{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,30]],"date-time":"2025-06-30T16:26:33Z","timestamp":1751300793822},"reference-count":33,"publisher":"Croatian Society for Medical Biochemistry and Laboratory Medicine","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biochem. med. (Online)"],"published-print":{"date-parts":[[2020,2,15]]},"abstract":"<jats:p>This is a case report of a challenging diagnosis of IgE monoclonal gammopathy of undetermined significance, which transformed into myeloma, then transformed into IgE-producing plasma cell leukaemia in a 71-year-old male who was followed in Brest, France, from 2015 to 2019. The IgEproducing variant is the rarest sub-type of multiple myeloma, and plasma cell leukaemia is considered to be the rarest and the most aggressive of human monoclonal gammopathies. In November 2015, hypogammaglobulinemia was detected during a systematic check-up. A kappa light chain monoclonal gammopathy was first diagnosed due to an increase of the free kappa\/lambda light chains ratio. No monoclonal immunoglobulin was detected by either serum protein electrophoresis (Capillarys 2, Sebia, Issy-les-Moulineaux, France) or immunofixation (Hydrasys 2, Sebia, Issy-les-\nMoulineaux, France). In June 2018, a blood smear led to the diagnosis of plasma cell leukaemia. A monoclonal peak was detected and identified as IgE-kappa. Analysis of an archival sample taken three years earlier, revealed the presence of a monoclonal IgE, which had been missed at diagnosis. Chemotherapy with bortezomib and dexamethasone was introduced. The patient survived 10 months after the diagnosis of leukaemia. This case shows that an abnormal free light chain ratio should be considered as a possible marker of IgE monoclonal gammopathy even in the absence of a solitary light chain revealed by immunofixation. In addition, the use of an undiluted serum may increase the sensitivity of the immunofixation for\nthe detection of IgE monoclonal gammopathies compared to the 1:3 dilution recommended by the manufacturer.<\/jats:p>","DOI":"10.11613\/bm.2020.010801","type":"journal-article","created":{"date-parts":[[2019,12,13]],"date-time":"2019-12-13T17:24:31Z","timestamp":1576257871000},"page":"134-142","source":"Crossref","is-referenced-by-count":9,"title":["A case of IgE myeloma transformed into IgE-producing plasma cell leukaemia"],"prefix":"10.11613","volume":"30","author":[{"given":"Nicolas","family":"Galakhoff","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Pharmaco-Toxicology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Richard","family":"Eveillard","sequence":"additional","affiliation":[{"name":"Department of Haematology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ma\u00ebl","family":"Padelli","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmaco-Toxicology, Martinique University Hospital, Fort-de-France, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Luc","family":"Carr\u00e9","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmaco-Toxicology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c9ric","family":"Lippert","sequence":"additional","affiliation":[{"name":"Laboratory of Haematology, Brest University Hospital, Brest, France; Universit\u00e9 de Brest, INSERM, EFS, UMR 1078, GGB, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Buors","sequence":"additional","affiliation":[{"name":"Laboratory of Haematology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cile","family":"Aubron","sequence":"additional","affiliation":[{"name":"Medical Intensive Care, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H\u00e9l\u00e8ne","family":"Kerspern","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmaco-Toxicology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrian","family":"Tempescul","sequence":"additional","affiliation":[{"name":"Department of Haematology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyril","family":"Leven","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Pharmaco-Toxicology, Brest University Hospital, Brest, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"4885","published-online":{"date-parts":[[2020,2,15]]},"reference":[{"key":"key20191213122425_r1","doi-asserted-by":"crossref","first-page":"e538","DOI":"10.1016\/S1470-2045(14)70442-5","article-title":"International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma.","volume":"15","author":"Rajkumar","year":"2014","journal-title":"Lancet Oncol"},{"key":"key20191213122425_r2","doi-asserted-by":"crossref","first-page":"21","DOI":"10.4065\/78.1.21","article-title":"Review of 1027 patients with newly diagnosed multiple myeloma.","volume":"78","author":"Kyle","year":"2003","journal-title":"Mayo Clin