Paraproteinemia(multiple Myeloma Flashcards
Another name for paraproteins
Monoclonal immunoglobulin and M-protein
What are paraproteins
Paraproteins are immunoglobulins that are produced from a single clone of plasma cells
Plasma cells produces?
They produce immunoglobulins
What are B cells
They are a type of white cells that produces plasma cells
What is paraproteinaemia(2 definitions )
1)This is the presence of excessive amount of paraproteins in the plasma
2) This can be defined as an heterogeneous disorder that is characterized by two things
1)proliferation of a single clone of plasma cells
2.presence of excessive amounts of monoclonal protein and its fragments in blood
What is the cause of paraproteinaemia
It is usually due to clonal transformation of B cells
What are the fragments of monoclonal immunoglobulin
Light chains and heavy chains
Diseases associated with paraproteinaemia (6)
1.smouldering myeloma
2.multiple myeloma
3.monoclonal gammopathy of undetermined significance (MGUS)
4.plasma cell leukemia
5.Heavy chain disease
6.waldenstroms macroglobulinaemia
Paraproteinaemia can be categorized based on monoclonal protein/fragment found in blood
List the monoclonal fragments and the disease they are associated with
1.light chains only(Bence jones protein)-multiple myeloma and AL amyloidosis(light chains amyloidosis)
2.Heavy chains only(Heavy chains disease )
3.whole immunoglobulins (M protein)
Abnormal plasma cell variants (list them)
Mott cell
Flame cells
Describe the cytoplasm of Mott cell
Classic “bunch of grapes appearance with ig inclusions(Russell bodies)
Mott cell is usually seen in
Plasma dyscrasia (Both the symptomatic and the asymptomatic
Reactive plasmacytoses( There is an increase in the proliferation of plasma cells and it is usually seen in auto immune conditions.e.g Haishimoto syndrome
Describe the the flame cell cytoplasm
It resembles red flames
It is commonly seen in IgA myeloma then in IgG and in IgM
Immunophenotypes of malignant plasma cells
1.CD38
2.CD138 positive
The most common chromosomal abnormality seen in paraproteinaemia
Chromosome 14 t(11:14)
The differentials seen in chromosome 14
t(11:14)
t(4:14)
t(14:16)
The two chromosomal abnormality and which one has a poor prognosis
Chromosome 13 and chromosome 14
Chromosome 13 has a poor prognosis
Roles of cytokines in multiple myeloma
Interleukin 6 is an important mediator in inflammatory response
It is produced after the release of of IL-1 and TNF(tumor necrosis factor)
It is a potent growth factor of myeloma cells
Elevated levels at the time of diagnosis is associated with aggressive disease and poor prognosis
IL-6,IL-1 and TNF are osteoclasts activating factors and mediate osteolytic bone lesions
