Prediction of immunoglobulin M reduction via therapeutic dose of simple plasma exchange and double filtration plasmapheresis using membrane separation in patients with hyperviscosity syndrome caused by Waldenstrom macroglobulinemia

2018 ◽  
Vol 33 (5) ◽  
pp. 611-615 ◽  
Author(s):  
Yoshihisa Miyamoto ◽  
Yoshifumi Hamasaki ◽  
Akihiko Matsumoto ◽  
Kent Doi ◽  
Eisei Noiri ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Therapeutic Dose ◽  
Membrane Separation ◽  
Immunoglobulin M ◽  
Hyperviscosity Syndrome ◽  
Waldenström Macroglobulinemia ◽  
Double Filtration Plasmapheresis ◽  
Waldenstrom Macroglobulinemia ◽  
Double Filtration

scholarly journals PLASMA EXCHANGE AND DOUBLE FILTRATION PLASMAPHERESIS IN CHRONIC GLOMERULONEPHRITIS PATIENTS WITH GUILLAIN-BARRE SYNDROME

Renal Failure ◽  
2002 ◽  
Vol 24 (3) ◽  
pp. 387-389
Author(s):  
Tsukasa Nakamura ◽  
Chifuyu Ushiyama ◽  
Kaoru Hirokawa ◽  
Shiori Osada ◽  
Teruo Inoue ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Guillain Barre Syndrome ◽  
Chronic Glomerulonephritis ◽  
Double Filtration Plasmapheresis ◽  
Guillain Barré Syndrome ◽  
Double Filtration ◽  
Guillain Barré

scholarly journals Immunoglobulin M (IgM) multiple myeloma versus Waldenström macroglobulinemia: diagnostic challenges and therapeutic options: two case reports

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Simona Elba ◽  
Alessia Castellino ◽  
Roberto Soriasio ◽  
Claudia Castellino ◽  
Margherita Bonferroni ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Case Reports ◽  
Immunoglobulin M ◽  
Therapeutic Options ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia

Establishment and characterization of a novel Waldenström macroglobulinemia cell line, MWCL-1

Blood ◽  
2011 ◽  
Vol 117 (19) ◽  
pp. e190-e197 ◽  
Author(s):  
Lucy S. Hodge ◽  
Anne J. Novak ◽  
Deanna M. Grote ◽  
Esteban Braggio ◽  
Rhett P. Ketterling ◽  
...  
Keyword(s):  
Cell Line ◽  
Plasma Cells ◽  
Immunoglobulin M ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Representative Cell ◽  
Representative Model ◽  
Dna Resequencing ◽  
Tumor Clone

Abstract Waldenström macroglobulinemia (WM) is a rare, lymphoplasmacytic lymphoma characterized by hypersecretion of immunoglobulin M (IgM) protein and tumor infiltration into the bone marrow and lymphatic tissue. Our understanding of the mechanisms driving the development and progression of WM is currently by the shortage of representative cell models available for study. We describe here the establishment of a new WM cell line, MWCL-1. Comprehensive genetic analyses have unequivocally confirmed a clonal relationship between this novel cell line and the founding tumor. MWCL-1 cells exhibit an immunophenotype consistent with a diverse, tumor clone composed of both small B lymphocytes and larger lymphoplasmacytic cells and plasma cells: CD3−, CD19+, CD20+, CD27+, CD38+, CD49D+, CD138+, cIgM+, and κ+. Cytogenetic studies identified a monoallelic deletion of 17p13 (TP53) in both the cell line and the primary tumor. Direct DNA resequencing of the remaining copy of TP53 revealed a missense mutation at exon 5 (V143A, GTG>GCG). In accordance with primary WM tumors, MWCL-1 cells retain the ability to secrete high amounts of IgM protein in the absence of an external stimulus. The genetic, immunophenotypic, and biologic data presented here confirm the validity of the MWCL-1 cell line as a representative model of WM.