Proc"},{"key":"key20191213122425_r3","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1053\/j.seminhematol.2010.11.003","article-title":"Pathogenesis of monoclonal gammopathy of undetermined significance (MGUS) and progression to multiple myeloma.","volume":"48","author":"Zingone","year":"2011","journal-title":"Semin Hematol"},{"key":"key20191213122425_r4","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1016\/S0140-6736(10)60482-5","article-title":"Prevalence and risk of progression of light-chain monoclonal gammopathy of undetermined significance: a retrospective population-based cohort study.","volume":"375","author":"Dispenzieri","year":"2010","journal-title":"Lancet"},{"key":"key20191213122425_r5","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.clinbiochem.2017.09.015","article-title":"IgE monoclonal gammopathy: A case report and literature review.","volume":"51","author":"Hejl","year":"2018","journal-title":"Clin Biochem"},{"key":"key20191213122425_r6","doi-asserted-by":"crossref","first-page":"597","DOI":"10.3109\/10428199909058419","article-title":"IgE multiple myeloma.","volume":"32","author":"Macro","year":"1999","journal-title":"Leuk Lymphoma"},{"key":"key20191213122425_r7","doi-asserted-by":"crossref","first-page":"3724","DOI":"10.1182\/blood-2010-05-282632","article-title":"Incidence of hematologic malignancies in Europe by morphologic subtype: results of the HAEMACARE project.","volume":"116","author":"Sant","year":"2010","journal-title":"Blood"},{"key":"key20191213122425_r8","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1046\/j.1365-2141.2003.04355.x","article-title":"Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.","volume":"121","author":"International Myeloma Working Group","year":"2003","journal-title":"Br J Haematol"},{"key":"key20191213122425_r9","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1007\/s00277-005-0054-4","article-title":"Plasma cell leukemia: a rare condition.","volume":"85","author":"Jim\u00e9nez-Zepeda","year":"2006","journal-title":"Ann Hematol"},{"key":"key20191213122425_r10","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1159\/000109470","article-title":"Significantly better prognosis for patients with primary plasma cell leukemia than for patients with secondary plasma cell leukemia.","volume":"118","author":"Cha","year":"2007","journal-title":"Acta Haematol"},{"key":"key20191213122425_r11","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1002\/ajh.23600","article-title":"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.","volume":"89","author":"Katodritou","year":"2014","journal-title":"Am J Hematol"},{"key":"key20191213122425_r12","doi-asserted-by":"crossref","first-page":"1538","DOI":"10.1080\/10428194.2016.1250263","article-title":"Plasma cell leukemia: update on biology and therapy.","volume":"58","author":"Mina","year":"2017","journal-title":"Leuk Lymphoma"},{"key":"key20191213122425_r13","doi-asserted-by":"crossref","first-page":"2376","DOI":"10.1182\/blood-2012-05-408682","article-title":"How I treat plasma cell leukemia.","volume":"120","author":"van de Donk","year":"2012","journal-title":"Blood"},{"key":"key20191213122425_r14","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1038\/leu.2008.307","article-title":"International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders.","volume":"23","author":"Dispenzieri","year":"2009","journal-title":"Leukemia"},{"key":"key20191213122425_r15","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1038\/leu.2008.291","article-title":"Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma.","volume":"23","author":"Kyle","year":"2009","journal-title":"Leukemia"},{"key":"key20191213122425_r16","doi-asserted-by":"crossref","first-page":"1941","DOI":"10.1001\/jama.2012.3954","article-title":"Comparison of risk prediction using the CKD-EPI equation and the MDRD study equation for estimated glomerular filtration rate.","volume":"307","author":"Matsushita","year":"2012","journal-title":"JAMA"},{"key":"key20191213122425_r17","doi-asserted-by":"crossref","first-page":"2863","DOI":"10.1200\/JCO.2015.61.2267","article-title":"Revised International Staging System for Multiple Myeloma: A Report From International Myeloma Working Group.","volume":"33","author":"Palumbo","year":"2015","journal-title":"J Clin Oncol"},{"key":"key20191213122425_r18","unstructured":"Sebia Instructions. Available at: http:\/\/www.ilexmedical.com\/files\/Sebia%20inserts\/HYDRAGEL__IF_DM_Hydrasis.pdf Accessed July 9th 2019."