Myeloma Flashcards
What is the role of B cells?
adaptive immune system:
antibody production
antigen presenting cells
What is the Ig region of the B cell derived from
V-D-J region recombination
When immature B cells with Ig on their surface exit the bone marrow, where do they go?
follicle germinal center of the lymph node
When B cells return to the bone marrow from the follicle germinal center of the lymph node, what are they?
plasma cell
memory B cell
What are the features of plasma cells?
open chromatin for synthesising mRNA
blue cytoplasm
pale perinuclear area
What are causes of polyclonal increases in Igs?
infection
autoimmune
malignancy
liver disease
What are causes of monoclonal increases in Igs?
paraprotein
B cell disorder
How are immunoglobulins detected?
electrophoresis
albumin = +ve
gamma = -ve
What are Bence Jones protiens?
monoclonal Ig free light chain found in the urine
What causes Bence Jones proteins?
monoclonal increase in the amount of free light chains in proportion to heavy chains in the plasma meaning that they are secreted
How are Bence Jones proteins detected?
urine electrophoresis
What is paraproteinaemia?
proliferation of immunoglobulin producing cells
What is the presentation of Myeloma?
bone lesions increased calcium - stones, bones, groans, psychiatric moans bone pain - increased osteoclast, decreased osteoblast activity bone marrow failure paraprotein mediated affects: renal failure immunosuppression amyloid hyperviscosity
How is myeloma classified?
type of antibody produced
most common - IgG, IgA, Bence Jones
How does myeloma cause kidney impairement?
light chains cause tubular cell damage - cast nephropathy
How is response to myeloma treatment measured?
paraprotein
What is the median age at diagnosis of myeloma?
65
How are fit patients with myeloma treated?
high dose chemo
autologous HSC transplant
What is Monoclonal gammopathy of undetermined significance (MGUS)?
paraprotein <30g/L
bone marrow plasma cells <10%
What is AL amyloidosis?
mutation in light chain leading to altered structure
precipitates in tissues as insoluble beta pleated sheets and causes organ damage (kidneys, heart, liver)
How is AL amyloidosis treated?
chemo
How is AL amyloidosis diagnosed?
organ biopsy - stains congo red (apple green bifringent under polarised light)
SAP scan - identifies amyloid deposits
ECHO
nephrotic range proteinuria
What is Waldenstoms macroglobulinaemia?
IgM paraprotein
lymphoplasmacytoid neoplasm - clonal disorder of cells intermediate between a lymphocyte and plasma
How does Waldenstoms macroglobulinaemia present?
hyperviscosity - bleeding, cardiac failure, fatigue, visual disturbance, coma
B symtoms - night sweats, weight loss
How is Waldenstoms macroglobulinaemia treated?
chemo
plasmapheresis