Cryoglobulinemia as a Possible Primer for TRALI: Report of a Case

2019 ◽  
Vol 50 (3) ◽  
pp. 313-319 ◽  
Author(s):  
Cameron Beech ◽  
Deepika Kumar ◽  
Jeanne Hendrickson ◽  
Sudhir Perincheri ◽  
Christopher Tormey ◽  
...  
Keyword(s):  
Pulmonary Complications ◽  
Treatment Modalities ◽  
Lymphoplasmacytic Lymphoma ◽  
Monoclonal Antibody Production ◽  
Laboratory Findings ◽  
Systemic Complications ◽  
Hyperviscosity Syndrome ◽  
Waldenström Macroglobulinemia ◽  
Recommended Treatment ◽  
Waldenstrom Macroglobulinemia

AbstractWaldenström macroglobulinemia (WM) is a form of lymphoplasmacytic lymphoma that can cause hyperviscosity syndrome due to unchecked monoclonal antibody production. Some patients are also found to have associated cryoglobulinemia, which can cause systemic complications including vasculitis, renal disease, and pulmonary complications. Cryoglobulins can also serve as a source of interference with various laboratory assays. Therapeutic plasma exchange (TPE) is one of the recommended treatment modalities to manage hyperviscosity.Herein, we present the case of an 84-year-old female patient with Waldenström macroglobulinemia who presented with hyperviscosity syndrome and discrepant laboratory findings, and who then developed transfusion-related acute lung injury (TRALI) during TPE. This case is one of many in the emerging possible linkages observed between cryoglobulinemia and TRALI.

scholarly journals TLR-mediated activation of Waldenström macroglobulinemia B cells reveals an uncoupling from plasma cell differentiation

2020 ◽  
Vol 4 (12) ◽  
pp. 2821-2836
Author(s):  
Jennifer Shrimpton ◽  
Matthew A. Care ◽  
Jonathan Carmichael ◽  
Kieran Walker ◽  
Paul Evans ◽  
...  
Keyword(s):  
Cell Differentiation ◽  
B Cells ◽  
B Cell ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Immunoglobulin M ◽  
B Cell Differentiation ◽  
Waldenström Macroglobulinemia ◽  
Plasma Cell Differentiation ◽  
Waldenstrom Macroglobulinemia

Abstract Waldenström macroglobulinemia (WM) is a rare malignancy in which clonal B cells infiltrate the bone marrow and give rise to a smaller compartment of neoplastic plasma cells that secrete monoclonal immunoglobulin M paraprotein. Recent studies into underlying mutations in WM have enabled a much greater insight into the pathogenesis of this lymphoma. However, there is considerably less characterization of the way in which WM B cells differentiate and how they respond to immune stimuli. In this study, we assess WM B-cell differentiation using an established in vitro model system. Using T-cell–dependent conditions, we obtained CD138+ plasma cells from WM samples with a frequency similar to experiments performed with B cells from normal donors. Unexpectedly, a proportion of the WM B cells failed to upregulate CD38, a surface marker that is normally associated with plasmablast transition and maintained as the cells proceed with differentiation. In normal B cells, concomitant Toll-like receptor 7 (TLR7) activation and B-cell receptor cross-linking drives proliferation, followed by differentiation at similar efficiency to CD40-mediated stimulation. In contrast, we found that, upon stimulation with TLR7 agonist R848, WM B cells failed to execute the appropriate changes in transcriptional regulators, identifying an uncoupling of TLR signaling from the plasma cell differentiation program. Provision of CD40L was sufficient to overcome this defect. Thus, the limited clonotypic WM plasma cell differentiation observed in vivo may result from a strict requirement for integrated activation.

Somatic mutations in MYD88 and CXCR4 are determinants of clinical presentation and overall survival in Waldenström macroglobulinemia

Blood ◽  
2014 ◽  
Vol 123 (18) ◽  
pp. 2791-2796 ◽  
Author(s):  
Steven P. Treon ◽  
Yang Cao ◽  
Lian Xu ◽  
Guang Yang ◽  
Xia Liu ◽  
...  
Keyword(s):  
Overall Survival ◽  
Somatic Mutations ◽  
Clinical Presentation ◽  
Hyperviscosity Syndrome ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Key Points

Key Points Activating MYD88 as well as nonsense and frameshift WHIM-like CXCR4 somatic mutations are common in WM. CXCR4 NS mutations are present in aggressive cases including hyperviscosity syndrome, and MYD88 status is a determinant of survival.