},{"key":"key20191213122425_r19","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1111\/bjh.15092","article-title":"Prognostic indicators in primary plasma cell leukaemia: a multicentre retrospective study of 117 patients.","volume":"180","author":"Jurczyszyn","year":"2018","journal-title":"Br J Haematol"},{"key":"key20191213122425_r20","doi-asserted-by":"crossref","first-page":"1499","DOI":"10.1093\/annonc\/mdr480","article-title":"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.","volume":"23","author":"D\u2019Arena","year":"2012","journal-title":"Ann Oncol"},{"key":"key20191213122425_r21","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/1756-8722-3-32","article-title":"Prozone effect of serum IgE levels in a case of plasma cell leukemia.","volume":"3","author":"Talamo","year":"2010","journal-title":"J Hematol Oncol"},{"key":"key20191213122425_r22","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.cca.2013.07.006","article-title":"An IgE multiple myeloma: contradictory findings in clinical laboratory testing.","volume":"425","author":"Altinier","year":"2013","journal-title":"Clin Chim Acta"},{"key":"key20191213122425_r23","first-page":"944","article-title":"Serum protein electrophoresis and immunofixation by a semiautomated electrophoresis system.","volume":"44","author":"Bossuyt","year":"1998","journal-title":"Clin Chem"},{"key":"key20191213122425_r24","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1515\/CCLM.2009.121","article-title":"Stability of selected serum proteins after long-term storage in the Janus Serum Bank.","volume":"47","author":"Gislefoss","year":"2009","journal-title":"Clin Chem Lab Med"},{"key":"key20191213122425_r25","doi-asserted-by":"crossref","first-page":"e183","DOI":"10.1515\/cclm-2014-0182","article-title":"A rare condition: IgE type monoclonal gammopathy of undetermined significance.","volume":"52","author":"Caldini","year":"2014","journal-title":"Clin Chem Lab Med"},{"key":"key20191213122425_r26","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1136\/bmj.281.6239.539","article-title":"\u201cBenign\u201d monoclonal IgE gammopathy.","volume":"281","author":"Ludwig","year":"1980","journal-title":"BMJ"},{"key":"key20191213122425_r27","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1038\/leu.2012.336","article-title":"Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group.","volume":"27","author":"Fern\u00e1ndez de Larrea","year":"2013","journal-title":"Leukemia"},{"key":"key20191213122425_r28","first-page":"798","article-title":"Unusual myelomas: a review of IgD and IgE variants.","volume":"27","author":"Pandey","year":"2013","journal-title":"Oncology (Williston Park)"},{"key":"key20191213122425_r29","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1007\/s11914-017-0397-5","article-title":"Myeloma and bone disease.","volume":"15","author":"Panaroni","year":"2017","journal-title":"Curr Osteoporos Rep"},{"key":"key20191213122425_r30","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1080\/10428194.2018.1473574","article-title":"Secondary plasma cell leukemia: a multicenter retrospective study of 101 patients.","volume":"60","author":"Jurczyszyn","year":"2019","journal-title":"Leuk Lymphoma"},{"key":"key20191213122425_r31","doi-asserted-by":"crossref","first-page":"1570","DOI":"10.1182\/blood-2002-08-2436","article-title":"Intergroupe Francophone du My\u00e9lome. Translocation t(11;14)(q13;q32) is the hallmark of IgM, IgE, and nonsecretory multiple myeloma variants.","volume":"101","author":"Avet-Loiseau","year":"2003","journal-title":"Blood"},{"key":"key20191213122425_r32","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1007\/s11912-019-0754-x","article-title":"Plasma cell leukemia: definition, presentation and treatment.","volume":"21","author":"Gundesen","year":"2019","journal-title":"Curr Oncol Rep"},{"key":"key20191213122425_r33","doi-asserted-by":"crossref","first-page":"232","DOI":"10.3109\/10428194.2014.893304","article-title":"Lenalidomide (Revlimid), bortezomib (Velcade) and dexamethasone for the treatment of secondary plasma cell leukemia.","volume":"56","author":"Jimenez-Zepeda","year":"2015","journal-title":"Leuk Lymphoma"}],"container-title":["Biochemia medica"],"original-title":[],"link":[{"URL":"https:\/\/www.biochemia-medica.com\/en\/journal\/30\/1\/10.11613\/BM.2020.010801\/fullArticle","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,2,17]],"date-time":"2020-02-17T13:38:25Z","timestamp":1581946705000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.biochemia-medica.com\/en\/journal\/30\/1\/10.11613\/BM.2020.010801"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2,15]]},"references-count":33,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2020,2,15]]},"published-print":{"date-parts":[[2020,2,15]]}},"URL":"https:\/\/doi.org\/10.11613\/bm.2020.010801","relation":{},"ISSN":["1846-7482","1330-0962"],"issn-type":[{"value":"1846-7482","type":"electronic"},{"value":"1330-0962","type":"print"}],"subject":[],"published":{"date-parts":[[2020,2,15]]}}}