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Karthik Bommannan,Man Updesh Singh Sachdeva,Pankaj Malhotra,Narender Kumar,Prashant Sharma,Shano Naseem,Jasmina Ahluwalia,Reena Das,Neelam Varma,Gaurav Prakash,Alka Khadwal,Radhika Srinivasan,Subhash 대한혈액학회 2016 Blood Research Vol.51 No.1
BackgroundPlasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×109/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma.MethodsClinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.ResultsBetween January 2007 and December 2014, ten PPCL and four SPCL patients were inves-tigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months.ConclusionWe highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to un-mask possible significant effects on pathogenesis.
Praveen Sharma,Man Updesh Singh Sachdeva,Narender Kumar,Sunil Bose,Parveen Bose,Varun Uppal,Pankaj Malhotra,Deepak Bansal,Neelam Varma,Jasmina Ahluwalia 대한혈액학회 2021 Blood Research Vol.56 No.2
Background Platelet aggregation studies using conventional light transmission aggregometry (LTA) have several disadvantages and require strict pre-analytical measures for reliable results. We aimed to examine the utility of flow cytometric platelet aggregation (FCA) assay in detecting platelet function defects (PFDs) in patients with a history of bleeding symptoms. Methods Sixty-four participants (24 patients and 40 healthy controls) were included in this study. LTA and FCA assay were performed simultaneously in patients and healthy controls. In the FCA assay, two portions of platelets from the same individual were labeled separately with CD31-FITC and CD31-PE. After mixing and stimulation with agonists, the double- colored platelet aggregates were visualized using a flow cytometer. The results generated using the two techniques were compared and correlated. Results The patients’ median age was 17 years (range, 3‒72 yr) with a male-to-female ratio of 1:1.7. There was substantial agreement between LTA and FCA assay in detecting a PFD (=0.792). Four patients showing a Glanzmann thrombasthenia-like pattern on LTA exhibited an abnormal FCA. A functional defect in collagen binding was detected on the FCA assay conducted in two immune thrombocytopenic patients with severe bleeding. Conclusion FCA assay can be used to identify functional defects in platelets, with potential applications in thrombocytopenic individuals. It also facilitates the diagnosis of inherited bleeding disorders with platelet defects.
Karthik Bommannan,Man Updesh Singh Sachdeva,Pankaj Malhotra,Narender Kumar,Prashant Sharma,Shano Naseem,Jasmina Ahluwalia,Reena Das,Neelam Varma,Gaurav Prakash,Alka Khadwal,Radhika Srinivasan,Subhash 대한혈액학회 2016 Blood Research Vol.51 No.1
BackgroundPlasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×109/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma.MethodsClinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively.ResultsBetween January 2007 and December 2014, ten PPCL and four SPCL patients were inves-tigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months.ConclusionWe highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to un-mask possible significant effects on pathogenesis.