IgG4 plasma cell myeloma without clinical evidence of IgG4-related disease: a report of two cases.
Deonne Thaddeus V. Gauiran,Deonne Thaddeus V. Gauiran,Krista M Marcon,Krista M Marcon,Mari L. DeMarco,Mari L. DeMarco,Angela W.S. Fung,Angela W.S. Fung,Grace van der Gugten,Andre Mattman,Andre Mattman,Mollie N. Carruthers,Kevin W. Song,Luke Y.C. Chen +13 more
TLDR
These cases illustrate the importance of performing serum protein electrophoresis in tandem with IgG subclasses to distinguish between polyclonal and monoclonal increases in serum IgG4.Abstract:
Background: Serum IgG4 is typically measured to investigate for Immunoglobulin G4-related Disease (IgG4-RD), a fibroinflammatory condition associated with polyclonal increase in serum IgG4. However, increased IgG4 can also be monoclonal, and little is known about IgG4 myeloma. Methods: We describe two cases of IgG4 myeloma without clinical, radiologic, or laboratory features of IgG4-related disease. Results: An 84 year old man presented with anemia and compression fractures and a 77 year old man presented with anemia, hypercalcemia and renal failure. Both had markedly elevated monoclonal serum IgG4, 34 g/L and 48 g/L in the beta region, and increased IgG positive bone marrow plasma cells, 50% and 80%, respectively. Neither had clinical or radiological manifestations of IgG4-related disease (IgG4-RD) such as salivary or lacrimal gland swelling, autoimmune pancreatitis , or retroperitoneal fibrosis. Both cases responded well to standard myeloma therapy. The IgG4 paraprotein caused spuriously elevated beta-2 microglobulin of 45.2 mg/L in case two due to interference with the assay. Conclusion: These cases illustrate the importance of performing serum protein electrophoresis in tandem with IgG subclasses to distinguish between polyclonal and monoclonal increases in serum IgG4. The lack of typical IgG4-RD features in these two patients suggests that monoclonal elevation in serum IgG4 alone is insufficient to cause the organ damage characteristic of IgG4-RD. Larger studies of IgG myeloma subtypes are warranted to explore whether IgG1, IgG2, IgG3 and IgG4 myeloma differ in natural history and whether the interference with beta-2 microglobulin is specific to IgG4 monoclonal proteins.read more
Citations
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Polyclonal hypergammaglobulinaemia: assessment, clinical interpretation, and management
Eric J. Zhao,Eric J. Zhao,Catherine V Cheng,Catherine V Cheng,Andre Mattman,Andre Mattman,Andre Mattman,Luke Y.C. Chen,Luke Y.C. Chen +8 more
TL;DR: In this article, a practical approach to assessing and managing polyclonal hypergammaglobulinaemia in adults is presented, which can also provide an important diagnostic clue of rare diseases such as histiocyte disorders, autoimmune lymphoproliferative syndrome, Castleman disease, and IgG4-related disease.
Journal ArticleDOI
Clinical utility of serum IgG4 measurement.
Julia L. Varghese,Angela W.S. Fung,Andre Mattman,Tien T.T. Quach,Deonne Thaddeus V. Gauiran,Mollie N. Carruthers,Luke Y.C. Chen +6 more
TL;DR: Limitations of current clinical methods used in quantitation of serum IgG4 concentrations will be discussed, including the common immunonephelometric assays and LC-MS/MS based assays.
Journal ArticleDOI
Immune Dysregulation in IgG4-Related Disease.
Jiachen Liu,Wei Yin,Lisa S. Westerberg,Pamela Lee,Quan Gong,Yan Chen,Lingli Dong,Chaohong Liu +7 more
TL;DR: A review of the most recent insights on IgG4-related disease can be found in this paper, focusing on immune dysregulation involved in the pathogenesis of this autoimmune condition.
Journal ArticleDOI
IgG4-Related Disease as Mimicker of Malignancy
WanLi Zhou,Timothy E. Murray,Liliana Cartagena,Howard John Lim,David F. Schaeffer,Graham W. Slack,Brian Skinnider,Eric Lam,Robert Irvine,Stephen W. C. Chung,Andre Mattman,Silvia D. Chang,Mollie N. Carruthers,Luke Y.C. Chen +13 more
TL;DR: In this article, the authors performed a retrospective single-center study examining how many patients with IgG4-related disease were initially believed to have malignancy, with particular attention to those who underwent potentially avoidable surgical procedures.
Journal ArticleDOI
Roles of IgG4 and IgG4/IgG ratio to IgG4-related disease in patients with elevated serum IgG4 level
TL;DR: Elevated serum IgG4 was found in a variety of diseases, especially in chronic diseases of various organs and manifesting a great value for IgG 4-RD diagnosis, and are available for the treatment evaluation of Igg4-RD.
References
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Consensus statement on the pathology of IgG4-related disease
Vikram Deshpande,Yoh Zen,John Kc Chan,Eunhee E Yi,Yasuharu Sato,Tadashi Yoshino,Günter Klöppel,J. Godfrey Heathcote,Arezou Khosroshahi,Judith A. Ferry,Rob C. Aalberse,Daniel Bloch,William R. Brugge,Adrian C Bateman,Mollie N. Carruthers,Suresh T. Chari,Wah Cheuk,Lynn D. Cornell,Carlos Fernandez-del Castillo,David G. Forcione,Daniel L. Hamilos,Terumi Kamisawa,Satomi Kasashima,Shigeyuki Kawa,Mitsuhiro Kawano,Gregory Y. Lauwers,Yasufumi Masaki,Yasuni Nakanuma,Kenji Notohara,Kazuichi Okazaki,Ji Kon Ryu,Takako Saeki,Dushyant V. Sahani,Thomas C. Smyrk,James Robert Stone,Masayuki Takahira,George Webster,Motohisa Yamamoto,Giuseppe Zamboni,Hisanori Umehara,John H. Stone +40 more
TL;DR: This statement proposes a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy, and advocates the use of strict criteria for accepting newly proposed entities or sites as components of the IgG 4- related disease spectrum.
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TL;DR: Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.
Journal ArticleDOI
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Zachary S. Wallace,Ray Naden,Suresh T. Chari,Hyon K. Choi,Emanuel Della-Torre,Jean Francois Dicaire,Phillip A. Hart,Dai Inoue,Mitsuhiro Kawano,Arezou Khosroshahi,Marco Lanzillotta,Kazuichi Okazaki,Cory A. Perugino,Amita Sharma,Takako Saeki,Nicolas Schleinitz,Naoki Takahashi,Hisanori Umehara,Yoh Zen,John H. Stone +19 more
TL;DR: In this article, an international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR).
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H CHAIN SUBGROUPS OF MYELOMA PROTEINS AND NORMAL 7S γ-GLOBULIN
Howard M. Grey,Henry G. Kunkel +1 more
TL;DR: Through the use of a variety of antisera to isolated myeloma proteins, four subgroups of 7S γ-globulin type proteins were readily distinguished and the unique antigenic character of both groups was localized to the H chains, although different determinants were involved for different antisersa.
Journal ArticleDOI
Subclasses of human gamma-2-globulin based on differences in the heavy polypeptide chains.
William D. Terry,John L. Fahey +1 more
TL;DR: Three subclasses of human γ2-globulin (IgG) molecules were detected in normal human serum with antiserums prepared in monkeys, and the distinguishing antigenic characteristics of each subclass were associated with the heavy polypeptide chains and the F (fast) fragments resulting from treatment of γ1- globulins with papain.
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