Abstract
Plasma cell leukemia represents a unique subset of patients with multiple myeloma. The operational definition requires that the patient have a blood leukocyte count >10,000 and at least 2000 circulating plasma cells. This also must constitute 20% of circulating cells. Plasma cell leukemia is classified as primary when the initial diagnosis is with circulating cells and secondary when it occurs at the time of relapse. Extramedullary plasmacytomas can be either outside of the cortical bone or extension from the cortical bone and represent a high-risk form of multiple myeloma. This book chapter deals with the natural history, biology, clinical outcomes, and therapy of these two uncommon manifestations of multiple myeloma.
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References
Agnelli L, Musto P, Todoerti K et al (2012) Analysis of transcriptome, mirnome and genomic profiles in association with clinical outcome in a prospective series of primary plasma cell leukemia. Blood 120(21):3938
Ahn JS, Jung SH, Yang DH et al (2014) Patterns of relapse or progression after bortezomib-based salvage therapy in patients with relapsed/refractory multiple myeloma. Clin Lymphoma Myeloma Leuk 14(5):389–394. https://doi.org/10.1016/j.clml.2014.02.004
An G, Qin X, Acharya C et al (2015) Multiple myeloma patients with low proportion of circulating plasma cells had similar survival with primary plasma cell leukemia patients. Ann Hematol 94(2):257–264. https://doi.org/10.1007/s00277-014-2211-0
Anagnostopoulos A, Gika D, Hamilos G et al (2004) Treatment of relapse refractory multiple myeloma with thalidomide-based regimens: identification of prognostic factors. Leuk Lymphoma 45:2275–2279
Avet-Loiseau H, Daviet A, Brigaudeau C et al (2001) Cytogenetic, interphase, and multicolor fluorescence in situ hybridization analyses in primary plasma cell leukemia: a study of 40 patients at diagnosis, on behalf of the Intergroupe Francophone du Myelome and the Groupe Francais de Cytogenetique Hematologique. Blood 97(3):822–825
Avigdor A, Raanani P, Levi I et al (2001) Extramedullary progression despite a good response in the bone marrow in patients treated with thalidomide for multiple myeloma. Leuk Lymphoma 42:683–687
Barbieri M, Mosca L, Musto P et al (2012) Genome-wide approach identify recurrent 8p21.2 loss in more aggressive form of primary plasma-cell leukemia. Haematologica 97:S46
Barlogie B, Anaissie E, Rhee F et al (2007) The Arkansas approach to therapy in patients with multiple myeloma. Best Pract Res 20:761–781
Bezieau S, Devilder MC, Avet-Loiseau H et al (2001) High incidence of N and K-Ras activating mutations in multiple myeloma and primary plasma cell leukemia at diagnosis. Hum Mutat 18(3):212–224
Blade J, Kyle RA (1999) Nonsecretory myeloma, immunoglobulin D myeloma, and plasma cell leukemia. Hematol Oncol Clin North Am 13(6):1259–1272
Bladé J, Rosiñol L (2007) Complications of multiple myeloma. Hematol Oncol Clin North Am 21:1231–1246
Bladé J, Fernández de Larrea C, Rosiñol L, Cibeira MT, Jiménez R, Ponles R (2011) Soft-tissue plasmacytomas in multiple myeloma: incidence, mechanisms of extramedullary spread, and treatment approach. J Clin Oncol 29:3805–3812
Bladé J, Fernández de Larrrea C, Rosiñol L (2015) Extramedullary disease in multiple myeloma in the era of novel agents. Br J Haematol 169:763–765
Blimark C, Holmberg E, Juliusson G et al (2013) Real world data in myeloma: experiences from the swedish population-based registry on 2494 myeloma patients diagnosed 2008–2011. Blood 122(21):1972
Cha CH, Park CJ, Huh JR, Chi HS, Suh CW, Kang YK (2007) Significantly better prognosis for patients with primary plasma cell leukemia than for patients with secondary plasma cell leukemia. Acta Haematol 118(3):178–182
Chak LY, Cox RS, Bostwick DG, Hoppe RT (1987) Solitary plasmacytoma of bone: treatment, progression and survival. J Clin Oncol 5:1811–1815
Chamberlain MC, Glanz M (2008) Myelomatous meningitis. Cancer 112:1562–1567
Chang H, Sloan S, Li D, Patterson B (2005) Genomic aberrations in plasma cell leukemia shown by interphase fluorescence in situ hybridization. Cancer Genet Cytogenet 156(2):150–153
Chang H, Yeung J, Xu W, Ning Y, Patterson B (2006) Significant increase of CKS1B amplification from monoclonal gammopathy of undetermined significance to multiple myeloma and plasma cell leukaemia as demonstrated by interphase fluorescence in situ hybridisation. Br J Haematol 134(6):613–615
Chang H, Qi X, Yeung J, Reece D, Xu W, Patterson B (2009) Genetic aberrations including chromosome 1 abnormalities and clinical features of plasma cell leukemia. Leuk Res 33(2):259–262. https://doi.org/10.1016/j.leukres.2008.06.027
Charbonnier A, Michalet M, Xhaard A et al (2014) Allogeneic hematopoietic stem cell transplantation (ALLOHSCT) for primary plasma cell leukemia (PPCL): a prospective study of IFM group. Haematologica 99:165
Chiecchio L, Dagrada GP, White HE et al (2009) Frequent upregulation of MYC in plasma cell leukemia. Genes Chromosomes Cancer 48(7):624–636. https://doi.org/10.1002/gcc.20670.
