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ChaPter 80  Monoclonal Gammopathies                 1093


             In HCDD, a population of clonal plasma cells produces both   Lancet Oncol 2014;15(12):e538–48. doi:10.1016/S1470-2045(14)70442-5.
           light and heavy monoclonal immunoglobulin chains  or only   PubMed PMID: 25439696.
           short (truncated) heavy chains that are unable to form amyloid   9.  Rajkumar SV. Overview of the management of multiple myeloma.
           fibrils but are deposited as granules in the tissues. HCDD can   UpToDate: Wolters Kluwer; 2016.
           be identified by immunofluorescence staining of biopsy tissue   10.  Palumbo A, Avet-Loiseau H, Oliva S, et al. Revised International Staging
                                                                    System for Multiple Myeloma: A Report From International Myeloma
           with anti–heavy chain antibodies. 26                     Working Group. J Clin Oncol 2015;33(26):2863–9. doi:10.1200/
           Management and Prognosis                                 JCO.2015.61.2267. PubMed PMID: 26240224, PubMed Central PMCID:
                                                                    PMC4846284.
           The therapeutic approaches for both LCDD and HCDD are similar   11.  Rajkumar SV. Smoldering multiple myeloma. UpToDate: Wolters Kluwer;
           to those employed for patients with MM. Serial free light chain   2016.
                                                            26
           assays can be used to monitor response to treatment in LCDD.    12.  Kristinsson SY, Holmberg E, Blimark C. Treatment for high-risk
           The prognoses of LCDD and HCDD vary considerably, depending   smoldering myeloma. N Engl J Med 2013;369(18):1762–3. doi:10.1056/
           on the nature, number, and extent of organ involvement. 26  NEJMc1310911#SA1. PubMed PMID: 24171527.
                                                                  13.  Rajkumar SV. Plasma cell leukemia. UpToDate: Wolters Kluwer; 2016.
                                                                  14.  Rajkumar SV. Diagnosis and management of solitary plasmacytoma of
               ON the hOriZON                                       bone. UpToDate: Wolters Kluwer; 2016.
                                                                  15.  Rajkumar SV. Diagnosis and management of solitary extramedullary
            •  Two monoclonal antibody drugs (mAb) have recently been approved   plasmacytoma. UpToDate: Wolters Kluwer; 2016.
              by the FDA for the treatment of multiple myeloma, with more on the
              way.                                                16.  Kyle RA, Rajkumar SV. Criteria for diagnosis, staging, risk stratification
            •  Caution is warranted when monitoring myeloma patients on mAb   and response assessment of multiple myeloma. Leukemia 2009;23(1):3–9.
              drugs, as these biologicals may cause false positive results by protein   doi:10.1038/leu.2008.291. PubMed PMID: 18971951, PubMed Central
              electrophoresis of the serum and immunofixation electrophoresis (IFE).  PMCID: PMC2627786.
                                                                  17.  Rajkumar SV. POEMS syndrome. UpToDate: Wolters Kluwer; 2016.
                                                                  18.  Rajkumar SV. Epidemiology, pathogenesis, clinical manifestations and
           Please check your eBook at https://expertconsult.inkling.com/   diagnosis of Waldenstrom macroglobulinemia. UpToDate: Wolters
                                                                    Kluwer; 2016.
           for self-assessment questions. See inside cover for registration   19.  Owen RG, Treon SP, Al-Katib A, et al. Clinicopathological definition of
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                                                                    from the Second International Workshop on Waldenstrom’s
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