Blood viscosity in Waldenstrom macroglobulinemia

Blood ◽  
1977 ◽  
Vol 49 (4) ◽  
pp. 507-510 ◽  
Author(s):  
MR MacKenzie ◽  
TK Lee
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Direct Measurement ◽  
Blood Viscosity ◽  
Whole Blood ◽  
Hyperviscosity Syndrome ◽  
Whole Blood Viscosity ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Low Serum

Patients with Waldenstrom macroglobulinemia were studied for the presence or absence of the hyperviscosity syndrome, the relative serum viscosity value, and the calculated whole blood viscosity to identify a level at which symptoms occurred. The majority of symptomatic patients had whole blood viscosity values above 8.0 centipoises. There was a direct correlation between whole blood viscosity and relative serum viscosity, r = 0.75. One patient with central nervous system abnormalities was identified as having a high whole blood viscosity but a low serum viscosity. It was concluded that the vast majority of patients with the hyperviscosity syndrome will be identified by measuring the relative serum viscosity. In patients with central nervous system findings and a low serum viscosity, the whole blood viscosity should be determined either by direct measurement or by calculation.

scholarly journals Primary Therapy of Waldenström Macroglobulinemia With Bortezomib, Dexamethasone, and Rituximab: WMCTG Clinical Trial 05-180

2009 ◽  
Vol 27 (23) ◽  
pp. 3830-3835 ◽  
Author(s):  
Steven P. Treon ◽  
Leukothea Ioakimidis ◽  
Jacob D. Soumerai ◽  
Christopher J. Patterson ◽  
Patricia Sheehy ◽  
...  
Keyword(s):  
Herpes Zoster ◽  
Peripheral Neuropathy ◽  
Response Rates ◽  
Immunoglobulin M ◽  
Primary Therapy ◽  
Waldenström Macroglobulinemia ◽  
Bone Marrow Disease ◽  
Waldenstrom Macroglobulinemia ◽  
Best Response ◽  
Prophylactic Antiviral Therapy

Purpose We examined the activity of bortezomib, dexamethasone, and rituximab (BDR) in patients with symptomatic, untreated Waldenström macroglobulinemia (WM). Patients and Methods A cycle of therapy consisted of bortezomib 1.3 mg/m2 intravenously; dexamethasone 40 mg on days 1, 4, 8, and 11; and rituximab 375 mg/m2 on day 11. Patients received four consecutive cycles for induction therapy and then four more cycles, each given 3 months apart, for maintenance therapy. Twenty-three patients received a median of seven cycles of treatment. Results Median bone marrow disease involvement declined from 55% to 10% (P = .0004), serum immunoglobulin M levels declined from 4,830 to 1,115 mg/dL (P < .0001), and hematocrit increased from 29.8% to 38.2% (P = .0002) at best response. The overall response rates and major response rates were 96% and 83% with three complete responses, two near complete responses, three very good partial responses, 11 partial responses, and three minor responses. Responses occurred at a median of 1.4 months. With a median follow-up of 22.8 months, 18 of 23 patients remained free of disease progression. Peripheral neuropathy was the most common toxicity, and it resolved to grade ≤ 1 in 13 of 16 patients at a median of 6.0 months. Four of the first seven treated patients developed herpes zoster, resulting in the institution of prophylactic antiviral therapy. Conclusion The results demonstrate that BDR produces rapid and durable responses, along with high rates of response and complete remissions in WM. Herpes zoster prophylaxis is necessary with BDR, and reversible peripheral neuropathy was the most common toxicity leading to premature discontinuation of bortezomib in 61% of patients. Exploration of alternative schedules for bortezomib administration that includes weekly dosing should be pursued.

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