Cifola I, Lionetti M, Pinatel E et al (2015) Whole-exome sequencing of primary plasma cell leukemia discloses heterogeneous mutational patterns. Oncotarget 6(19):17543–17558
Colovic M, Jankovic G, Suvajdzic N, Milic N, Dordevic V, Jankovic S (2008) Thirty patients with primary plasma cell leukemia: a single center experience. Med Oncol 25(2):154–160. https://doi.org/10.1007/s12032-007-9011-5
Corwin J, Lindberg RD (1979) Solitary plasmacytoma of bone vs. extramedullary plasmacytoma and their relationship to multiple myeloma. Cancer 45:1007–1013
Costello R, Sainty D, Bouabdallah R et al (2001) Primary plasma cell leukaemia: a report of 18 cases. Leuk Res 25(2):103–107
D'Arena G, Valentini CG, Pietrantuono G et al (2012) Frontline chemotherapy with bortezomib-containing combinations improves response rate and survival in primary plasma cell leukemia: a retrospective study from GIMEMA Multiple Myeloma Working Party. Ann Oncol 23(6):1499–1502. https://doi.org/10.1093/annonc/mdr480
Dimopoulos MA, Hillengas H, Usmani S et al (2015) Role of magnetic resonance imaging in the management of multiple myeloma: a consensus statement. J Clin Oncol 33:657–664
van de Donk NW, Lokhorst HM, Anderson KC, Richardson PG (2012) How I treat plasma cell leukemia. Blood 120(12):2376–2389
Dores GM, Landgren O, McGlynn KA et al (2008) Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: incidence and survival in the United States, 1992-2004. Br J Haematol 144:86–94
Drake MB, Iacobelli S, van Biezen A et al (2010) Primary plasma cell leukemia and autologous stem cell transplantation. Haematologica 95(5):804–809. https://doi.org/10.3324/haematol.2009.013334
Durie BGM, Harouseau JL, San Miguel JF et al (2016) International uniform response criteria for multiple myeloma. Leukemia 20:1467–1473
Esparis-Ogando A, Alegre A, Aguado B et al (2005) Bortezomib is an efficient agent in plasma cell leukemias. Int J Cancer 114(4):665–667
Fassas AB, Ward S, Muwalla F et al (2004) Myeloma of the central nervous system: strong association with unfavourable chromosomal abnormalities and other high-risk disease features. Leuk Lymphoma 45:291–300
Fernandez de Larrea C, Kyle RA, Durie BG et al (2013) Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia 27(4):780–791. https://doi.org/10.1038/leu.2012.336.
