Plasma cell disorders Flashcards
(24 cards)
Where are B cells produced and initially developed?
Bone marrow
What happens to B cells after they leave the bone marrow?
Travel to the follicle germinal centre of the lymph node
Identify antigen and improve the fitby somatic mutation by somatic mutation orbe deleted
May return to the marrow asplasma cell or cicrculate asmemory cell
What are plasma cells?
Factory cell
Pumps out antibody
What does a plasma cell nucleus look like?
Clock face
What are the 2 different types of increase in immunoglobulins?
Polycloncal
Monoclonal
What is a paraprotein?
monoclonal immunoglobulin - marker of underlying B-cell disorder
How are immunoglobulins detected?
Serum electrophoresis
How is Bence-Jones protein detected?
Urine electrophoresis
What are the firect tumour cell effects ofmyeloma?
Bone lesions Increased calcium Bone pain Replace normal bone marrow Marrow failure
What are the paraprotein mediated effects of myeloma?
Renal failure
Immune suppression
Hyperviscosity
Amyloid
How does lytic bone disease present on head x-ray?
Pepper pot skull (holes where bone has been eaten away by plasma cells in cell wall)
Which cells become overactive in myeloma lytic bone disease?
Osteoclasts
How can myeloma cause hypercalcaemia?
Calcium from bones is leaked into blood
How does myeloma affect the kidney?
Tubular cell damage by light chains
Light chain deposition - cast nephropathy
Hyppercalcaemia and Hyperuricaemia
NSAIDs
Which proteins to light chains bind with to form casts?
Tamm-Horsfall
How is myeloma treated?
Steroids
Alkylating agents
‘Novel agents’
High dose chemo & transplant in fit patients
How is response to treatment measured in myeloma?
Paraprotein level
What should not be given for bone pain in myeloma?
NSAIDs
What is MGUS?
Paraprotein <30g/l
Bone marrow plasma cells<10%
No evidence of myeloma end organ damage
What is AL Amyloidosis?
Rare disorder
Small plasma cell clone
Mutation in the light chain which precipitates i tissues asaninsoluble beta pleated sheet
How is amyloidosis diagnosed?
Congo red stain (apple-green birefringence)
Rectal or fat biopsy
Evidence in other organs (SAP scan, echo, proteinuria)
What is Waldenstrom’s macroglobulinaemia?
Rare cancer ofcell halfway between lymphocyteandplasma cell- produce Igm
How might Waldenstrom’s macroglobulinaemia present?
Lymphadenopathy
Splenomegaly
Hyperviscosity (visual disturbance, confusion,coma, bleeding)
Neuropathy
Treat ment ofWM?
Chemo
Plasmapheresis
