Plasma cell dyscrasias

Plasma cell dyscrasias are a heterogeneous group of disorders caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. Currently, there are several subtypes of PCDs including

(i) monoclonal gammopathy of undetermined significance (MGUS)-serum M protein of less than 30 g/L with fewer than 10% PCs in the bone marrow and no evidence of bone or organ damage.

(ii) asymptomatic myeloma- M protein concentration of greater than 30 g/L, with more than 10% PCs in the bone marrow, but with no related tissue or end organ damage or clinical sequelae such as hypercalcemia, renal failure, anemia, and bone lesions

(iii) multiple myeloma-M protein concentration of >30 g/L, greater than 10% PCs in the bone marrow, and evidence of organ and tissue damage

(iv) PC leukemia-PC leukemia is similar to multiple myeloma but differs in presentation due to the presence of PCs in the peripheral blood circulation.

(v) plasmacytoma-Plasmacytoma is a discrete solid PC tumor found either in the bone (solitary bone plasmacytoma) or soft tissues (extramedullary plasmacytoma)

(vi) amyloidosis-Primary amyloidosis is a rare disorder caused by a PC which produces intact immunoglobulins or most commonly immunoglobulin light chain fragments and rarely heavy chain fragments.

Immunophenotyping:

 light chain restricted, CD38 and CD138 positive, CD45 negative or dim, with the majority being CD56+, CD117+, and CD19−

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Figure shows CD45- population which is positive for CD38 , CD138, CD117, Cd13 and cL while negative for CD3, cCD22, CD19, CD56, CD10, CD33, HLA-DR, cK.

References

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