Gonsalves WI, Rajkumar SV, Go RS et al (2014a) Trends in survival of patients with primary plasma cell leukemia: a population-based analysis. Blood 124(6):907–912. https://doi.org/10.1182/blood-2014-03-565051
Gonsalves WI, Rajkumar V, Go RS et al (2014b) Trends in survival of patients with primary plasma cell leukemia: a population-based analysis from 1973 to 2010. J Clin Oncol 32(15 Suppl 1):8608
Gozzetti A, Cesare A, Lotti F et al (2012) Extramedullary intracranial localization of multiple myeloma and treatment with novel agents: a retrospective survey of 50 patients. Cancer 118:1574–1584
Granell M, Calvo X, Garcia A et al (2015) Prognostic impact of circulating plasma cells on survival of patients with multiple myeloma. Haematologica 100:517
Guifang O, Huiling Z, Yongwei H et al (2010) Haploidentical stem cell transplantation used in treating primary plasma cell leukemia: a case report. J Med Coll PLA 25(1):54–57. https://doi.org/10.1016/S1000-1948%2810%2960018-4
Husain MM, Metzer WS, Bider EF (1987) Multiple intraparenchymal brain plasmacytomas with spontaneous intratumoral hemorrhage. Neurology 20:619–623
Iriuchishima H, Murakami H, Ozaki S et al (2014) Primary plasma cell leukemia in the era of novel agent: report of multicenter study from Japanese society of myeloma. Blood 124(21):2008
Jaskiewicz AD, Herrington JD, Wong L (2005) Tumor lysis syndrome after bortezomib therapy for plasma cell leukemia. Pharmacotherapy 25(12):1820–1825
Jimenez Zepeda VH, Reece DE, Trudel S, Chen CI, Tiedemann RE, Kukreti V (2013) Lenalidomide (revlimid), bortezomib (velcade) and dexamethasone (RVD) for the treatment of secondary plasma cell leukaemia. Blood 122(21):5398
Jimenez-Zepeda VH, Dominguez VJ (2006) Plasma cell leukemia: a rare condition. Ann Hematol 85(4):263–267
Jimenez-Zepeda VH, Neme-Yunes Y, Braggio E (2011) Chromosome abnormalities defined by conventional cytogenetics in plasma cell leukemia: what have we learned about its biology? Eur J Haematol 87(1):20–27. https://doi.org/10.1111/j.1600-0609.2011.01629.x
Juliusson G, Celsing F, Turesson I et al (2000) Frequent good partial remissions from thalidomide including best response ever in patients with advamced refractory and relapsed myeloma. Br J Haematol 109:89–96
Jurczyszyn A, Grzasko N, Gozzetti A et al (2016) Central nervous system involvement by multiple myeloma: a multi-institutional retrospective study of 172 patients in daily clinical practice. Am J Hematol 91(6):575–580
Katadritou E, Terpos E, Kastritis E et al (2015) Lack of survival improvement with novel anti-myeloma agents for patients with multiple myeloma and central nervous system involvement: the Greek Myeloma Study Group experience. Ann Hematol 94:2033–2042
Katodritou E, Terpos E, Kelaidi C et al (2013) Treatment with bortezomib-based regimens improves overall response and predicts for survival in patients with primary or secondary plasma cell leukemia: analysis of the greek myeloma study group. Haematologica 98:331
Katodritou E, Terpos E, Symeonidis AS et al (2014) Clinical features, outcome, and prognostic factors for survival and evolution to multiple myeloma of solitary plasmacytomas : a report of the Greek myeloms study group in 97 patients. Am J Hematol 89:803–808
Knowling MA, Harwwd AR, Bergsagel DE (1983) Comparing extramedullary plasmacytomas with solitary and multiple plasma cell tumours of bone. J Clin Oncol 1:255–262
Kraj M, Kopec-Szlezak J, Poglod R, Kruk B (2011a) Flow cytometric immunophenotypic characteristics of 36 cases of plasma cell leukemia. Leuk Res 35(2):169–176. https://doi.org/10.1016/j.leukres.2010.04.021
Kraj M, Poglod R, Kopec-Szlezak J, Kruk B, Letowska M (2011b) Clinical and immunophenotypic characteristics of 63 plasma cell leukemia (PCL) cases. Haematologica 96:S67
Kryukov F, Ihnatova I, Nemec P et al (2013) Cell cycle gene sets coordination in multiple myeloma and plasma cell leukemia. Blood 122(21):1901
