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Path - Plasma cell neoplasms + related disorders (i) (5) lymphoplasmacytic…
Path - Plasma cell neoplasms + related disorders (i)
Intro
dyscrasia
ancient Greek term
abnormal/disordered state of body, esp blood
e.g. neoplastic prolif of plasma cells
secrete monoclonal intact Ig (on cell surface, 2 heavy + 2 light chains) or Ig fragments (free light chain)
gammapathies
6 classifications
myeloid stem cells -> myeloblasts -> granulocytes -> lymphocytes
lymphokines = cks made by T cells
activate B cells to differentiate into...
plasma cells
secrete Igs from cell surface into blood
nucleus on periphery
clumped chromatin @ periphery
clock face appearance in nucleus
halo (pallor) in cytoplasm around nucleus
prominent golgi app (site of protein/Ig production)
memory cells
go back to BM
5 Ig types
A - alpha heavy chain
M - mu heavy chain
G - gamma heavy chain
D - delta heavy chain)
E - epsilon heavy chain
2 types of light chains (kappa or lambda)
2) smouldering multiple myeloma
assymptomatic
often seen as a multiple myeloma precursor (75% progress)
BM = 10-60% plasma cells
M protein > 3g/dL (abnormal ig fragment produced in excess)
3) solitary myeloma (plasmacytoma)
solitary lesions
osseous
single symptomatic area of bone destruction
patients usually younger than those with multiple myeloma
spine, pelvis, femur
progresses to multiple myeloma in 10-20 yrs
extra-osseous
soft tissue
lung, oronasopharynx, nasal sinuses
cured by local resection or radiotx
progression to multiple myeloma is rare
aka extra medullary
serum Ig level usually normally
4) Monoclonal gammopathy of undetermined significance
most common cause of monoclonal gammopathy
incidence = 3% when 50+, 5% when 70+
M spike (but < 3 g/dL)
BM < 10% plasma cells
asymptomatic
no Bence-Jones proteins or bone lesions or hypercalcaemia or amyloid
early stage multiple myeloma
same chromo aberrations as multiple myeloma, but generally benign
progression = 1% per year
5) lymphoplasmacytic lymphoma
affects elderly (60-70 y/o)
more common in males
neoplastic cells produce monoclonal IgM
monomer
pentameter (macroglobulin) = Waldenstrom's Macroglobulinaemia
hyperviscosity syndrome even more pronounced
mean survival = 4-5 yrs
no bone lesions
weakness
weightloss
lymphadenopathy
increased IgM in serum
M spike
autoimmune haemolysis of IgM coated RBCs
hyperviscosity syndrome
visual impairment
retinal veins distend + haemorrhage
neurological symptoms (headaches+dizziness)
due to sluggish flow
bleeding
macroglobulins interfere with clotting factors + platelet function
cryoglobulinaemia
precipitation of macroglobulins @ low temp
Raynaud's phenomenon
Tx = plasmapheresis
removal, treatment + return of plasma
6) Other rare entities
e.g. heavy chain disease (associated with lymphomas)