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      Immunoglobulin D Multiple Myeloma, Plasma Cell Leukemia and Chronic Myelogenous Leukemia in a Single Patient Treated Simultaneously with Lenalidomide, Bortezomib, Dexamethasone and Imatinib

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          Abstract

          Multiple myeloma (MM) is a neoplastic lymphoproliferative disorder characterized by uncontrolled monoclonal plasma cell proliferation. Among different isotypes of MM, immunoglobulin D (IgD) MM is very rare, representing only 1 to 2% of all isotypes. Chronic myelogenous leukemia (CML) is a neoplastic myeloproliferative disorder of pluripotent hematopoietic stem cell, which is characterized by the uncontrolled proliferation of myeloid cells. An 88-year-old male was diagnosed simultaneously with IgD kappa MM and CML. A distinctive feature in this patient was the progression to plasma cell leukemia without any symptomatic myeloma stage. He was treated simultaneously with lenalidomide, bortezomib and imatinib. Synchronous occurrence of these rare hematological malignancies in a single patient is an exceedingly rare event. Multiple hypotheses to explain co-occurrence of CML and MM have been proposed; however, the exact etiological molecular pathophysiology remains elusive.

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          Most cited references15

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          Chronic myeloid leukemia.

          C Sawyers (1999)
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            Chronic myeloid leukemia--advances in biology and new approaches to treatment.

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              Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group.

              Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. The diagnosis is based upon the percentage (≥ 20%) and absolute number (≥ 2 × 10(9)/l) of plasma cells in the peripheral blood. It is proposed that the thresholds for diagnosis be re-examined and consensus recommendations are made for diagnosis, as well as, response and progression criteria. Induction therapy needs to begin promptly and have high clinical activity leading to rapid disease control in an effort to minimize the risk of early death. Intensive chemotherapy regimens and bortezomib-based regimens are recommended followed by high-dose therapy with autologous stem cell transplantation if feasible. Allogeneic transplantation can be considered in younger patients. Prospective multicenter studies are required to provide revised definitions and better understanding of the pathogenesis of PCL.
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                Author and article information

                Journal
                Hematol Rep
                Hematol Rep
                HR
                Hematology Reports
                PAGEPress Publications, Pavia, Italy
                2038-8322
                2038-8330
                17 March 2016
                17 March 2016
                : 8
                : 1
                : 6295
                Affiliations
                [1 ]Departments of Internal Medicine, Abington Memorial Hospital/Abington-Jefferson Health , PA, USA
                [2 ]Departments of Hematology and Oncology, Abington Memorial Hospital/Abington-Jefferson Health , PA, USA
                [3 ]Departments of Pathology, Abington Memorial Hospital/Abington-Jefferson Health , PA, USA
                Author notes
                Abington Memorial Hospital/Abington-Jefferson Health, Department of Internal Medicine, 1200 Old York Road, Abington, 19001 PA, USA. +1.267.471.0165 - +1.215.481.4361. nali@ 123456abingtonhealth.org

                Contributions: the authors contributed equally.

                Conflict of interest: the authors declare no potential conflict of interest.

                Article
                10.4081/hr.2016.6295
                4815947
                27103978
                565b552f-f200-4e6a-8eb2-b099b2a594d1
                ©Copyright N. Ali et al.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 04 November 2015
                : 19 January 2016
                : 19 January 2016
                Page count
                Figures: 1, Tables: 2, Equations: 0, References: 20, Pages: 5
                Categories
                Case Report

                Hematology
                immunoglobulin d multiple myeloma,plasma cell leukemia,chronic myelogenous leukemia,bortezomib,imatinib

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