Immunoglobulin free light chains: Do they have a role in plasma cell leukemia?
The assay of serum free light chains (FLCs) is established in the diagnosis and prognosis of several plasma cell dyscrasias, but its significance in plasma cell leukemia (PCL) has not been reported so far. PCL is a rare and aggressive disease with a poor prognosis. The authors describe two cases of PCL with divergent clinical profiles where the serum FLC assay was available. Although both patients had greatly elevated serum beta(2)-microglobulin, one patient had much higher levels of serum FLCs and evidence of renal impairment, which were absent in the other; however, the latter, who had multisystem involvement, showed a more rapidly downhill course with early mortality.
Available from: Manickam Niraimathi
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ABSTRACT: Plasma cell leukemia (PCL) represents a rare and aggressive form of plasma cell dyscrasia which can be primary (pPCL) or secondary (sPCL). It is diagnosed based on absolute plasma cell count of more than 2.0 × 10(9)/l or a relative proportion of greater than 20% of the peripheral blood leukocyte count. Although pPCL and sPCL share several clinical features, important differences exist. Patients with pPCL are younger; often have extra osseous organ involvement (liver, spleen and other extramedullary sites), increased frequency of renal failure, fast declining performance status and rapid progression to the terminal stage. Patients with sPCL have advanced bone disease. Presented in this article is India data of a short series of five cases of PCL diagnosed at a tertiary care centre from south India over last 5 years. All cases were de novo and had varied spectrum of presentation and so were not suspected to be plasma cell dyscrasia clinically. Detailed hemato-pathological evaluation clinched the diagnosis in all the cases.
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