Waltham: UpToDate; 2017. Direct trafficking pathways from the Golgi apparatus to the plasma membrane. Positron emission tomography–computed tomography of patient 2. Ann Hematol 2012; 91: 243–8, Vavricka SR, Burri E, Beglinger C, Degen L, Manz M: Serum protein electrophoresis: an underused but very useful test. Patients with MGUS and smoldering WM should be monitored, on observation, for progression to symptomatic WM. His neurological assessment did not show any neurological impairment, just intense fatigue. HHS Lancet 2017; 18: 241–50, Gerecke C, Fuhrmann S, Strifler S, Schmidt-Hieber M, Einsele H, Knop S: The diagnosis and treatment of multiple myeloma. : Phase II trial of weekly bortezomib in combination with rituximab in relapsed or relapsed and refractory Waldenstrom macroglobulinemia. 80 0 obj <> endobj These patients should undergo a complete diagnostic workup comprising pathological, radiological, and serological examinations to establish the diagnosis and plan the most appropriate treatment in order to improve the prognosis. Copyright © 2011-2020 WMFC.ca. 10-20% of monoclonal gammopathies are of isotype IgM. Non-Hodgkin’s lymphoma. 20 in a series providing the latest information for patients, caregivers and healthcare professionals www.LLS.org • Information Specialist: 800.955.4572 Highlights l Waldenström macroglobulinemia … Waldenström Macroglobulinemia Facts No. Case Rep Dermatol Med. 123 0 obj <>stream Schuster SR, Rajkumar SV, Dispenzieri A, et al. His IgM monoclonal spike was significantly reduced (1700 mg/L). Paiva B, Montes MC, García-Sanz R, Ocio EM, Alonso J, de Las Heras N, Escalante F, Cuello R, de Coca AG, Galende J, Hernández J, Sierra M, Martin A, Pardal E, Bárez A, Alonso J, Suarez L, González-López TJ, Perez JJ, Orfao A, Vidríales MB, San Miguel JF. A correct differential diagnosis between immunoglobulin M multiple myeloma and Waldenström macroglobulinemia is a critical point in the setting of a new immunoglobulin M monoclonal gammopathy onset. When considering ibrutinib, the risks of bleeding and atrial fibrillation must be considered; concurrent use of novel oral anticoagulants should be avoided, and patients with baseline or treatment emergent atrial fibrillation, should be co-managed with a cardiologist. IgM MM is a rare hematological disease representing less than 0.5% of all myeloma cases [1]. The images were obtained with Navios, Backman Coulter flow cytometry, Digital polymerase chain reaction plot for MYD88 L625P mutation on bone marrow samples. Haematologica 2014; 99: 984–96, Murray DL, Ryu E, Snyder MR, Katzmann JA: Quantitation of serum monoclonal proteins: relationship between agarose gel electrophoresis and immunonephelometry. Rummel MJ, Niederle N, Maschmeyer G, et al. Gertz M, Buadi FK: Case vignettes and other brain teasers of monoclonal gammopathies. A diagnosis of immunoglobulin M-κ multiple myeloma was made. However, most compelling is the MRR of 50% seen in the MYD88WT cohort, with a VGPR rate of 26.9% and a 12-month PFS of 72%. Patient 1 was a candidate for bortezomib plus thalidomide and dexamethasone, followed by autologous stem cell transplant consolidation. IgM monoclonal gammopathy of undetermined significance (MGUS) is a pre-cancerous condition with a rate of progression to WM of 1.5% per year, while symptomatic WM, is characterized by 10% or greater bone marrow infiltration by lymphoplasmacytic lymphoma, together with end organ damage related to tumor infiltration … The result of testing for the MYD88 L265P mutation was positive. When autologous stem cell transplant (ASCT) may be considered at disease relapse, nucleoside analog-based therapy should be avoided prior to stem cell harvest. The presence of translocation t(11; 14) detected by fluorescence in situ hybridization (FISH) is shown in IgM MM but is absent in WM [6]. h�b```�����@����9v0��:���z�}P�1V4���������>I(f`�g`Te1e�f:�T���Q���i�;S?S�ȡ��9�'t�3�H)3�r�g�̨3�j Also, for IgM MM, likely other myelomas, CRAB (hypercalcemia, renal failure, anemia, and bone lesions) criteria, and myeloma-defining events (bone marrow plasma cells > 60%, kappa/lambda ratio ≥ 100, > 1 magnetic resonance imaging focal lesion) lead treatment decisions [1, 3]. WM belongs to a spectrum of IgM plasma cell dyscrasias. Get the latest research from NIH: https://www.nih.gov/coronavirus. Leukemia. Patient 1 was a 70-year-old Caucasian man who was an only child. Combinations of bortezomib-rituximab +/– dexamethasone have been associated with major (≥ partial response) response rates (MRR) of 57–83%, while carfilzomib-rituximab-dexamethasone has shown an MRR of 68%, and ixazomib-rituximab-dexamethasone, an MRR of 50%. Bortezomib should be avoided in patients with baseline peripheral neuropathy, while carfilzomib should be used cautiously in those with underlying cardiac conditions. Treatment algorithms for first-line treatment of…, Treatment algorithms for first-line treatment of patients with Waldenström’s macroglobulinemia (modified from [26]). 1a–d, 2a). [7], comprised 134 cases of IgM MM that showed as pathognomonic features the presence of bone marrow clonal plasma cells CD38+ and/or CD138+, cyclin D1+, and t(11,14)-positive in almost 40% of cases. Can J Neurol Sci. His serum creatine level was stable in known chronic kidney disease (creatinine 1.54 mg/dl), and his liver function was normal. Rituximab monotherapy is generally a sub-optimal choice for patients needing systemic therapy, with overall response rates (minor + partial response) of 20–50%, and short progression-free survival (12–24 months). Best Pract Res Clin Haematol. Clin Chem 2009; 55: 1523–9, CCC Ulm, Department of Internal Medicine III, University Hospital Ulm: Dr. med Grunenberg, Prof. Dr. med. In these series, only 15 patients were tested for MYD88 L265P mutation, and all showed negative results. His neurological assessment did not show any neurological impairment. In: Lichtman  MA, Kipps  TJ, Seligsohn  U, Kaushansky  K, Prchal  JT, eds. Lancet 2013; 381: 1203–10, Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, et al. Patients with this disease should be treated in the setting of a clinical trial if possible. Waldenstrom macroglobulinemia (WM) is a type of non-Hodgkin lymphoma (NHL). Smoldering WM is associated with a rate of progression to symptomatic WM, amyloidosis or lymphoma of 12% per year for the first 5 years from diagnosis, followed by 2% per year thereafter. 2003;101(4):1570–1. Springer Nature. Epub 2018 Nov 28. Frontline therapy with rituximab, in combination with either an alkylating agent or proteasome inhibitor +/– dexamethasone, is associated with rapid response, and its efficacy is independent of gene mutation profile. MGUS vs. Cookies policy. Google Scholar. The two patients we describe in this report showed the same bone marrow diagnosis. Essential monoclonal gammopathy. Translocation t(11,14)(q13; q32) is the hallmark of IgM, IgE, and nonsecretory multiple myeloma variants. Differential diagnosis with other IgM-related entities, such as IgM MGUS …