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Nevertheless, these two hematological entities require different treatments and have different outcomes, so a correct diagnosis is mandatory. Bonilla-Valentín FJ, Cerra J, Cáceres-Perkins W, Alsina M. Case report of IgM multiple myeloma: diagnosing a rare hematologic entity. Lancet. This disease’s symptoms, treatment, and outlook vary. Google Scholar. Waldenström macroglobulinemia, one of the malignant monoclonal gammopathies, is a chronic, indolent, lymphoproliferative disorder. He had a mild fever with a body temperature of 38.2 °C. Castillo JJ, Gustine J, Meid K, et al. a Patient 1. b Patient 2. Chapter Chapter 11: Multiple Myeloma and Other Plasma Cell Dyscrasias. Our patient 2 was considered unfit and underwent rituximab plus cyclophosphamide plus dexamethasone, with a good response. Background: Immunoglobulin M multiple myeloma and Waldenström macroglobulinemia are two different hematological diseases with the common finding of an immunoglobulin M monoclonal … He had undergone surgery for renal lithiasis at the age of 20 years and transurethral resection of the prostate 2 years before his presentation at our clinics. Blood. It is a slow-growing type of non-Hodgkin lymphoma. 2014 Jun 19. It can be based on chemoimmunotherapy regimens, such as rituximab plus bendamustine in suitable patients or rituximab plus cyclophosphamide plus dexamethasone in unsuitable ones [11, 12]. 2018;29(Suppl 4):iv270; Ann Oncol. WM is an IgM-secreting lymphoplasmacytic lymphoma that is more likely characterized by enlarged adenopathy, hepatomegaly and/or splenomegaly, anemia, IgM component–related symptoms such as hyperviscosity and peripheral neuropathy, and sometimes constitutional symptoms [3, 4]. Patient 1 (Caucasian man) presented with increased serum protein and immunoglobulin M (7665 mg/L) with an M-spike electrophoresis of 4600 mg/L. Waltham: UpToDate; 2017. ORRs in 31 response-eligible Waldenström macroglobulinemia patients were 71.4% and 47.1% for the 2/7 and 5/14 cohorts, respectively. Multiple myeloma, Waldenström's macroglobulinemia and benign monoclonal gammopathy are examples of diseases in which there is an uncontrolled proliferation of cells involved in antibody synthesis. It is characterized by the presence of a high level of a macroglobulin (immunoglobulin M [IgM]), elevated serum viscosity, and the presence of a lymphoplasmacytic infiltrate in the bone marrow. The authors declare that they have no competing interests. Immunoglobulin M (IgM) multiple myeloma (MM) and Waldenström macroglobulinemia (WM) are two different hematological diseases with the common finding of an IgM monoclonal gammopathy of unknown significance (MGUS) .IgM MM is a rare hematological disease representing less than 0.5% of all myeloma … During this phase, all patients with myeloma on a 2/7 schedule received tablets. Predicting Progression of Developing Myeloma … All authors read and approved the final manuscript. Malignant proliferation of plasmacytoid lymphocytes; IgM >50g/L (5 g/dL) Bence Jones proteinuria in 10% of cases; Hyperviscosity syndrome (due to … Computed tomography showed many enlarged lymph nodes and splenomegaly. The data that support the findings in this report were collected from clinical/pathological/laboratories/radiologic reports and charts recorded in paper or informatics systems and are available from the corresponding author on reasonable request. Google Scholar. Testing for somatic MYD88 L265P mutation showed a negative result (Fig. Because the neoplastic clone was CD20−, CD56−, CD138+, and cyclin D1+ with the absence of a somatic MYD88 L265P mutation and with a positivity for t(11,14), and in consideration of the patient’s clinical presentation (absence of adenopathy, organomegaly, and B-constitutional symptoms), a diagnosis of WM was unlikely, and IgM-κ MM was finally defined. Some months later, new worsening of anemia and progressive thrombocytopenia were observed (Hb 8.5 g/dl, platelets 40,000/mm3, with normal blood cell count of leukocytes 8350/mm3, neutrophils 3400/mm3). PubMed Google Scholar. Maintenance therapy with lenalidomide (10 mg/day) was planned in case of a good response. The result of testing for the MYD88 L265P mutation was positive. Differential diagnosis with other IgM-related entities, such as IgM MGUS and WM, still represents a challenge for clinicians. Cookies policy. Part of These types are based on the immunoglobulin (protein) produced by the myeloma cell. Our patient 1 showed both a clinical presentation (no adenopathy and splenomegaly, no B symptoms, and no new-onset bone pain, but a moderate macrocytic anemia) and bone marrow histology suggestive of IgM MM. The cancer cells in people with WM are similar to those of 2 other types of cancer: multiple myeloma and non-Hodgkin lymphoma. The presence of an MYD88 mutation is usually absent in IgM MM; in contrast, it is pathognomonic in WM . His serum creatinine and calcium levels were normal, while a moderate macrocytic anemia was observed (hemoglobin [Hb] serum level 9.5 g/dl; while platelet and leukocyte counts were normal). A diagnosis of immunoglobulin M-κ multiple myeloma was made. Plasmacytoma, an early-stage plasma cell dyscrasia, originates from the same type of malignant plasma cells but is characterized by solitary cell proliferation that forms a mass. A correct differential diagnosis between IgM MM and WM is a critical point in the management of patients with new-onset IgM monoclonal gammopathy. 