Landsburg DJ, Vogl DT, Plastaras JP, Stadtmauer EA (2014) Melphalan/total body irradiation-conditioned myeloablative allogeneic hematopoietic cell transplantation for patients with primary plasma cell leukemia. Clin Lymphoma Myeloma Leuk 14(6):e225–e228. https://doi.org/10.1016/j.clml.2014.07.012
Lebovic D, Zhang L, Alsina M et al (2011) Clinical outcomes of patients with plasma cell leukemia in the era of novel therapies and hematopoietic stem cell transplantation strategies: a single-institution experience. Clin Lymphoma Myeloma Leuk 11(6):507–511. https://doi.org/10.1016/j.clml.2011.06.010
Lionetti M, Musto P, Todoerti K et al (2011) The investigation of genomic profiles in plasma cell leukemias by means of an integrative microarray approach. Haematologica 96:194
Lionetti M, Musto P, Di Martino MT et al (2013) Biological and clinical relevance of miRNA expression signatures in primary plasma cell leukemia. Clin Cancer Res 19(12):3130–3142. https://doi.org/10.1158/1078-0432.CCR-12-2043
Lloveras E, Granada I, Zamora L et al (2004) Cytogenetic and fluorescence in situ hybridization studies in 60 patients with multiple myeloma and plasma cell leukemia. Cancer Genet Cytogenet 148(1):71–76
Mahindra A, Kalaycio ME, Vela-Ojeda J et al (2012) Hematopoietic cell transplantation for primary plasma cell leukemia: results from the Center for International Blood and Marrow Transplant Research. Leukemia 26(5):1091–1097. https://doi.org/10.1038/leu.2011.312
Marionneaux S, Monsalve B, Plante N, Shulman S, Vega AM (2006) The application of Beckman Coulter VCS technology at a major cancer center, with emphasis on the detection of circulating immature plasma cells in plasma cell leukemia. Lab Hematol 12(4):210–216
Mayr NA, Wen BC, Hussey DH et al (1990) The role of radiation therapy in the treatment of solitary plasmacytomas. Radiother Oncol 17:293–303
Meiss JM, Butler JJ, Osborne BM, Ordoñez NG (1987) Solitary plasmacytoma of bone and extramedullary plasmacytoma. A clinicopathologic and immunohistochemical study. Cancer 59:1475–1485
Mesguish C, Fardanesh R, Tanenbaum L et al (2014) State of the art imaging of multiple myeloma: comparative review of FDG PET/CT imaging in various clinical settings. Eur J Radiol 83:2203–2223
Minnema MC, van de Donk NW, Zweegman S et al (2004) Extramedullary relapses after allogeneic non-myeloablative stem cell transplantation in multiple myeloma patients do not negativaly affect treament outcome. Bone Marrow Transplant 34:1057–1065
Moreau P, Cavo M, Sonneveld P et al (2014) Combination of international scoring system 3, high lactate dehidrogenase, and t(4;14) or del (17p) identifies patients with multiple myeloma (MM) treated with front-line autologous stem-cell transplantation at high risk of early MM progression-related death. J Clin Oncol 32:2173–2180
Mosca L, Musto P, Fabris S et al (2011) Integrative genomic analysis of primary plasma cell leukemia reveals strong gene and microRNA dosage effect. Haematologica 96:S54
Mosca L, Musto P, Todoerti K et al (2013) Genome-wide analysis of primary plasma cell leukemia identifies recurrent imbalances associated with changes in transcriptional profiles. Am J Hematol 88(1):16–23. https://doi.org/10.1002/ajh.23339
Muchtar E, Raanani P, Yeshurum M, Shpilberg O, Magen-Nativ H (2014) Myeloma in scar tissue – an underrepresented phenomenon or an emerging entity in the novel agents`era? A single center series. Acta Haematol 132:39–54
Musto P, Rossini F, Gay F et al (2007) Efficacy and safety of bortezomib in patients with plasma cell leukemia. Cancer 109(11):2285–2290
Musto P, D'Auria F, Petrucci MT et al (2010) First line therapy with lenalidomide and dexamethasone in primary plasma cell leukemia. Haematologica 95:S43
Musto P, D'Auria F, Petrucci MT et al (2011a) Efficacy and safety of lenalidomide in combination with low dose dexamethasone (LD) as first line treatment of primary plasma cell leukemia (PPCL). Haematologica 96:126
