Waldenstrom macroglobulinemia (mak-roe-glob-u-lih-NEE-me-uh) is a rare type of cancer that begins in the white blood cells.If you have Waldenstrom macroglobulinemia, your bone marrow produces too many abnormal white blood cells that crowd out healthy blood cells. eCollection 2020. Clin Lymphoma Myeloma Leuk 2011; 11: 74–6, Xu L, Hunter ZR, Tsakmaklis N, et al. endstream endobj 81 0 obj <. 2015;373:584–6. Esophagogastroduodenoscopy and colonoscopy demonstrated jejunal angiodysplasia, so he was treated with iron support, which led to a partial recovery of his Hb level. 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. WM belongs to a spectrum of IgM plasma cell dyscrasias. The abnormal white blood cells produce a protein that accumulates in the blood, impairs circulation and causes complications.Waldenstrom macroglobulinemia is considered a type of non-Hodgkin's lymphoma. Our patient 2 was considered unfit and underwent rituximab plus cyclophosphamide plus dexamethasone, with a good response. In the setting of a WM diagnosis, treatment is different and has been changing in recent years. Blood 2015; 126: 1392–4, Tedeschi A, Benevolo G, Varettoni M, et al. On the basis of the proven efficacy of Bruton tyrosine kinase inhibitors such as ibrutinib in MYD88 L256P–related hematological disease, ibrutinib is considered by the recent guidelines for treatment of WM in both first and further lines of therapy [8, 9]. Evidence-based treatment decisions in Waldenström's macroglobulinemia now rely mainly on small-scale, single-armed trials. 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 … Because of his high IgM serum level, three plasma exchange procedures were performed before treatment initiation. van de Donk NW, Palumbo A, Johnsen HE, et al. His IgM monoclonal spike was significantly reduced (1700 mg/L). He had a mild fever with a body temperature of 38.2 °C. Epub 2013 Apr 22. statement and However, patients with symptomatic WM, defined as WM associated with a disease-related hemoglobin level of less than 10 g/dL, a platelet count of less than 100×109/L, bulky adenopathy or organomegaly, symptomatic hyperviscosity, severe neuropathy, amyloidosis, cryoglobulinemia, cold agglutinin disease, or evidence of disease transformation, should be considered for immediate therapy. Br J Haematol 2011; 153: 309–17, Swiecicki PL, Hegerova LT, Gertz MA: Cold agglutinin disease. Waldenström's macroglobulinemia is a type of cancer affecting two types of B cells: lymphoplasmacytoid cells and plasma cells. 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]. MYD88 L265P somatic mutation in Waldenström's macroglobulinemia. https://doi.org/10.1186/s13256-020-02380-2, DOI: https://doi.org/10.1186/s13256-020-02380-2. 2017;92:746–51. N Engl J Med 2006; 354: 1362–9, Eisele L, Durig J, Huttmann A, et al. 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. 3b). As expected, translocation t(11,14) was detected by FISH analysis, and the result of testing for the MYD88 L256P mutation was negative. Considering the patient’s age and comorbidities, FISH analysis was not performed. With a final diagnosis of IgM MM, the treatment for young patients who are transplant eligible is composed of a bortezomib-based induction therapy (for example, bortezomib plus thalidomide plus dexamethasone), followed by ASCT and maintenance treatment [10]. : Ibrutinib in previously treated Waldenstrom‘s macroglobulinemia. On the basis of these data, a diagnosis of WM was made. Waldenstrom macroglobulinemia (WM) is a type of non-Hodgkin lymphoma (NHL). Multiple Myeloma vs. Waldenstrom’s. His low-dose computed tomographic scan excluded lytic bone lesions and did not reveal evidence of any adenopathy or organomegaly. IgM MM is characterized by the neoplastic proliferation of plasma cells preferentially in the bone marrow, producing a monoclonal immunoglobulin in the blood and/or urine [2]. Background: Curr Opin Neurol 2009; 22: 480–5, Sokol RJ, Hewitt S, Stamps BK: Autoimmune haemolysis: an 18-year study of 865 cases referred to a regional transfusion centre. Get the latest research from NIH: https://www.nih.gov/coronavirus. About 93–97% of patients with WM have a somatic mutation in MYD88, an adaptor protein in the B cell receptor pathway that triggers downstream signaling through BTK and IL-1 receptor-associated kinases, which, in turn, promotes NF-κB signaling. Maintenance therapy with lenalidomide (10 mg/day) was planned in case of a good response. Article  Glavey SV, Leung N: Monoclonal gammopathy: The good, the bad and the ugly. Median age (years) at diagnosis is 63 for blacks, and 73 for whites. 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]. 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%. Otherwise, he had a silent medical history and did not consume any chronic medication before his hematological disease diagnosis. Lancet Oncol 2014; 15: e538–48, Buske C, Leblond V, Dimopoulos M, et al. 107 0 obj <>/Filter/FlateDecode/ID[<4AD642BED12795469F3E8238D4EE01E3>]/Index[80 44]/Info 79 0 R/Length 123/Prev 195085/Root 81 0 R/Size 124/Type/XRef/W[1 3 1]>>stream 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. However, clinical presentation and radiological analysis suggested different hematological diseases, demonstrating that biopsy alone is not sufficient for a correct final diagnosis.