6 2223(Leu3kä 7 Das menschliche Immunsystem Um Ihnen die Entstehung und das Verständnis der Erkrankung zu er-leichtern, stellen wir Ihnen kurz die wesentlichen Grundzüge unseres Immunsystems vor: … Atacicept, a specific inhibitor of BLys and APRIL, was used in a phase I study for 14 patients with myeloma (MM) and 4 with Waldenström's macroglobulinemia (WM). In: Post TW, editor. 3a). His liver function was normal, but his β2-microglobulin was increased (3.15 mg/dl). S. et al. Patient 1 was a candidate for bortezomib plus thalidomide and dexamethasone, followed by autologous stem cell transplant consolidation. Chapter 37: Multiple Myeloma and Other Plasma Cell Disorders. 123(25):3999-4000. He underwent ASCT in November 2019, reaching a very good partial remission, and maintenance treatment with lenalidomide is ongoing. Around 0.5-1% of MGUS cases progress to myeloma each year and current standards for predicting the risk of progression are inadequate. All authors contributed to manuscript editing. Learn more about the disease, and its symptoms, causes, and treatments. The two patients we describe in this report showed the same bone marrow diagnosis. On the basis of a comprehensive diagnostic workup, these patients were treated with different approaches. Owen RG, Treon SP, Al-Katib A, et al. Moreover, higher importance is given to a precise diagnostic assessment because the availability of target biological drugs (such as ibrutinib or venetoclax) could represent a powerful therapeutic strategy if given to selected patients. Patient 2 was diagnosed with Waldenström macroglobulinemia and received rituximab, cyclophosphamide, and dexamethasone. Distinguishing these two diagnoses is critical as the approach to therapy is different. Waldenström's macroglobulinemia is a type of cancer affecting two types of B cells: lymphoplasmacytoid cells and plasma cells. The initial laboratory test results showed remarkably increased levels of total serum protein (10,700 mg/L) and IgM (7665 mg/L), with protein electrophoresis showing an M-spike of 4600 mg/L (43.3%). AU - Merlini, Giampaolo. Waldenström's macroglobulinemia and multiple myeloma may merely be opposite ends of the same disease spectrum, with intermediate forms, such as the case in point, occurring. Waldenström macroglobulinemia (WM) is a low-grade B-cell clonal disorder characterized by lymphoplasmacytic bone marrow involvement associated with monoclonal immunoglobulin M. Although WM remains to be an incurable disease with a heterogeneous clinical course, the recent discovery of mutations in the MYD88 and CXCR4 genes further enhanced our understanding of its pathogenesis. Bone marrow biopsy slides of representative immunohistochemistry. Terms and Conditions, Y1 - 2004. He showed hypertension, chronic bronchopneumopathy, a known kidney injury, and a cardioembolic stroke episode related to chronic atrial fibrillation without clinical outcomes. Kastritis E, Leblond V, Dimopoulos MA, et al. His IgM monoclonal spike was significantly reduced (1700 mg/L). The combination of the ixazomib, dexamethasone, and rituximab (IDR) yields durable responses and maintains an excellent safety profile in patients with Waldenström macroglobulinemia (WM), according to results of a study published in Blood Advances.. https://doi.org/10.1186/s13256-020-02380-2, DOI: https://doi.org/10.1186/s13256-020-02380-2. The data were collected in accordance with the Declaration of Helsinki and good clinical practice guidelines. His bone marrow biopsy revealed immunoglobulin M-κ multiple myeloma. 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. 2017;376(14):1311–20. Ann Oncol. 1a–d, 2a). Before his hematological diagnosis, he was receiving treatment with many chronic medications: valsartan/amlodipine for hypertension, salbutamole inhaled for chronic bronchopneumopathy, and bisoprolol and anticoagulation for atrial fibrillation. Multiple Myeloma vs. Waldenstrom’s. Because Waldenstrom macroglobulinemia is similar to some B-cell lymphomas or multiple myeloma, treatment can often be similar for these patients. Manage cookies/Do not sell my data we use in the preference centre. Common in elderly population (~10% in patients >75 years old), No treatment needed, but must be monitored as 1-1.5% will progress to Multiple Myeloma per year, Neoplastic proliferation of a single plasma cell lineage aka, Paraprotein >30g/L (>3g/dL) [serum and/or urine]. Induction treatment included bortezomib, dexamethasone, and thalidomide (bortezomib 1.3 mg/m2 on days 1, 4, 8, and 11 of each 28-day cycle; dexamethasone 40 mg/day once per week; thalidomide 100 mg/day) for a total of four 28-day courses. Treated with