Musto P, D’Auria F, Petrucci MT et al (2011b) Final results of a phase II study evaluating lenalidomide in combination with low dose dexamethasone as first line therapy for primary plasma cell leukemia. Blood 118(21):2925
Musto P, Fraticelli VL, Mansueto G et al (2012) Bendamustine as salvage therapy in multiple myeloma: a retrospective, multicenter study from the italian compassionate use program in 78 heavily pre-treated patients. Blood 120(21):2971
Musto P, Neri A, Simeon V et al (2013) Conclusive analysis of clinical and molecular results. From RV-PCL-PI-350 trial, the first prospective study of a novel agent (lenalidomide) in primary plasma cell leukemia. Haematologica 98:10–11
Muzzafar T, Kaul S, Gonzalez-Berjon JM, Shah J (2013) Plasma cell leukemia: impact of cytogenetic profile on prognosis. Lab Investig 93:350A. https://doi.org/10.1038/labinvest.2013.24
Myers B, Grimley C, Crouch D et al (2001) Lack of response to thalidomide in plasmacytomas. Br J Haematol 115:324
Nanni C, Zamagni E, Versari A et al (2016) Image interpretation criteria fod FDG PET/CT in multiple myeloma; a new proposal from an Italian expert panel.IMPeTUs (Italian Myeloma criteria for PET USe). Eur J Nucl Med Mol Imaging 43(3):414–421
Nieuwenhuizen L, Biesma DH (2007) Central nervous system myelomatosis: review of the literature. Eur J Haematol 80:1–9
Nooka A, Kaufman J, Muppidi S et al (2012) Plasma cell leukemia: sustained responses are possible with innovative treatment strategies. Haematologica 97:601
Pagano L, Valentini CG, De Stefano V et al (2011) Primary plasma cell leukemia: a retrospective multicenter study of 73 patients. Ann Oncol 22(7):1628–1635. https://doi.org/10.1093/annonc/mdq646
Papanikolaou X, Repousis P, Tzenou T et al (2013) Incidence, clinical features, laboratory findings and outcome of patients with multiple myeloma presenting with extramedullary relapse. Leuk Lymphoma 54:1459–1464
Parmar G, Masih-khan E, Atenafu EG et al (2014) Outcome of 17p deleted multiple myeloma (MM) in the era of novel agents and tandem transplantation: a single centre experience. Blood 124(21):4756
Pasmantier MW, Azar HA (1969) Extraskeletal spread in multiple plasma cell myeloma: a review of 57 autopsied cases. Cancer 23:167–174
Pasqualetti P, Festuccia V, Collacciani A, Acitelli P, Casale R (1996) Plasma cell leukemia. A report on 11 patients and review of the literature. Panminerva Med 38(3):179–184
Patriarca F, Prosdocimo S, Tomadini V et al (2005) Efficacy of bortezomib therapy for extramedullary relapse of myeloma after autologous and non-myeloablative allogeneic transplantation. Haematologica 90:278–279
Pavlovic A, Radic-Kristo D, Ostojic Kolonic S et al (2012) Atypical blast morphology of primary plasma cell leukemia with renal involvement and plasmablasts in urine. Cytopathology 23:79. https://doi.org/10.1111/cyt.12004
Peijing Q, Yan X, Yafei W et al (2009) A retrospective analysis of thirty-one cases of plasma cell leukemia from a single center in China. Acta Haematol 121(1):47–51. https://doi.org/10.1159/000210555
Pellat-Deceunynck C, Barille S, Jego G et al (1998) The absence of CD56 (NCAM) on malignant plasma cells is a hallmark of plasma cell leukemia and of a special subset of multiple myeloma. Leukemia 12(12):1977–1982
Pérez Simón JA, Sureda A, Fernández- Avilés F et al (2006) Reduce-intensity conditioning allogeneic transplantation is associated with a high incidence of extramedullary relapse in multiple myeloma patients. Leukemia 20:542–545
Perez-Andres M, Almeida J, Martin-Ayuso M et al (2005) Clonal plasma cells from monoclonal gammopathy of undetermined significance, multiple myeloma and plasma cell leukemia show different expression profiles of molecules involved in the interaction with the immunological bone marrow microenvironment. Leukemia 19(3):449–455
Posner JB (1987) Back pain and epidural spinal cord compression. Med Clin North Am 71:185–201
