis every 3 months
What is the first line treatment for relapsed myeloma?
Bortezomib monotherapy
What is used to monitor response to treatment in patients with myeloma?
Paraprotein levels
What medication is given to all myeloma patients to manage osteoporosis and fragility fractures?
Zoledronic acid
How is spinal cord compression as a result of myeloma treated?
Dexamethasone and radiotherapy
What is the chance of a patient with MGUS developing myeloma at 10 years?
10%
Around 10-30% of patients with MGUS have what developing complication?
Demyelinating neuropathy
In MGUS, levels of paraproteinaemia will be less than what?
30g/L
In MGUS, bone marrow plasma cell concentration will be less than what?
10%
Who is Waldenstrom’s macroglobulinaemia typically seen in?
Older men
Waldenstrom’s macroglobulinaemia is a lymphoplasmacytoid malignancy characterised by the secretion of what monoclonal paraprotein?
IgM
Waldenstrom’s macroglobulinaemia typically causes hyperviscosity syndrome- what is the most common feature of this?
Visual disturbance
What treatment can be used to manage hyperviscosity that occurs in Waldenstrom’s macroglobulinaemia?
Plasmapheresis