bisphosphonates, steroids, chemotherapy (If below age 65 consider autologous hematopoietic stem-cell transplantation). He experienced rapidly progressive fatigue, and basal blood laboratory test results showed a severe normocytic anemia (Hb 7.3 g/dl) with normal platelet and white blood cell counts. Milder regimens can be used in frail patients. a Patient 1, MYD88 result, wild type. A correct differential diagnosis between IgM MM and WM still represents a challenge for clinicians because of the small number of cases of IgM MM reported in the literature. Waldenstrom's Macroglobulinemia. His clinical examination revealed that he had axillary bilateral enlarged adenopathy and palpable splenomegaly, 6-kg weight loss in the last few months, no night sweats, and no new-onset bone pain. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Multiple myeloma (Kahler's disease) is a malignant plasma cell dyscrasia characterized by uncontrolled proliferation and the diffuse infiltration of monoclonal plasma cells in the bone marrow. Patient 1 was a 70-year-old Caucasian man who was an only child. Lancet Oncol 2014; 15:e538. CD20 was positive in half of the patients. Written informed consent was obtained from the patients for publication of this case report and any accompanying images. In this report, we describe two cases of immunoglobulin M neoplasm with the same histological bone marrow presentation but with different clinical behavior, cytogenetics, and biological assessment. Waldenström's macroglobulinemia (Waldenström's) is a rare white blood cell cancer. N Engl J Med. Bendamustine plus rituximab versus CHOP plus rituximab as first line treatment for patients with indolent and mantle cell lymphomas: an open label, multicenter, randomized, phase III non inferiority trial. IgM multiple myeloma: disease definition, prognosis, and differentiation from Waldenström’s macroglobulinemia. Waldenström macroglobulinemia, one of the malignant monoclonal gammopathies, is a chronic, indolent, lymphoproliferative disorder. A bone marrow biopsy is mandatory for a correct diagnosis, together with a complete histopathological, immunohistochemical, cytogenetic, and molecular analysis. 2018;25(1):1073274817744448. 2018. https://doi.org/10.30637/2018.17-090. Some patients presented with hyperviscosity symptoms. MGUS vs. Multiple myeloma: clinical features, laboratory manifestations, and diagnosis. ORRs in 31 response-eligible Waldenström macroglobulinemia patients were 71.4% and 47.1% for the 2/7 and 5/14 cohorts, respectively. Article Elba, S., Castellino, A., Soriasio, R. et al. 2013;381:1203–10. 2018;29(Suppl 4):iv41–50. IgM multiple myeloma is a distinct entity; although constituting only 1% of all multiple myeloma cases, it must be distinguished from WM. The various immunoglobulins have different … Two different dose-intensive schedules will be employed in these phase I studies. There is no definitive treatment (chemotherapy and plasmapheresis for hyperviscosity syndrome). 2003;101(4):1570–1. On the basis of these data, a diagnosis of WM was made. b Patient 2, MYD88 L625P result, mutated. … Your email address will not be published. He had no positive history of allergy, was a smoker, and did not drink alcohol. Cilla N, Vercruyssen M, Ameye L, et al. The clinical presentation of these patients usually met CRAB criteria, with no splenomegaly and/or adenopathy. Esteban Braggio; Rafael Fonsec. His neurological assessment did not show any neurological impairment, just intense fatigue. His low-dose computed tomographic scan excluded lytic bone lesions and did not reveal evidence of any adenopathy or organomegaly. However, clinical characteristics of the two entities can overlap. Waldenstrom's macroglobulinemia is a rare type of cancer; specifically it is a type of non-Hodgkin Lymphoma. Positron emission tomography–computed tomography of patient 2. Since individuals with MGUS do not need treatment but only monitoring, it is important to distinguish this group from those with progressive plasma cell disorders. Upon admission to the emergency room, the patient’s blood pressure was 105/70 mmHg, and his pulse was 95 beats/minute with known atrial fibrillation rhythm. Waldenström’s macroglobulinaemia: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. It usually affects older adults and is primarily found in the bone marrow, although lymph nodes and the … Patient 2 was an 84-year-old Caucasian man with two brothers and one sister. This study is to evaluate the safety, efficacy and clinical benefit of BGB-3111 vs ibrutinib in subjects with MYD88 Mutation Waldenström's Macroglobulinemia. A priori, we defined IgM MM as a symptomatic clonal plasma cell proliferative disorder characterized by an IgM monoclonal protein … Among the most common AEs (>10%), atrial fibrillation (14% vs 2%), edema peripheral (19% vs 9%), muscle spasms (23% vs 10%), and pneumonia (12% vs 2%) were more frequent with ibrutinib than zanubrutinib, respectively, (2-sided P< .05 for all); whereas, the rate of neutropenia was higher with zanubrutinib (25%) than ibrutinib (12%; 2-sided P <.05).