Myeloma Flashcards
what is myeloma?
plasma cell malignancy
cascade to myeloma
MGUS (benign/ premalignant)
asymptomatic myeloma (malignant but no organ damage)
myeloma
how is myeloma classified?
according to type of antibody produced
median age at diagnosis of myeloma
65
direct tumour effects in myeloma
increased Ca2+, bone pain
bone marrow failure
lytic bone disease
what does lytic bone disease involve?
vertebral compression factures
spinal cord compression due to osteoclasts/osteoblasts
paraprotein effects in myeloma
renal failure
immune suppression
hyperviscosity
amyloid
how can myeloma affect the kidneys?
tubular cell damage by light chains
cast nephropathy (Tamm-Horsfall protein)
indirectly
how can myeloma indirectly affect the kidney
sepsis
hypercalcaemia + dehydration
NSAIDs used for pain
hyperuricaemia
diagnosis of myeloma
CRAB criteria
BJP in multiple myeloma
congo red staining
skull XR
CRAB criteria
high calcium
renal dysfunction
anaemia
bone disease
what is congo red staining used for?
amyloid deposition in myeloma
what is seen on skull XR in myeloma?
pepper pot skull/ lytic lesions (Rouleaux formation)
management of myeloma
- hydration
- stop nephrotoxic drug
- chemotherapy, steroids, alkylating agents, monoclonal Ab, stem cell transplant
- Symptom control= opiates (avoid NSAIDs), local radiotherapy, bisphosphonates, vertebroplasty
- thialiomide
what is MGUS?
monoclonal gammopathy of undetermined significance
precursor to myeloma
diagnosis of MGUS
paraprotein <30g/l with <10% marrow plasma cells
presentation of MGUS
no evidence of end organ damage
what is AL amyloidosis?
mutated light chains that can precipitate in tissues to amyloid fibrils (insoluble beta-pleated sheets)
where can the amyloid fibrils accumulate?
end organs causing damage
organ damage caused by amyloid fibrils
nephrotic syndrome cardiomyopathy organomegaly, deranged LFTs autonomic and peripheral neuropathy malabsorption
diagnosis of AL amyloidosis
biopsy with congo red stain
SAP scan
echo/ cardiac MRI
urinalysis
management of AL amyloidosis?
chemotherapy
what is Waldenstrom’s macroglobulinaemia?
lymphoplasmacytoid neoplasm of the IgM paraprotein
clonal disorder of cells intermediate between lymphocytes and plasma cells
presentation of Waldenstrom’s macroglobulinaemia
lymphadenopathy splenomegaly marrow failure hyper-viscosity syndrome B symptoms neuropathy
presentation of hyper-viscosity syndrome
fatigue visual disturbance confusion coma bleeding cardiac failure
triad= bleeding, visual change, neurological
management of Waldenstrom’s macroglobulinaemia
chemotherapy
plasmapheresis
management of hyperviscosity syndrome
remove plasma rich in IgM and replace with donor plasma