Pour L, Sevcikova S, Greslikova H et al (2014) Soft-tissue extramedullary multiple myeloma prognosis is significantly worse in comparison to bone-related extramedullary relapse. Haematologica 99:360–364
Ramsingh G, Mehan P, Luo J, Vij R, Morgensztern D (2009) Primary plasma cell leukemia: a surveillance, epidemiology, and end results database analysis between 1973 and 2004. Cancer 115(24):5734–5739. https://doi.org/10.1002/cncr.24700
Rasche L, Strifler S, Duell J et al (2014) The lymphoma-like polychemotherapy regimen “Dexa-BEAM” in advanced and extramedullary multiple myeloma. Ann Hematol 93:1207–1214
Rasillo A, Tabernero MD, Sanchez ML et al (2003) Fluorescence in situ hybridization analysis of aneuploidization patterns in monoclonal gammopathy of undetermined significance versus multiple myeloma and plasma cell leukemia. Cancer 97(3):601–609
Ravipati HP, Kaufman JL, Langston AA et al (2013) Survival outcomes of plasma cell leukemia (PCL) in the United States: A SEER analysis. J Clin Oncol 31(15 Suppl 1):8609
Reece DE, Phillips M, Chen CI et al (2013) Induction therapy with cyclophosphamide, bortezomib dexamethasone (cybord) for primary plasma cell leukemia (PPCL). Blood 122(21):5378
Rosiñol L, Cibeira MT, Bladé J et al (2004) Extramedullary multiple myeloma escapes the effect of thalidomide. Haematologica 89:832–836
Rosiñol L, Cibeira MT, Bladé J et al (2006) Bortezomib: an effective agent in extramedullary disease in multiple myeloma. Eur J Haematol 76:405–408
Rosiñol L, Oriol A, Teruel AI et al (2012) Superiority of bortezomib, thalidomide and dexamethasone (VTD) as induction pre-transplantation therapy in multiple myeloma: results of a randomized phase III PETHEMA/GEM study. Blood 120:1589–1596
Rosiñol L, Fernández de Larrea C, Bladé J (2014) Extramedullary myeloma spread triggered by surgical procedures: an emerging entity? Acta Haematol 132:36–38
Royer B, Merlusca L, Lioure B et al (2013) Bortezomib-doxorubicine-dex/bortezomibcyclophosphamide-dex for primary plasma cell leukemia: a phase 2 study by the IFM. Clin Lymphoma Myeloma Leuk 13:S116–S117
Ruggeri M, Caltagirone S, Gilestro M et al (2010) 12p, 1p, 1q, 5q, 11q abnormalities and immunophenotype in monoclonal gammopathy of undetermined significance, multiple myeloma and plasma cell leukemia. Haematologica 95:S68–S69
Saccaro S, Fonseca R, Veillon DM et al (2005) Primary plasma cell leukemia: report of 17 new cases treated with autologous or allogeneic stem-cell transplantation and review of the literature. Am J Hematol 78(4):288–294
San Miguel JF, Schlag R, Khuageva NK et al (2008) Bortezomib plus melphalan and prednisone for initial treatment of multiple myeloma. N Engl J Med 359:906–917
Schluteman KO, Fassas AB, Van Hemert RL et al (2004) Multiple myeloma invasion of the central nervous system. Arch Neurol 61:1423–1429
Short KD, Rajkumar SV, Larson D et al (2011) Incidence of extramedullary disease in patients with multiple myeloma in the era of novel therapy and the activity of pomalidomide in extramedullary myeloma. Leukemia 25:906–908
Sonneveld P, Goldschmith H, Rosiñol L et al (2013) Bortezomib-based versus non-bortezomib-based induction treatment before autologous stem-cell transplantation in patients with previously untreated multiple myeloma: a meta-analysis of phase III randomized, controlled trials. J Clin Oncol 31:3279–3287
Srikanth M, Davies FE, Wu P et al (2008) Survival and outcome of blastoid variant myeloma following treatment with the novel thalidomide containing regimen DT-PACE. Eur J Haemtol 81:432–436
Talamo G, Dolloff NG, Sharma K, Zhu J, Malysz J (2012) Clinical features and outcomes of plasma cell leukemia: a single-institution experience in the era of novel agents. Rare Tumors 4(3):e39. https://doi.org/10.4081/rt.2012.e39
Tiedemann RE, Gonzalez-Paz N, Kyle RA et al (2008) Genetic aberrations and survival in plasma cell leukemia. Leukemia 22(5):1044–1052. https://doi.org/10.1038/leu.2008.4
