plasma cell disorders Flashcards
(16 cards)
what is multiple myeloma ?
malignancy of plasma cells
dependant on IL6 associated with an overproduction of IgG and IgA
also associated with an excess in light chains
what does an excess of light chains result in ?
renal damage
AL amyloidosis
what is seen in SPEP ?
M spike
what are the clinicl features associated with multiple myeloma ?
CRAB
hypercalcemia
renal insufficiency
anemia
bony lytic lesions
what is the mechanism associated with hypercalcemia in multiple myeloma ?
due to increased cytokine secretion ( IL1, TNF-alpha, RANK-L ) there is an increse in osteoclast activity
what are the urinary findings associated with mutliple myeloma ?
light IG chains ( bence jones)
what are the findings associated with BM biopsy in mutiple myeloma ?
monoclonal plasma cells with clock face chromatin and intracytoplasmic inclusions containing IgG
what is seen on skull x ray in MM ?
pepper pot skull
what is the pathology that happens that allows for the formation of bence jones proteins ?
light chains combine with tamm horsfall proteins and form obstructing cast
what is seen on PBS in multiple myeloma ?
rouleux formation due tot he RBCs sticking together
what is MGUS ?
similar to MM but a milder form where there is less than 10% of monoclonal plasma cells in the bone marrow , but has a high chance of progressing to MM
what is the treatment for multiple myeloma and mantle cell lymphoma ?
proteasome - which induces arrest in G2-M phase via accumulation of abnormal proteins resulting in apoptosis
what is waldenstorm macroglobulinemia ?
b cell lymphoma
they look like plasma cells but they are not
the tumor cells differentiate into plasma cells
what type of spike is seen in wanderstor ?
IgM spike
what is the presentation of wanderstorm macroglobulinemia ?
hyperviscosity syndrome
neurologiucal disorders
mucosal bleeding
blurry vision
what is the treatment for hyper viscosity syndrome ?
plasmapheresis