Todoerti K, Musto P, Lionetti M et al (2011) Gene and miRNA expression profiles in plasma cell leukemias. Haematologica 96:S54
Todoerti K, Musto P, Agnelli L et al (2012) Transcriptome analysis of primary plasma cell leukemia tumors from a multicenter prospective gimema study: biological and clinical implications. Haematologica 97:S49
Todoerti K, Manzoni M, Fabris S et al (2014) Different gene and mirna expression profiles in primary and secondary plasma cell leukemia by high-resolution microarray analyses. Haematologica 99:S39
Tong D, Griffin TW, Laramore GE et al (1980) Solitary plasmacytoma of bone and soft tissues. Radiology 135:195–198
Usmani SZ, Heuck C, Mitchell A et al (2012) Extramedullary disease portends poor prognosis in multiple myeloma and is overrepresented in high-risk disease even in the era of novel agents. Haematologica 97:4761–4767
Usmani SZ, Sexton R, Ailawadhi S et al (2014) Swog 1211: initial report on phase I trial of RVD-elotuzumab for newly diagnosed high risk multiple myeloma (HRMM). Blood 124(21):4762
Vagnoni D, Travaglini F, Pezzoni V et al (2015) Circulating plasma cells in newly diagnosed symptomatic multiple myeloma as a possible prognostic marker for patients with standard-risk cytogenetics. Br J Haematol 170(4):523–531. https://doi.org/10.1111/bjh.13484.
Varettoni M, Corso A, Pica G et al (2009) Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: a longitudinal study on 1,003 consecutive patients. Ann Oncol 21:325–330
Varga C, Xie W, Laubach J et al (2015) Development of extramedullary myeloma in the era of novel agents: no evidence of increased risk with lenalidomide-bortezomib combinations. Br J Haematol 169:843–850
van Veen JJ, Reilly JT, Richards SJ et al (2004) Diagnosis of plasma cell leukaemia: findings of the UK NEQAS for Leucocyte Immunophenotyping scheme. Clin Lab Haematol 26(1):37–42
Weinstock M, Aljawai Y, Morgan EA et al (2015) Incidence and clinical features of extramedullary multiple myeloma in patients who underwent stem cell transplantation. Br J Haematol 169:851–858
Wiltshaw E (1976) The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine 55:217–237
Woodruff RK, Ireton HJC (1982) Multiple nerve palsies as the presenting features of meningeal myelomatosis. Cancer 49:1710–1712
Woodruff RK, Whittle JM, Malpas JS (1979) Solitary plasmacytoma. I. Extramedullary soft-tissue plasmacytoma. Cancer 43:2340–2343
Wu P, Davies F, Boyd K et al (2009) The impact of extramedullary disease at presentation in the outcome of myeloma. Leuk Lymphoma 50:230–235
Xu W, Li JY, Fan L et al (2009) Molecular cytogenetic aberrations in 21 Chinese patients with plasma cell leukemia. Int J Lab Hematol 31(3):338–343. https://doi.org/10.1111/j.1751-553X.2008.01037.x
Zamagni E, Cavo M (2012) The role of imaging techniques in the management of multiple myeloma. Br J Haematol 159:499–513
Zamagni E, Nanni C, Tachetti P et al (2014) Positron emission tomography with computed tomography-based diagnosis of massive extramedullary progression in a patient with high-risk multiple myeloma. Clin Lymphoma Myeloma Leuk 14:101–104
Zeiser R, Deaschler B, Bertz H et al (2004) Extramedullary vs. Medullary relapse after autologous or allogeneic hematopoietic stem cell transplantation (HSCT) in multiple myeloma (MM) and its correlation to clinical outcome. Bone Marrow Transplant 34:1057–1065
Zhuang J, Da Y, Li H et al (2014) Cytogenetic and clinical risk factors for assessment of ultra high-risk multiple myeloma. Leuk Res 38(2):188–193. https://doi.org/10.1016/j.leukres.2013.11.010
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Gertz, M.A., Rosinol, L., Bladé, J. (2018). Plasma Cell Leukemia and Extramedullary Plasmacytoma. In: Dimopoulos, M., Facon, T., Terpos, E. (eds) Multiple Myeloma and Other Plasma Cell Neoplasms. Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-319-25586-6_9
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